3rd Hammersmith Multidisciplinary Endocrine Symposium 2008 12 December 2008, London, UK Endocrine Abstracts December 2008 Volume 18 ISSN 1470-3947 (print) ISSN 1479-6848 (online)
3rd Hammersmith Multidisciplinary Endocrine Symposium 2008 12 December 2008, London, UK
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12 December 2008, London, UK
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Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008
CONTENTS
3rd Hammersmith MultidisciplinaryEndocrine Symposium 2008
ORAL COMMUNICATIONS ...... OC1–OC7
POSTER PRESENTATIONS ...... P1–P41
INDEX OF AUTHORS
Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008
Oral Communications
Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008
OC1 not be solely reserved for recurrent disease. The rarity of adrenocortical Bilateral adrenalectomy in apatient with congenital adrenal carcinoma makes the undertaking of adequately poweredrandomised trials hyperplasia difficult. Owais Chaudhri1 ,Emma Hatfield1 ,Katie Wynne2 ,Fausto Palazzo1 , Sanjeev Mehta1 ,Humera Shaikh1 ,Tricia Tan1 ,Niamh Martin1 & Karim Meeran1 1 Endocrine Unit, Department of Investigative Medicine, Imperial College Healthcare NHS Trust, London, UK; 2 Department of Endocrine Surgery, Imperial College Healthcare NHS Trust, London, UK. OC3 An unusual case of hypertension We present the case of a33-year-old woman diagnosed with congenital adrenal Binu Krishnan &Emma Bingham hyperplasia (CAH)due to classical 21-hydroxylase deficiencyfollowing asalt- Frimley Park Hospital, Frimley, Surrey, UK. losing crisis as anewborn. She had previously been controlled on dexamethasone 0.25 mg bd and fludrocortisone 100 mcg od, as evidenced by high-normal 17-hydroxyprogesterone (17-OHP) and low-normal testosterone levels (8.5 and A21-year-old femalepatient was referred from the eye clinic after she was noted 0.4 nmol/l respectively). to have bilateral papilloedema during aroutine eye examination. She gave a3 However, she had experienced significant symptoms of Cushing’s syndrome on months history of intermittent headaches and fleeting episodes of profuse this replacement regimen (body mass index 34.6 kg/m2 ,striae). Areductioninthe sweating and rash, unrelated to the headaches. She had been investigated by the dose of dexamethasone resulted in secondary amenorrhoea and distressing GP with routine blood tests which were found to be normal. hirsutism refractory to treatment by conservative measures. Efforts to identify a On examination, shewas notedtobetachycardic at 100beats/min and steroid replacement dose that adequately suppressed adrenal androgen production hypertensive at 189/130 mmHg. She had erythematous maculopapular rashes without causing further progression of her iatrogenic Cushing’s syndrome were over the lower forearm and hand bilaterally. Examination of the fundus confirmed unsuccessful. bilateral papilloedema. ECG showed evidence of left axis deviation and left The patient underwent along synacthen test to characterise the level of residual ventricular hypertrophy. CXR showed normal sized heart and clearlung fields. adrenal cortisol synthesis. Cortisol levels were undetectable throughout, in the She was commenced on Nifedipine MR 20 mg bd. Ultrasound of the abdomen presence of asignificant rise in 17-OHPlevels (peak levels54.6 nmol/l), revealed a4cm right adrenal mass. MRI of the brain was reported as normal.An confirming complete blockade of the21-hydroxylase enzyme.Bilateral MRI of the adrenals and MIBG scan were arranged. adrenalectomy was therefore considered as atreatment option to permit the use Nifedipine was stopped and she was commenced on Phenoxybenzamine 10 mg of lower doses of exogenous steroid and removing the source of excess androgens. bd. As the patient remained well with improved control of her blood pressure, she She underwentanuncomplicated bilateral laparoscopic adrenalectomy in October was commenced on Propranolol 40 mg bd after 3days. 2007. One year later, she is maintained on hydrocortisone replacement (10 mg The patient unfortunately had acardiac arrest and died despiteprolonged am, 5mglunchtime) and has gradually lost weight. Symptomatically, she has resuscitation. The coroner reported cause of death as acute pulmonary oedema improved significantly. secondary to malignant hypertension due to aright adrenal phaeochromocytoma. Discussion will revolve around the use of adrenalectomy to overcome the difficult Results of 3 ! 24 hurinecatecholamines obtained thereafter showed nor- need to balanceadequate suppressionofadrenal androgen production vs weight adrenalinelevelsalmost100 times more than thenormal levels at gain in women with CAH. 11 800/24 700/19 100 m g/24 h(normal range ! 100) and normetanephrine levels at 100/47/30 m g/24 h(normal range ! 3.3). This case highlights the difficulty in treating these extremely rare tumours and the potentially fatal complications due to an adrenergic crises in these patients.
OC2 Adrenocortical carcinoma presenting as Cushing’s syndrome: 2case reports Adeel Ghaffar1 ,Daniel Morganstein1 ,James Kirkby-Bott2 ,Jeannie Todd1 & Fausto Palazzo2 OC4 1 Department of Endocrinology and Metabolism, Hammersmith Hospital, MEN1: the full house Imperial College Healthcare NHS Trust, London, UK; 2 Department of Barbara McGowan, Annabel Fountain, Owais Chaudhri, Puja Mehta, Endocrine Surgery, Hammersmith Hospital, Imperial College Healthcare Tricia Tan, Emma Hatfield, Niamh Martin, Jeannie Todd &Karim Meeran NHS Trust, London, UK. Imperial College, London, UK.
A69-year-oldlady presented with pancreatitis, which was managed conserva- This gentleman was diagnosed with hyperparathyroidism at the age of 34 and was tively and resolved.However, imaging identifieda4cmleft adrenal lesion. She found to have multiple manifestations of MEN1. had no features of Cushing’s syndrome, and was normotensive, but serum Hyperparathyroidism potassium was low. Renin:aldosterone ratio was normal. She failed alow dose He was diagnosed with hyperparathyroidism and underwent aparathyroidectomy dexamethasone suppression test (LDDST).She underwentlaparoscopic with removal of 2glands. Fouryears later calcium levels were raised once again adrenalectomy. Histology showed an adrenocortical carcinoma extending into andtwo furtherhyperplastic glands were removed, with cure of his the adjacentadipose tissue. Sixteen months postoperatively she failed an LDDST. hypercalcaemia. AMRI adrenalsshowed a1.5! 1.4 cm soft tissue mass, thought to represent Gastrinoma and other pancreaticislet cell tumours recurrence of the adrenocortical carcinoma. She underwentrepeatsurgery with He developed heartburn, nausea, vomiting and abdominal pain, and on upper GI removal of the recurrent tumour, the left kidney, the spleen and asegmentof endoscopy wasfound to have moderate duodenitis.Hewas startedon colon. The histology showed multifocal adrenocortical carcinoma involving the lansoprazolewith immediate relief of his symptoms. Gut hormones were previous surgical bed. She was commenced on acourse of mitotane. Twenty-two elevated suggestive of agastrinoma and other islet cell tumours. Imaging revealed months after her initial surgery, imaging was performed in view of abdominal multiple duodenal and pancreatic lesions. Calcium stimulation tests revealed no pain.This showed local recurrence and peritoneal deposits. discrete gastrin releasing foci amenable to surgery. A60-year-oldman was referred to our centre with adiagnosis of ACTH Pituitary disease independentCushing’s syndromeand a7.6 cm rightadrenal mass,after He complained of impotence, reduced libido, weight gain andappeared presenting with hypertension. ACTwith contrast suggested that this was a Cushingoid. Cushing’s disease and aprolactinoma were diagnosed on the basis benign lesion. He underwentanopen adrenalectomy,asthe lesion was closely of elevatedurinary cortisols,failure to suppress cortisol on alow dose applied to the liver. Histology identified an adrenocortical tumour with ahigh dexamethasone suppression test, inferior petrosal sinus sampling and elevated mitotic rate and aKi67 index O 5%. This was consistent with an adrenocortical prolactin. Two pituitary lesions were seen on pituitary MRI. The patient carcinoma, and there was evidence of involvement of peri-adrenal fat. He underwent trans-sphenoidal hypophysectomy.Hewas started on hydrocortisone, subsequently underwent chemotherapy with mitotane. DDAVP, thyroxine and testosterone replacement. Mitotane is asynthetic derivative of the insecticide DDT. It inhibits cortisone Adrenal lesions metabolism, and also has cytotoxic effects on the adrenal cortex. Its role in the ACTscan of the adrenalsshowed bilateral nodularadrenal enlargement. management of adrenocortical carcinoma is unclear. Recentretrospective data Phaeochromocytoma was excluded on the basisofnormal catecholamine suggest that it should be used as adjuvant treatment followingsurgery, and should excretions.
Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008
Other lesions He developed lipomas on his zygomatic arch and thigh and an angiofibroma on Jul–Oct the nose. Date Feb ’04 Sep ’05 ’06 Dec ’06 Feb ’07 Jul ’08 Genetic testing MEN1was confirmed.One of his 3children has an MEN1 mutation. Calcito- 14.1 15.8 34.1– 41.3 49.1 46.8 Conclusions nin (ng/l) 32.9 Ourpatient developed afull houseofendocrineneoplasias. He presented a Normal 11.5* 11.5 18.9 18.9 18.9 18.9** diagnostic and management challenge requiring amulti-disciplinary approachto (ng/l) ensure optimal treatment. Peak calcitonin during apentagastrin stimulation test was *152.0 in 2004 and **173.0 in July 2008.
His CEA, urinary catecholamines and fasting gut hormones have remained normal. Genetic screening for MEN-1 and MEN-2 ( RET exons 1–20) was OC5 negative. Yearly thyroid ultrasound scans have been normal.AnMRI neck scan MEN 1with adrenal Cushing’s (a rare association) was normal in 2004, and aDimercaptosuccinic Acid (DMSA) scan was normal in KOShaafi, SRussell &SRoberts 2006. He is now24yearsold. Despite the developmentofprimary Chase Farm Hospital, Middlesex, UK. hyperparathyroidism at ayoung age and an increasingly elevated calcitonin level, there has been no histological evidence of C-cell hyperplasia, genetic evidence forincreased risk of medullary thyroid carcinoma(MTC), nor A36-year-oldgentleman presented to his GP 2years ago with hypertension and radiological evidence of MTC. There are surgical risks of hypoparathyroidism was commenced on valsartan.InJanuary 2008, he was noted to have mildly and voice changewith repeatneck surgery. Our MultidisciplinaryEndocrine deranged LFT and abdominal ultrasound scan showed mild fatty liver infiltration Meeting has concludedthat there is no current indication for prophylactic and bilateral renal stones. He was referred to Urology for investigation of renal thyroidectomy. The follow-up plan is for annual assay of calcitonin and neck stones and aCTIVU showed multiple renal calculi, a6! 5 ! 5cmsoft tissue ultrasound scans with fine needle aspiration of any thyroid nodules. In the absence enhancing lesion arising from the right adrenal gland and a7mm calcified density of asuspicious nodule, it is open for discussion whether there is alevel of basal or within the body of the pancreas of benign appearance. Adrenal protocol CT stimulated calcitonin which should promptfurther consideration of prophylactic confirmed the adrenal lesion. At this stage he was referred to the endocrine team thyroidectomy. for assessment of the adrenal mass. On examination he had aplethoric facial appearance, centripedal obesity and gynaecomastia. Twenty-fourhours urine collections for catecholamines and cortisol were normal on two occasions. An overnight dexamethasone suppressiontest and the low dose dexamethasone suppression tests were both positive, with suppressed basal ACTH. Routine bloods revealed elevated calcium3.07 mmol/lwith elevated PTH 16.3 pmol/l. OC7 Baseline pituitary profile revealed elevated prolactin 709 mU/l and normal Management of familial medullary thyroid cancer: not as simple as it gonadotrophins LH 2.3 IU/l, FSH 1.7 IU/l, testosterone 13.3 nmol/l. seems He underwentlaparoscopic right adrenalectomy.Histopathology confirmed Leena Krishnan &James Ahlquist cortisol producing adrenal adenoma. Since removal of the adrenal adenoma he Endocrine Unit, Southend Hospital, Southend on Sea, Essex, UK. has remained offantihypertensive medication. Postoperatively the cortisol level suppressed on low dose dexamethasone suppression test. Optimal management of familial medullary thyroid cancer (MTC) involves early MRIpituitary showed 7mmpituitary microadenoma and sesta Mibi parathyroid genetic diagnosis of affectedindividuals and prophylactic thyroidectomy. Patients scan revealed increased uptake in the left inferior positionsuggestive of diagnosed later may have biochemical evidence of metastases which are clinically parathyroid adenoma, but there was also delayed uptake in the other three silent. We report afamily which illustrates the management difficulties which parathyroid glands. He is due for parathyroid surgery and he is likely to undergo may arise with this approach. SC, ahealthy 25-year-old man, presented for four gland removal. We are currently in the process of investigating whether he assessment for MTC. His mother had had atotal thyroidectomy for MTC with no has apancreatic tumour with fasting gut hormones screen. clinical evidence of metastases, but after surgery she had araised calcitonin level On review of the literature there are few reportedcases of MENIwith concurrent (0.36 m g/l, normal ! 0.08 by RIA).Investigationhas shown no definite adrenal adenoma producing cortisol. Forty percent of MEN cases are associated localisation of metastases, serum calcitonin remains elevated at 169 ng/l (by with adrenal nodular hyperplasiawith no endocrinopathy. Thereforewewould ICMA), she has had no further treatment and she remains well. SC had araised like to submit this interesting case for presentation at The Third Hammersmith serum calcitonin level, and thyroidectomy confirmed MTC. After surgery the Multidisciplinary Symposium. calcitonin remained elevated (8.8 m g/l by RIA) and he underwent bilateral neck dissection and external radiotherapy to the neck and mediastinum. He also remains well, with no clinical evidence of recurrence, but the serum calcitonin has risen over 8years, currently 2735 ng/l (normal ! 18.9 ng/l by ICMA). RET proto- oncogene analysis was performed in 1999 to identify amarkerfor MTC in this family. No abnormality was found in exons 10 or 11, the two exons then known to harbour causative mutations. Further analysisidentifiedamutation in exon 15 OC6 (codon 891 TCGO GCG) in SC, his mother and his twin brother MC. This Case report: prophylactic thyroidectomy for increasing calcitonin mutation was not known to be adisease marker at that time (though is now levels? recognised as one), and careful discussion with the family was needed to explain KatieWynne, Anthony Goldstone, Fausto Palazzo, Jeannie Todd & the interpretationofthis finding. MC underwentthyroidectomy, had C-cell Karim Meeran hyperplasia and remains well with normal serum calcitonin. Other family Imperial College NHS Healthcare Trust, London, UK. members have been screened for the mutation; positive individuals have undergone thyroidectomy. This family highlights the issues which arise when a novel mutation, not yet recognised as adisease marker, is used to advise family A19-year-old man was referred to Endocrinology Clinic with aminimally raised members. Despite amultidisciplinary approach involving endocrinologist, calcitonin level.Hehad apast historyofprimary hyperparathyroidism resulting in surgeon, radiotherapist, geneticist and biochemist, optimal management of a renal colic aged 17 years, and underwent three gland parathyroidectomy aged 18 patient with araised serum calcitonin level afterthyroidectomy is not clear. SC years. Histology demonstrated asingle right lower parathyroid adenoma with has received extensive treatment without obvious benefit, whereas his mother has normal thyroidbiopsy. He has no family history of endocrine disease and is a had no further treatment and is well, but both have residual disease. Is there any non-smoker. His calcium has remained normal since with detectable PTH.Over 5 further intervention which will help these individuals? years there has been aprogressive increase in his basal calcitonin level:
Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008
Poster Presentations
Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008
P1 individuals with a46XY male karyotype are resistant to testosterone due to a Can random urinary 5-hydroxyindolacetic acid/creatinine results and defect of the androgen receptor. 24 hour urinary 5-hydoxyindolacetic acid levels predict carcinoid A16-year-old femaleofnon-consanguineous parents presented with primary amenorrhoea. There was no family history of delayedpuberty.She was of normal patient survival? 0 00 2 Simon May1 ,Robert Peaston2 &Petros Perros2 female appearance (height 5 9 ,BMI 19 kg/m ). However, both axillary and 1 University of Newcastle upon Tyne, Newcastle upon Tyne, UK; 2 Freeman pubic hair was absent and she had underdeveloped breasts (Tanner Stage 2). Hospital, Newcastle upon Tyne, UK. External genitalia appeared normal with no clitoromegaly. Her vaginal canal was 5cminlength. Biochemical screening revealed elevated levels of LH 38.4 U/l (3–16), FSH The use of 24 hurinary 5-hydroxyindolacetic acid (5-HIAA) concentrationsto 12.9 U/l (0.5–8) and testosterone 17.7 nmol/l (1–2.5) but low levels of serum detect carcinoid patientsisawell established procedure while random urinary oestradiol 138 pmol/l. Androstenedione, DHEAS, 17-OH progesterone, prolactin 5-hydroxyindolacetic acid/creatinine ratio (5-HIAA/cr) may also offer asimpler and thyroid function were all normal.Apelvic US confirmed absence of both approach in the diagnosis of carcinoid tumours. However, there is uncertainty uterus and ovaries. Subsequent MRI failed to identify any obvious gonadal tissue. regarding the abilityofthese tests to offer aprognostic indicator for patients. Chromosomalscreening confirmed a46XY karyotype. We retrospectively reviewed the initial paired urinary 5-HIAA/cr and 24 hurinary The findings were consistent with adiagnosis of complete androgen insensitivity 5-HIAA results for 176 carcinoid patients diagnosed between 2005 and 2008. The syndrome (AIS),anX-linkedrecessive disorder with an incidence of mortality and dates of death for this population were investigated and Kaplan– approximately 1of20000 births. The patient received psychological counselling Meier survival plots (0, 180, 360, 540 and 720 days) createdfor different valuesof and support from the AIS Support Group (AISSG, Registered UK Charity) and the two tests. During this time the mortality status of 137 patients were collected, has coped well with her diagnosis returningtofull time education. She has been of which 28 (20.4%) had died at the end of the study. commenced on low dose oestrogentoenhance her breast development and is For both the 24 hurinary 5-HIAA and spot urinary 5-HIAA/cr tests survival rates being treated with non-surgical pressure dilation to her vaginal canal. Surgical decreased with increasingvalues in the respective initial tests. However, in both exploration is planned to identify and remove remnant gonadal tissue. tests they did not become significant until 720 days follow up and it was only for the highest value groups. The initial 24 hurinary 5-HIAA group for values P 100 had a0.61 (CI 0.43–0.77) survival at 720 days compared to asurvival rate of 0.86 (CI 0.74–0.94) for the # 20 and 0.86 (CI 0.74–0.94) for the 21–99 valuegroups. Initial spot urinary 5-HIAA/cr patients with an initial value of P 10 had a720 day survival of 0.52 (CI 0.3–0.7) compared to a0.92 (CI 0.80–0.97) survival for P4 values # 1and asurvival rate of 0.86 (CI 0.74–0.94) for the 2–9 group. This data suggests that differing values for both 24 hurinary 5-HIAA and spot Management of aphaeochromocytoma in pregnancy 1 1,2 1 urinary 5-HIAA/cr tests may predict different survival rates at 2years follow up in Barbara McGowan ,Catherine Williamson ,Karim Meeran , 1,2 1,3 1,3 1 carcinoid patients. Anita Banerjee ,Lee Min ,Bill Fleming ,Duncan Bassett & Tricia Tan1 1 Department of Investigative Medicine, Imperial College, London, UK; 2 Institute of Reproductive and Developmental Biology, Hammersmith Hospital, London, UK; 3 Department of Surgery, Hammersmith Hospital, P2 London, UK. Acomparsion of random urinary 5-hydroxyindolacetic acid/creatinine results with 24 hour urinary 5-hydroxyindolacetic acid values in A30-year-old lady was transferred to our hospital at 37 weeks of pregnancy. carcinoid patients Hypertension was noted at 24 weeks of gestation and managed with methyldopa Simon May1 ,Robert Peaston2 &Petros Perros2 and labetalol. Despite treatment,BPwas labile with asystolic of 90–220 and 1 University of Newcastle upon Tyne, Newcastle upon Tyne, UK; 2 Freeman diastolic of 50–129 mmHg. She reportedoccasional palpitations but not chest Hospital, Newcastle upon Tyne, UK. pain or shortness of breath. The patient had ahistory of migrainous headaches but not hypertension prior to pregnancy. Her brother had previously had an operation for abenign abdominal lesion. Abdominal ultrasound demonstrated a5.2! Although 24 hurinary 5-hydroxyindolacetic acid (5-HIAA) concentration is a 5.7 cm mass superio-posteriorly to the right kidney. Twenty-four hour urine well established test for the detection and monitoring of carcinoid tumours, noradrenaline excretion was grossly elevated at 4470 nmol/24 h(non-pregnant compliance, adequacy of collection and the influence of dietary sourcesof normal range 0–560 nmol/24 h). Adrenaline and dopamine levels were normal. A serotonin are problems associated with this approach. Recently, arandom urinary diagnosis of aright-sidedphaeochromocytoma was made. On admission, 5-hydroxyindolacetic acidcreatinineratio (R/5-HIAA/cr) test hasbecome labetalol and methyldopa were stopped. She was hydrated with IV fluids and available which can be used in outpatient departments, however its correlation treated with phenoxybenzamine 0.5 mg/kg over 2–4 hfor 3consecutivedays. She to 24 h5-HIAA (24/5-HIAA) values remains uncertain. developed atachycardia and was commenced on propranolol 40 mg tds. An We retrospectively analysed 1113 paired 24 hurinary 5-HIAA and spot urinary 1 elective Caesarean section at 37 2⁄ weeks performed under continued IV alpha and 5-HIAA/cr from 176 patients (mean age 61 ( S . D .12.99, 52% male)with carcinoid beta blockade resulted in the delivery of ahealthy and normotensive baby girl. tumours diagnosed between 2005 and 2008. Intravenous phenoxybenzamine was discontinued 2days after delivery and oral Thecorrelation betweenthe two tests wasexaminedusing Pearson’s productmoment phenoxybenzamine commenced. She was discharged home 5days later on correlation co-efficient(r ). Overall arelatively strong correlation existedbetween the phenoxybenzamine 10 mg tds and propanolol 40 mg tds. Post-operatively she tests(r Z 0.79, P ! 0.0001).Whenthe results were limited to the first paired sample remained asymptomatic with lying and standing BPs of 130/70 and 115/80 fromeachpatient (toremovethe confoundingeffectofrepeatedmeasurements)the respectively. An MIBG scan 3weeks later showed avid uptake consistent with a correlation forthe overall sample ( r Z 0.83, P ! 0.0001),for men(r Z 0.87, P ! 0.0001) right-sided phaechromocytoma. Calcium and calcitonin were normal. Adrena- andfor women(r Z 0.81, P ! 0.0001)remainedstrong.Interestingly, thecorrelation lectomy was performed 5weeks post-partum. She remains normotensive after betweenthe testswas stronger forages ! 55 ( r Z 0.99, P ! 0.0001),55–64 ( r Z 0.92, withdrawal of antihypertensive medication, and catecholamineexcretion levels P ! 0.0001)and O 75 ( r Z 0.93, P ! 0.0001).However,the 65–75 grouphad areduced remained normal. Genetic testing revealed that both she and her brother carry a correlation ( r Z 0.41, P ! 0.05). Whilstthisstudiedhighlightsthe poorer correlation in germline mutationinSDH-B. the65–75 agerange it is notclear whythese results were observed. In conclusion, we believethatthere is aassociatedcorrelation between thesetwo tests andthatrandomurinary 5-HIAA/cr testscollected in the out-patients could be substitutedfor 24 hurinary 5-HIAA testsincarcinoid patients.
P5 Temporary extracorporeal jugulo-femoral venous bypass under local anaesthesia to relieve SVC obstruction prior to total thyroidectomy P3 Mary Sligo, Duncan Farquhar-Thomson, David Cove &Nicholas Lagattolla Agenetic cause for primary amenorrhoea Dorset County Hospital, Dorchester Dorset, UK. Marcus Martineau &Masud Haq Northwick Park Hospital, London, UK. The induction of general anaesthesia and initiation of positivepressure ventilation in cases of superior vena caval(SVC) obstruction carries an unacceptably high Primary Amenorrhoea is usually the result of agenetic or anatomical abnormality. risk of cerebralvenous congestion, and with it, the risk of cerebraloedema and Androgen insensitivitysyndrome (AIS)isanuncommoncause in which death. Two similar cases have been dealt with successfully in our unit: both had
Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008 thyrotoxic retrosternalmultinodular goitres and SVC obstruction, and both had diagnostic for phaeochromocytoma. In conclusion, this case highlights the tracheal compression mandating surgery. importance of the clinical history when making adiagnosis as biochemical and Followingcontrol of the hyperthyroidism by our medical colleagues (without functional testingare not 100% reliable. We recommend that with aclinical improvement of the symptoms of compression), the patients were submitted to historytypical forphaeochromocytoma despitelackofbiochemical and surgery. The patients were fully prepared for thyroidectomy with additional functional evidence, alpha-blockade should be considered prior to surgery. exposureoftheir chest, abdomen and groins. Under local anaesthesia, the left internal jugular vein andthe left sapheno-femoral junctionwereexposed and controlled. After full heparinisation, an 8mmdiameter armoured PTFE graft was anastomosed to each using 5/0 prolene sutures, the graft remaining extracorporeal. Duplex was used to insonate the ipselateral common femoral vein P9 before and after clamp removal confirming considerably augmented venous return via the graft. Challenges in the management of Cushing’s syndromeinthe severely ill With the SVC obstruction thus considerably relieved, we enabled safe induction of patient 1 1,2 1 1 general anaesthesia and positivepressure ventilation. Both patientsunderwent AECFountain ,BMCMcGowan ,OChaudhuri ,SSaha , 1,2 1 1 1 1,2 uneventful total thyroidectomy though cervical incisionsalone, with minimal BCTField ,WDhillo ,JFTodd ,APGoldstone ,NMMartin , 1,2 1 blood loss. The grafts were removed at the end of the procedure. Both patients KMeeran &TTan 1 2 recovered without mishap with compression symptoms and signs abolished. Hammersmith Hospital, London, UK; Charing Cross Hospital, London, We recommend this simple technique over the insertion of endovenous stents, UK. which can be difficult to place satisfactorily in the presence of massive compressing goitres,inthe situationofSVC obstruction caused by retrosternal goitres requiring We present a57-year-old female with Cushing’s syndrome characterised by new surgery. type 2diabetes, hypertension,weightgain, bruising,proximalmyopathyand depression. Shealsohad poorly-healing cellulitic ulcers on both legs. Investigations: hypokalaemiaand ACTH-dependentCushing’s syndrome.Low dose dexametha- sone suppression test: T Z 0ACTH85ng/l, cortisol 907nmol/l, T Z 48 hcortisol 807. High dose dexamethasonesuppression test failed to suppress cortisol ! 50% P6 baseline,with T Z 0cortisol 1372 and T Z 48 hcortisol825.MRI pituitary: left sided microadenoma. CT chest,abdomen and pelvis:nopotential ectopicsourceofACTH. In view of significant hypokalaemia,and failuretosuppress cortisol during a Thisabstract appears as OC4. HDDST,anectopicACTHsourceremained adifferentialdiagnosis to Cushing’s disease. Inferior petrosalsinus sampling was notpossiblebecause shewas too unwell:1.Psychiatric problems:paranoia,agitation,obsessive-compulsive behaviour, anorexiaand severe.2.Sepsis(cellulitis andperforatedsigmoid diverticulum):requiring laparatomy,which in turn wascomplicatedbywound dehiscence.3.Inadequate controlofcortisol production despitemaximal ketoconazoleand metyrapone treatment,cortisol levelsremained O 1000 nmol/l. Sheneededcontinuous intravenouspotassium replacement. We turned to subhyponotic etomidateadministered on ITU. Initiallythiswas successful in P7 reducingserum cortisol to around 200nmol/lbut,within2weeks, hercortisol production escapedfrometomidate control (5 mg/h). We added ketoconazoletothe etomidate with stillinadequate control of cortisol (approximately 500nmol/l). Thisabstract appears as OC6. Bilateral adrenalectomy as an emergency treatment wasconsideredbut it was felt that herpreviousperitonitis andimpaired woundhealing would jeopardize it’s success.Itwas decided to performtrans-sphenoidal hypophysectomy despite the lack of adefinitive diagnosis. Histologyconfirmedacorticotrophadenoma.Serum cortisol level offhydrocortisoneinthe earlypost-operative period was168 nmol/l. This case highlights thefollowing points:A.The useofetomidateasaninhibitor of steroidogenesis, and itsplace in themanagementofCushing’s syndrome.B.The P8 timing of surgery for Cushing’s syndrome, in particular how long should Clinical diagnosis of phaeochromocytoma leads to correct perioperative biochemical controlofhypercortisolismbemaintainedbeforesurgery?C.The managementdespite negative biochemical and functional investigations inaccuracyofthe high dose dexamethasonesuppressiontest. Annabel Fountain1 ,Jeannie Todd1 ,Karim Meeran1 ,Fausto Palazzo1 & Hypophysectomydespite thelackofadefinitive diagnosis. Histologyconfirmeda Stephen Robinson2 corticotrophadenoma.Serum cortisol level offhydrocortisoneinthe earlypost- 1 Hammersmith Hospital, London, UK; 2 St Mary’s Hospital, London, UK. operativeperiodwas 168nmol/l. Thepresentation will discuss thefollowing points: A. Theuse of etomidate as an inhibitorofsteroidogenesis,and itsplace in the We present a44-year-old female referred to us with atwo year history of episodic management of Cushing’s syndrome. palpitations, chest tightness, headaches and pallor associatedwith hypertension. B. Theindicationand timing of surgery forCushing’s syndrome,inparticularhow Shehad previously been extensivelyinvestigatedbyneurologistsand long shouldbiochemical controlofhypercortisolismbemaintainedbefore surgery? cardiologists including MRI of the brain, renal ultrasonography, 24 htape and C. TheMentalCapacityAct andhow this legislation affectsthe managementofill echocardiography –all normal.Given the history, aCTofthe adrenalswas patients with neuropsychiatricmanifestationsofCushing’s syndrome. performed in 2007 which revealed a1.5 cm nodule in the right adrenal with abnormal enhancement. The left adrenal was normal.Several 24-h urine collections for catecholamines were done to investigate the possibility of phaeochromocytoma and revealed normal levels apart from aborderline urinary adrenaline on one occasion. Twenty-four hours urinary free cortisol and overnight dexamethasone suppressiontests were also normal. As she had persistent and P10 troubling symptoms, CT scan was repeated in February 2008 which showed that Positive correlation between radioisotope and CT/MRI imaging the adrenal nodule had increasedto2.2 cm over the preceding 9months. MIBG techniques in functioningadrenal adenomas may obviate the need scan was performed to assess activity and showed no abnormaluptake. MRI in for invasive adrenal vein sampling May 2008 confirmed abnormal enhancement and the nodule was now 3cmin Annabel Fountain, Vladimir Vaks &Alison Wren diameter. With the mass enlarging and the symptomatology it was elected that Chelsea &Westminster Hospital, London, UK. this lady should undergo right laproscopic adrenalectomy.Despite the lack of positivebiochemical and functional imaging findings for aphaeochromocytoma, the history led us to recommend prior alpha-blockadeinthe form of oral A48-year-oldAfro-Caribbean femalewas referred from Accident &Emergency in phenoxybenzamine and atenolol and perioperative intravenous phenoxybenza- December 2007 with low serum potassium. She had been diagnosed elsewhere mine. This lady underwent right adrenalectomy on 27th May 2008. Intraopera- with hypertension due to primary aldosteronism at the age of 28 but was then lost to tively it was noted by the anaesthetist that her blood pressurewas labile when the Endocrine follow-up. She reported intolerance to spironolactone –blood pressure tumour was being handled.Histology showed awell-circumscribed, encapsu- was controlled with Amiloride until 1999 and then Amlodipine. Questioningin lated, nodularcentral lesion which stains positively forchromagranin, clinic, January 2008, revealed that she had had extensive investigations when synaptophysinand PGP9.5,negative forinhibin andMelan-A,therefore previously assessed includingCT, MRIand radioisotope scans, but these were
Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008 unavailable. In March 2008, she complained of tiredness and weight gain, easy hump, truncal obesity, paper thin skin with bruising and proximal musclewasting. bruising and oligomenorrhoea over the preceding year. Investigations 2008: His biochemistry showed elevated midnight cortisol levels, no suppression of aldosterone:renin ratioO 4200. MRI abdomen:3cm right adrenal adenoma, cortisol post high dose dexamethasone and an undetectable ACTH. Accompanying Iodocholesterol scan:increased tracer uptake in the region of the upper pole of the abnormalities included low gonadotrophin levels, persistent hypokalaemia and rightkidneywith no traceruptakeofthe left adrenal consistent with derangement of the liver enzymes. ACTscan of his chest, abdomen and pelvis hyperfunctioning right adrenal adenoma. Low dose dexamethasone suppression showed two masses in the right adrenal measuring 7.2! 6.2! 5.7 cm and 2.8 cm in test (LDDST): T Z 0304 nmol/l, T Z C 24 251 nmol/l, T Z C 48 232 nmol/l with diameter respectively. There was no evidence of surrounding structure invasion but suppressed ACTH! 5ng/l, supportive of adrenal Cushing’s syndrome. Twenty- considerableintraabdominal and intrahepatic fat was noted. The diagnosis of four hours urinary cortisols were raised: 328 and 461 nmol/l, 24 hurinary ACTH independentCushing’s syndrome secondary to adrenal tumours was made. catecholamines were normal. It was recommended that the 3cmadenoma be The patient was initially treated with Ketoconazole but due to further deterioration removed surgically. The gold standard investigation for lateralising an adrenal of his liver function was switchedtoMetyrapone which was titrated to suppress source of aldosterone or cortisol is adrenal vein sampling (AVS). However, as the cortisol levelsfor 6weeks. Oral hypoglycaemic agents and potassium replacement iodocholesterol scan showed suppression of the contralateral adrenal gland, it was were also added to the treatment regime. Alaparoscopicadrenalectomy was extremely unlikely that the source of aldosterone and cortisol was the left adrenal. attempted but due to the size of the adrenal masses the procedure was converted to The patient was not submitted for (AVS) as she was extremely anxious and did not an open one. The right adrenal was successfully excised and there was no wish to undergo invasive testing. She underwent right adrenalectomy 23.09.2008, intraoperativeevidence of surrounding structure invasion. Three weeks post with steroid cover. Histology confirmed abenign adrenal corticaladenoma.She operatively the doses of his antihypertensives were reduced,the oral hypogly- was discharged on hydrocortisone 10, 5, and 5mg, fludrocortisone 50 mcg daily caemic agents stopped and he had normal cortisol responses.Histology of the and amlodipine 10 mg od. She no longer requires potassium replacement. She will excised mass was compatible with adrenal cortical carcinoma. He was started on undergo Short SynACTHen test on 22.10.2008 to establish whether she can Mitotane. His prognosis is expected to be good in the absence of metastatic spread discontinue her hydrocortisone therapy. If she passes this, she will have aLDDST which is found at presentation in 75% of patientswith adrenal carcinoma. to confirm cure of her Cushing’s syndrome. This case illustrates that, although adrenal vein samplingisthe gold standard, the presence of localising imaging of more than one modalitymay be sufficient to proceed to surgery.
P13 Unusual hypoglycaemia: real or factitious? Sanjeev Sharma, Francesca Swords &Nicoletta Dozio P11 Norfolk &Norwich University Hospital, Norwich, UK. Treatment resistant acromegaly Paul Grant Royal Sussex County Hospital, Brighton, UK. Factitious hypoglycaemia is characterised by high insulin levelsbut accompanied with low Proinsulin and C-peptide levels and anegative sulfonylurea screen We presenta54-year-old woman who was initially diagnosed with type 2 The issue of treatment-resistant pituitary tumour growth remains relatively under- diabetes in 2002 and by 2004, she was converted to insulin treatment due to poor explored. We describe the case of agentleman whose diagnosisand management tolerance to Metformin. She also had aprevious history of Manic depressive involved several medical disciplines and his management proved challenging psychosis and treated with lithium. over the long term. In the months prior to her admission, due to persistent hypoglycaemic episodes, A47-year-old gentleman who was seen by avariety of specialists before and after her insulin requirement had to be dramatically reduced from atotal of 60 to diagnosis for the complications of his aggressive acromegaly. 15 units/day. Initially seen in 1997 by an Occupational Health Physician as he complained of Shewas admittedwithrepeated, severe episodes of neuroglycopenia, andafter enlargement of both hands making his workdifficult. He was subsequently stopping allforms of insulin,was monitored as an in-patient. Her4-hourlyblood referred on to aUrologist because of erectile dysfunction. The urologist queried glucose level remained between 3and 7mmol/land wasinterspersedwithfurther the diagnosis of diabetes and arranged for him to have an OGTT and see a hypoglycaemic episodes.Duringone such episode, her bloodglucose wasaslow as Diabetic nurse specialist. The DSN found that he had impaired glucose tolerance 1.4mmol/lbut herconcomitantplasmainsulin levelswas ! 2pmol/l(Perkin Elmer and gave diet and lifestyle advice. insulinimmunoassay; normalZ 0–60 pmol/l)and C-peptide was46pmol/l(174– He was subsequently reviewed by his General Practitioner who thought that he 960pmol/l).Urinary screenfor sulfonylureawas negative. Insulinomainvestigated may have an underlying endocrinopathy and referred him on to see aCardiologist with adynamic CT scan of thepancreasand IFG-2:IGF-1ratio 2.2(normal: ! 10). for features of heart failure, and. Shebafflingly continued to have dramaticand sustained hypoglycaemiadespite not An endocrinologist who diagnosed him with Acromegaly.Hewas found to have a receivingany insulin. At asubsequenthypoglycaemic episodewithblood glucose of large pituitary macro-adenoma which had eroded the pituitary fossa and cavernous 1.2mmol/l, standardmethods didnot detectany insulin butanalternative assay sinus. method (Delfia insulin assay) showedinsulin Aspartlevels of O 200pmol/l. He was seen by aNeurosurgeon who performed two trans-sphenoidal debulking Subsequent follow up revealedthatshe had been working at thesamementalcare operations with mixed results. He went on to have Radiotherapy administered by home,where-inshe previouslywas apatient.Her hypoglycaemicsymptomshave Nuclear Medicine. nowceasedand she is doingwellonMetformin alone.Atthismodernage of insulin Following thesetreatmentshewas stillfound to have active diseasewithelevated analogues,thisfascinating story reminds us theneedofawareness aboutthe IGF-1, despite medicalmanagementwithOctreotide. Thepatient subsequently complexities of insulinassays, andtheir importance in diagnosing factitious declinedfurther radiotherapy duetoconcernsabout cognitivedecline and histumour hypoglycaemia. was notfelttobeamenabletofurther neurosurgery.Heisdue to be consideredfor treatment with Pegvisomant (a growthhormonereceptorantagonist). Thereexistsaclearneedtostudy thecausesand management of treatment-resistant Acromegaly,particularly in terms of tumour progression. Well-designedMRI studiesinpatientspotentially at riskfromanaggressivetumourwould be very useful to verifythe true rates of therapeutic failureacrossall treatment modalities. P14
This abstractappears as OC7.
P12 Cushing’s syndromesecondary to adrenocortical carcinoma without metastases at diagnosis Alexander Miras1,2,Fausto Palazzo1,2 &Safdar Naqvi1,2 P15 1 St Peter’s Hospital, Chertsey, Surrey, UK; 2 Hammersmith Hospital, An unusual case of agland in the neck London, UK. Tee Wei Siah, Binu Krishnan, Emma Bingham &Jennifer Tringham Frimley Park Hospital, Surrey, UK. A60-year-old gentleman of previously good health presented with a6months historyofresistanthypertension, newly diagnosed Diabetes Mellitus, worsening A23-year-oldman initially presented to his GP with palpable‘glandinhis neck’ for obesityand proximal muscle weakness. Clinical examination revealed abuffalo 9years. He was otherwise well with no medical problems. He has afamily history
Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008 of hypothyroidism. Examination revealed right submandibular gland enlargement 20 years old and underwent trans-frontal surgeries and radiotherapy twice and and lymph nodes in the anterior cervical region bilaterally. His TPO antibody was further transsphenoidal surgery in 1980. She was on prednisolone 2mg C 1mg negative and the only abnormalresult was araised TSH of 12.7. He was and thyroxine 50 mcg daily. commenced on 50 m gofthyroxine daily. Ultrasound of the neck showed no normal She initially presented to the ophthalmology clinic. Examination revealed apupil- thyroidglandand an abnormal oval lesion beneath the strap muscles underlyingthe sparing 3rd nerve palsy of the right eye, with complete ptosis, proptosis and normal right submandibular gland. This was confirmed on CT scan, which showed reduced visual acuity (6/9). She was referred to the medical team.CThead another swelling at the base of the tongue. Technetium Thyroid scan showed demonstrated a2! 1.5 cm homogeneoussoft tissue mass in the medial aspect of increased uptake in the region of the right submandibulargland and at the base of the right orbit. MRI revealed awell-circumscribed mass arising within the the tongue with absence of uptake in the usual region of the thyroid. This confirmed anterior ethmoid air cells extending to the right frontal sinus. The pituitary fossa the presenceofectopic thyroid tissue. Ectopic thyroid tissue has been reported to be was enlargedbut empty.IGF-1,prolactin and free T4 levels were normal. A found at the foramen caecum and along the thyroglossal duct, where its localization multidisciplinary discussion involving radiologists, ophthalmologists, endocri- can be explained by the embryogenesis of the thyroid gland. The presence of nologists andotolaryngologistsconcludedthatthe MRIappearancewas thyroidtissue outside this pathway has been reported in the submandibular region, suggestive of afrontalethmoid mucocele. Evacuation of the right frontal parotid gland, gallbladder, skin, liver, trachea, mediastinum,heart, lung, mucocele was performed by the otolaryngologists via endoscopic approach with duodenum, iris, and adrenal glands and these cannotbeexplained readily by antibiotics and steroid cover. She had complete resolution of the diplopia, ptosis embryogenesis. It has been suggested that the possibilities for the presence of and proptosis and visual acuity improved (6/6) post-operatively. Unfortunately, thyroidtissue in the submandibular regioninclude abnormal migration during the she had uncontrolled atrial fibrillation post-operatively and apositive troponin Iof course of embryonal development, spread of tissue during surgery on anormally 0.09 m g/l ( ! 0.04 m g/l). She was treated as acute coronary syndrome and located thyroidgland and metastasis of thyroid carcinoma. Malignancy in this subsequent coronary angiography did not show any significant coronary artery patient is unlikely because of anegativefamily history for thyroidcancer and the disease. She was dischargedwithout further complication. young age of the patient. This case represents an unusual description of thyroid Mucocelesare slow-expanding cystic lesionswithrespiratoryepithelium tissue at right submandibular region and foramen caecum. containing mucus caused by obstruction of sinus ostium1 .They can extend intraorbitally and intracranially2 .Mucocele formation is arare complication of transsphenoidal surgery. There is no reported case following trans-frontal surgery. We reported acase of frontal ethmoid mucocele presented 50 yearsafter trans-frontal surgery to apituitary adenoma. P16 Reference 1. EvansC.Aetiology and treatment of frontoethmoidal mucocele. JLaryngol Primary hyperparathyroidism and pregnancy 1 2 1 Otol 1981 95 361–375. James Kirkby-Bott ,Catherine Williamson ,Fausto Palazzo , 2. Lai PC, Liao SL, Jou JR &Hou PK. Transcaruncular approachfor the Anita Banerjee1 ,Karim Meeran1 &Tricia Tan1 1 2 management of frontoethmoid mucoceles. Br JOphthalmol 2003 87 699–703. Hammersmith Hospital, London, UK; Queen Charlotte and Chelsea doi: 10.1136/bjo.87.6.699. Hospital, London, UK.
Primary hyperparathyroidism (HPT)inpregnancy is an uncommon phenomenon, mostly occurring in the 2nd or 3rd trimester. HPT in pregnancy may cause complications affecting both the mother (renal stones, pancreatitis) and fetus (neonatal tetany, seizures, intra-uterine growth retardation and preterm labour). We report two recent cases that highlight the potential risks. P18 Results Thepatients, 37 and35years oldatpresentationrespectively, both presented with symptomatichypercalcaemia. Diagnosiswas proven biochemically.One had This abstract appears as OC1. undergone localisation studiesatanother centre pre-pregnancy andfallenpregnant during work-up. Patient1onlyhad pre-op ultrasoundlocalisationshowing an enlargedright inferior gland. Patient2whopresented pre-pregnancy hadhad anegativesestaMIBI performed at anothercentreand an ultrasound showing twolikelyadenomas. At surgery 5gland hyperplasiawas found.Patient 1underwent minimallyinvasive parathyroidectomy and a4.8 gadenoma was removed. She has persistent hyperparathyroidism and re-localisation post partum localises aleftinferiorgland. She is awaiting abilateral re-exploration.Bothpatientsare undergoinggenetic P19 screening.Bothwentontohaveuncomplicateddeliveriesand healthy babies. Conclusions Young hyperparathyroidism: to localize or not HPTinpregnancytypically affects ayoung agegroup where multiplegland disease Sarah Skennerton, George Tharakan, Niamh Martin, Karim Meeran & and inherited syndromesare morecommon. Localisation studieswithUSS without Daniel Morganstein sestaMIBImay be misleading.Given thelimitations of localisation,abilateral Imperial College Healthcare NHS Trust, London, UK. exploration in thesecondtrimester C / K use of intra-operative PTHassay is advisable.Young women being investigated forhypercalcaemiashouldbeadvised A37-year-old lady was referred to the endocrine clinic with araised serum to avoidpregnancy until fully investigatedand treated. An MDTwith calcium of 2.73 mmol/l in the presence of an inappropriately raised parathyroid endocrinologists, endocrine surgeons, obstetric physicians, radiologists and hormone of 8.7 pmol/l. Calcium:creatinine clearance ratio was elevated at 0.018, geneticistsisrequired foroptimal management. excluding familial hypercalcaemic hypocalciuria. Past medical history revealed an episode of renal stones 17 years ago but arenal ultrasound excluded nephrocalcinosis. Adiagnosisofprimary hyperparathyroidism was made. Sporadic hyperparathyroidism is due to asingle adenomainaround 80% of cases so pre-operative localization studiesare frequently performedtoenable P17 minimally invasive approaches. However the patient’s young age of onset raises Acase of frontal ethmoid sinus mucocele: alate complication of the possibility of an inherited predisposition, for example MEN 1or2. pituitary surgery? Parathyroid glandular involvement is more diffuse in MEN than in sporadic Elaine Hui1 ,Anjali Amin1 ,Sandro Chiti-Batelli2 ,Pooja Patel3 ,Nick Linton4 hyperparathyroidism and removal of all four glands is therefore the preferred &Jonathan Valabhji1 option. Patientsare however renderedpermanently hypoparathyroidand 1 Department of Metabolic Medicine, St Mary’s Hospital, Imperial College dependent on lifelong alphacalcidol with calciumsupplements. Therefore the Healthcare NHS Trust, London, UK; 2 Department of Otolaryngology-Head- patient was referred for genetic and biochemical screening for MEN, as apositive Neck Surgery, St Mary’s Hospital, London, UK; 3 Department of Neurology, result would dramatically alter the surgical management and render pre-operative St Mary’s Hospital, London, UK; 4 Department of Cardiology, St Mary’s localization redundant. Hospital, London, UK. This case study highlights the importance of considering MEN1or2syndromes in young patients presenting with primary hyperparathyroidism prior to localization studies or surgery. A69-year-old Iraqi lady presented with a4-day history of diplopia,headacheand right eye swelling. She had apituitary adenoma diagnosed in Beirut when shewas
Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008
P20 endocrinology out-patients. At out-patient review, it was noted that the baseline Lymphocytic hypophysitis secondary to aruptured Rathke’s cleft cyst ACTH from his earlier SST was elevated at 52.0 ng/l suggesting subclinical (RCC): adiagnostic and managementchallenge hypocortisolaemia. The dose of his hydrocortisone was increasedto15mg, Puja Mehta1 ,Frederico Roncaroli2 ,Amrish Mehta2 ,Maneesh Bhojak2 , 10 mg, 5mg, as he is about to start further immunotherapy (Bevacizmab). We JamesLawrence3 ,Emma Hatfield2 ,Karim Meeran1 &Waljit Dhillo2 present acase of what we believe to be adrenal toxicity due to Sunitinib, with 1 Hammersmith Hospital, London, UK; 2 Charing Cross Hospital, London, clinical findings and investigations suggesting predominantly mineralocorticoid UK; 3 Salisbury District Hospital, Salisbury, UK. deficiency. Adrenal toxicity with Sunitinib has been reported previously in animals but not humans. Such subclinical toxicity may be difficult to detect unless unmasked by physiological stressors. We highlight the importance of knowing Hypophysitis describes inflammatory pituitary lesions which can be classified both abaselineACTH and peak cortisol when interpreting aSST, and also the into lymphocytic (autoimmune), granulomatous or xanthomatous. Rathke’s cleft difficulty in determining what is an appropriate random cortisol in an acutely cysts (RCC) are usuallyassymptomatic, benign tumours derived from remnants unwell patient. Another potential explanation for the discordance between of Rathke’s pouch. We present acase of lymphocytic hypophysitis secondary to a cortisol results and his clinicalstate is abnormal cortisol binding globulinlevels, ruptured RCC and the first reported case of post-surgical recurrence. which must be considered. A34-year-oldfemale presented with secondary amenorrhoea and fatigue. There were no features of infection, autoimmunity or granulomatousdisease. Investigations revealed panhypopituitarism and she was prescribed replacement corticosteroids,thyroxineand HRT. Shesubsequently developed diabetes insipidus and was commenced on desmopressin. MRIscans showed expansion of aperipherally enhancing pituitary mass abutting the optic chiasm and transsphenoidalhypophysectomywas performed.Post-operativeimaging P23 demonstrated initialtumour debulkingand then re-growth at 14 months with The need for acoordinated multidisciplinary approach in the chiasmal compression, requiringasecond hypophysectomy. Review of the management of morbid obesity histology confirmed alymphocytic hypophysitis with underlying ruptured RCC. Leena Mukherjee, Sufyan Hussain, John Flood &Rashmi Kaushal Her most recentMRI scan (7 months after the second hypophysectomy) West Middlesex University Hospital,London, UK. demonstrated further expansion of the pituitary mass, and atrial of high-dose prednisolone has been commenced, with repeat MRI planned to assess response. If there is asub-optimal response, we will considerfurther surgery and/or We describe the case of a22-year-old South Asian male presenting to Endocrine radiotherapy. Services with morbid obesity (BMIO 50), sexual immaturity and agoraphobia. The contents of aruptured RCC is thought to drive asterile inflammatory or Born at full term following an uncomplicated pregnancy, he reached all autoimmune process. There have been nine cases in theliteratureof developmental milestones appropriately. His problems began aged 15 after hypophysitis associated with aruptured RCC, only three of these casesinvolved witnessing amurder, with reactivedepression and hyperphagia. By the age of 18, lymphocytic infiltrates. We describe the fourth reported case, who required he weighed over 140 kg, had poor self-image and suicidal ideation. He was multidisciplinary team management. Our patient is the first case to exhibit managed initially by several multidisciplinary teams, including the Eating recurrence after surgical debulking. Treatment options include high dose Disorders Psychologists, Psychiatrists and Dieticians, although the emphasis was steroids, surgery and radiotherapy, although there is no consensus in the on onlyhis psychiatric conditionwithlittle communicationbetweenthe literature regarding management. specialties. On referral to the Endocrine Service, he was clearly hypogonadal with aBMI of 64. Investigations confirmed hypogonadotrophic hypogonadism, (LH 1IU/l, FSH 5IU/l, Testosterone 0.6 nmol/l) with anormal karyotype. The remainder of his pituitary and adrenal function was normal. An MRI of the pituitary was also normal. His serumleptinwas 63.4 ng/ml, insulin116 pmol/l,pro-insulin 12 pmol/l and split pro-insulin 10 pmol/l. Despite being known to several multidisciplinary teamsfor his co-morbidities, his P21 weight continued to soar. He developed severe sleep apnoea, hypertension, arthritis and finally turned to heavy alcohol consumption. It became clearthatthe only wayforward wastoestablish organised This abstract appears as OC2. communication between the teams. With diabeticeducation and anti-obesity medication he was able to lose 20 kg in weight.Hewas then referred to atertiary Obesity Clinic weighing 177.6 kg (BMI 69). With organisedcontribution from all the specialties involved,agastric banding procedure was eventually performed, resulting in a12-month weight loss of 40.6 kg (BMI 53.5). At 28 years of age, his management is currently shared by Endocrinology and Gastrointestinal Surgery. Plans for an jejunoileal bypass have been agreed with support from the Morbid Obesity team consisting of aDietician, Psychologist, Consultant Physician, Psychiatrist and Fitness Instructor. P22 Primary adrenocortical insufficiency despite a‘normal’ short synacthen test SRMehta, BCTField, OBChaudhri, HShaikh, DLMorganstein, N MMartin, ECIHatfield &KMeeran Endocrine Unit, Department of Investigative Medicine, Imperial College P24 Healthcare NHS Trust, London, UK. Surveillance of aslowly progressive non-benign adrenal incidentaloma Debbie-Ann Charles, Yuk-Fun Liu, Audrey Jaques &Paul Carroll Guy’s and St Thomas’ Hospital NHS Foundation Trust, London, UK. A60-year-old gentleman who had previouslyundergone aright nephrectomy for renalcell carcinoma was admitted electively for aleft adrenalectomy due to metastatic disease. Prior to this he had been treated with immunotherapy Adrenal incidentalomasare tumours of the adrenal incidentally discovered during (Sunitinib) and radiotherapy for pulmonary and bony metastases respectively. He evaluation for non-adrenal disease. The National Institute of Health consensus was given perioperative cover with hydrocortisone. Ashort synacthen test (SST) definition usually excludes lesions found during evaluation for malignancy.We performed the morning after discontinuinghydrocortisone showed abaseline presentacase of an initially non-secretoryadrenal incidentalomathat cortisol of 406 nmol/l rising to 469 nmol/l at 30 min and 555 nmol/l at 60 min. He progressively increased in size over 6years and became hormonally active. was clinically euadrenal, so it was decidedthat he did not requirefurther A78-year-oldhypertensive male was found to have aright adrenal mass during hydrocortisone. He was discharged home 3days later. Three weeks later, he investigation for arectalcarcinoma. He underwent asuccessful anterio-posterior presented with profound nausea and tiredness. On examination he had postural resection requiring no adjuvanttherapy. Initial surveillance under the surgeons hypotension. Plasma sodium was 129 mmol/l, potassium4.4 mmol/l and random involved 6monthly abdominal computed tomograms (CT). Over a2year period cortisol 689 nmol/l.Hewas started on hydrocortisone 20 mg, 10 mg, 10 mg and the mass increased in size from 2to3cm in longest diameter. Neither unenhanced fludrocortisone 100 mcg once daily. His symptomsresolved over the next 48 h, CT nor chemical shift MRI (CSI) was compatible with abenign adenoma. He was and the hydrocortisone was reduced to 10 mg, 5mg, 5mgprior to review in therefore referred to endocrinology.
Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008
He was asymptomatic and normotensive on amlodipine and doxazosin. Initial Results hormonal evaluation showed that his cortisol circadian rhythm was preserved, The diagnosisofprimary hyperparathyroidism (HPT) was established using 48 hdexamethasone suppression test suppressed to 59 nmol/l and electrolytes standard departmental protocols. In all three cases the glands were seen on MIBI and aldosterone:renin ratio was normal.Urinary catecholamines were not and CT and localised to amediastinal compartment. At surgery removal of the elevated. Over the next 4years, he remained completely asymptomatic. Follow- thymus and all other fatty tissue in the superior/anterior mediastinum was needed. up imaging demonstrated awellcircumscribedmassthatslowly but Frozen section was used to confirm the presence of parathyroid tissue. In all cases progressively attained asize of 3.4! 2.9 cm with anecrotic centre. There was parathyroid tissue was demonstrated on permanentsection histology and serum no loss of signal intensity on CSI. Periodic hormonal evaluation showed normal calcium and PTH levels promptly returned to within the normal range. All cortisol, androgens and aldosterone production. At his 6year follow-up urinary patients made swift recovery with little analgesic requirement. catecholamines and metanephrines were elevated. MIBG showed persistent Conclusion uptake in the right adrenal.Adrenalectomy was performed and histology Although case reports of mediastinoscopic and thoracoscopic removal exist confirmed aphaeochromocytoma. Unless there is clearradiological confidence careful and meticulous dissection of the mediastinal compartments is needed to that abenign adenoma is present surveillance and re-assessment of functionality ensure removal of all parathyroid tissue. Traditionally this has been performed is warranted. using amedian sternotomy,but manubriotomy appears to provide asafe curative approach with ashort recovery.
P25 Earlyreport of ultrasoundvocal cord assessment for patients P27 undergoingcervicotomy James Kirkby-Bott, Amanda Mortier, Gavin Royle &KDewbury Unusual case of hyperpigmentation Southampton University Hospitals Trust, Southampton, UK. Heba El-Gayar &Owais Chaudhri Imperial College, London, UK.
Introduction The majority of dedicated endocrine surgery centres in the UK and abroad Cutaneouspigmentation results fromthe synthesisofmelanin by the routinelyperform pre-operative vocalcordchecksprior to cervicotomy. melanocytes. Its distribution patterninthe surrounding keratinocytes determines Ultrasound scanning is increasingly used as routine imaging in the work up of the actualcolour of the skin. ACTH and a -MSH are equipotent at the patients being assessed with thyroid and parathyroid disease. We report on the melanocortin-1 receptor (MC-1R) that is expressed on the cell surface of additional use of USS as amethodofperforming pre-operative vocal cord checks. melanocytes. Activation of these receptors stimulates both proliferation of Method melanocytes and melanin synthesis. We report the early results on 32 patients undergoing USS as an assessment of We present the case of a66-year-oldlady. Originally from Ghana, she has lived in thyroidstatus,USS guided FNAC and assessment of lymph node status prior to the UK for over 40 years. She presented with an 18 months history of generalised thyroidectomy. In addition to this all patients had vocal cord function assessed. hyperpigmentation. Cortisol day curve and arandom ACTH were both normal. A Whilstinthe supine position the vocal folds were assessed during quiet skin biopsy showed anormal number of melanocytes but with hyperpigmentation respiration and phonation. The patientsthen underwentnaso-endoscopyto of the dermis and epidermis. Levelsof a -MSH levels were undetectable in the validatethe USS vocal cord findings. plasma. MRI pituitary was normal. Imaging revealed an octreotide avid lesion Results within the mid zone of the left lung. Unfortunately CT thorax followingthe Thirty-two patients (26 female 6male) underwent thyroidsurgery. None had octreotide scan did not show any corresponding lesion. thyroidcancer. Thirty patients vocal folds were seen on USS to move normally; Her skin continued to darken. She went back to Ghana for aholiday where this corresponded with normal vocal cord movementonnaso-endoscopy. Two unfortunately she was shunned by her family,asthere was abeliefthat she was patients had calcified thyroid cartilage makingUSS interpretation too difficult to cursed. She is extremely desperate for acure however but is not keen on having be reliable. surgery. Conclusion Discussion USS assessment of vocal cord functionappears to be possible and feasible in the Our a -MSH assay is validated for in vitro release from hypothalamic explants. Its great majority of cases. Greater numbers are needed to confirm the accuracy of accuracy on plasmasamples is unknown. An a -MSH secreting tumour therefore USS in abnormal vocal cord movementand the proportion of patientsinwhom cannot be excluded. Other options for therapyincludeoctreotide or surgery. USS vocal cord assessment is successfullyundertaken. However these findings However, the success of therapy will be difficult to judge as any improvement in would suggest it is auseful means of assessing vocal cord function prior to this lady’s skin colour is likely to be slow. cervicotomy in most patients.
P28 P26 Acase of frontal ethmoid sinus mucocele: alate complication of Manubrial split provides adequate exposure for mediastinal exploration pituitary surgery for parathyroid adenoma Elaine Hui, Anjali Amin, Sandro Chiti-Batelli, Pooja Dassan, Nick Linton & Edward Lake, James Kirkby-Bott, Fawzia Imtiaz, James Jackson & Jonathan Valabhji Fausto Palazzo St Mary’s Hospital, London, UK. Hammersmith Hospital, London, UK. A69-year-oldlady presented with a4-day history of diplopia,headacheand right Introduction eye swelling. She had apituitary adenomadiagnosed in Beirut when she was 20 The traditional approach to mediastinal ectopic parathyroid adenomas has been years old and underwent trans-frontal surgeries and radiotherapy twice and further through amedian sternotomy. With improved localization techniques it has transsphenoidal surgery in 1980. She initially presented to the ophthalmology become possible to use less invasive approaches to access the mediastinum. We clinic. Examination revealed apupil-sparing 3rd nerve palsy of the right eye, with presentour recent experience of three cases of mediastinal parathyroid adenomas complete ptosis, proptosis and reduced visual acuity(6/9). She was referred to the successfully treated using amanubriotomy approach which provides good access medical team. CT head demonstrated a2! 1.5 cm homogeneoussoft tissue mass to the superior and anterior mediastinum. in the medial aspect of the right orbit. MRIrevealed awell-circumscribed mass Method arising within the anterior ethmoid air cells extending to the right frontal sinus. All patients had pre-operative localization with sestamibi and USS. Where MIBI The pituitary fossa was enlarged but empty. IGF-1,prolactin and free T4 levels highlighted amediastinal gland SPECT MIBI and contrast CT imaging were used were normal. Amultidisciplinary discussion involving radiologists, ophthalmol- to confirm the adenoma’s position in the superior/anteriormediastinum. ogists, endocrinologists and otolaryngologists concluded that the MRI appearance
Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008 was suggestive of afrontal ethmoid mucocele. Evacuation of the right frontal Aim mucocele was performed by the otolaryngologists via endoscopicapproach. She Population studieshave shown that basal calcitoninconcentrations are below had complete resolution of the diplopia, ptosis and protosis and visual acuity 10 ng/l in the normal population. Ten nanograms per literisused as adiagnostic improved (6/6) post-operatively. Unfortunately,she had uncontrolled atrial cut-offbymost laboratories and patients with levels higher than this are offered a fibrillation post-operatively and apositive troponin Iof0.09 m g/l ( ! 0.04 m g/l). pentagastrin stimulation test to exclude medullary thyroidcarcinoma. However, Shewas treatedasacute coronary syndromeand subsequent coronary these guidelines were adopted from studies that used the Cisbio Immunoradio- angiography did not show any significant coronary artery disease. She was metric assay for measuring calcitonin. Most laboratories have now moved to discharged without further complication. chemiluminescent assays. Our laboratory uses the Diasorin Liaison chemi- Mucocele is aslow-expanding cystic lesion withrespiratoryepithelium luminescent. We assessed the validity of continuing to use 10 ng/l as adiagnostic containing mucus caused by obstruction of sinus ostium and can extend cut-offfor our assay. intraorbitally and intracranially. It is arare complication of transsphenoidal Methods surgery. We report acase of frontalethmoid mucocele presented 50 years after Sixty-one healthy, volunteers (35femaleand 26 male)wererecruited trans-frontal surgery to apituitary adenoma. (07/Q0406/18). Age ranges were 21–64 years (meanZ 39.9) for males and 19–58 years (meanZ 30.7) for females. Asingle basal blood sample was collected into plaintubes and allowed to clot. Samples were immediately centrifuged, stored at K 20 8 Cand assayed using the Diasorin Liason Calcitonin assay. Results The manufacturers quote areference range of ! 5.5 ng/l for females and ! 18.9 ng/l for males. Our study showedthat the upper limit for males (22 ng/l) is P29 similar to the manufacturer’s range. However, the value for females (10.8 ng/l) is double the recommendation but is in keeping with observations made in our clinical practice and similar to the reference ranges quoted by manufacturers of This abstract appears as OC3. most other calcitonin assays. Two out of 35 females and 7out of 26 males had levels above 10 ng/l. Conclusion Our study revealed that 6% of femalesand 27% of normal maleshad levels greater than 10 ng/l. This suggests that adhering to athreshold of 10 ng/l for pentagastrin stimulation test will result in asignificant number of patients with normal results being subjected to apentagastrin test unnecessarily. We therefore recommend that the old diagnostic cut-offof10ng/l be reviewed.
P30 Atypical location and treatment for arare neuroendocrine tumour Heba El-Gayar, Daniel Morganstein &Karim Meeran Imperial College, London, UK.
Somatostatinomas are rare neuroendocrine tumors usuallyarising from the pancreasand duodenum.Symptoms includehyperglycemia,cholelithiasis, diarrhea and steatorrhoea. Treatment with somatostatin (SST) analogue may appear paradoxical, but can lower SST levels and improve symptoms. Case P32 A60-year-oldgentleman presented with diarrhoea, opening his bowels between 12 Primary hyperparathyroidism presenting in pregnancy and 13 times aday causing him to stop working and become depressed. Repeated Ali Abbara, Varunika Lecamwasam, Kevin Baynes, Richard Bell, fasting gut hormone profiles showed an isolated elevated SST levels. CT abdomen Thomas Kurzawinski, Mahadevan Neila,Akkib Rafique &Faris Kubba did not show any pancreaticlesions and colonoscopy was normal.Octreotide scan Ealing Hospital, London, UK. revealed awell defined lesion in the base of the right lung, confirmed on aCT thorax. Visceral angiography with calcium stimulation and samplingfrom the femoral artery revealed elevated SST in all samples ( O 400 pmol/l). SST levels A25-year-old pregnant lady presented to the accidentand emergency department taken from the right atrium and femoral artery, showed ahigher level in the arteries at 16 weeks gestation with intractable vomiting, weight loss and lethargy. She (venous 152 pmol/l,arterial 185 pmol), suggesting apulmonary source. However, reported a2months history of hyperemesis gravidarum managed in the the surgical risks of resection were thought to be high. He had atrial of Octreotide community prior to admission. therapywithout benefit. Four years later, he was treated with acombination of She had no past medical history and her only medication was of aPolish antenatal Lanreotide 30 mg every 14 days, and Octreotide 50 mcg TDS,resulting in a vitamin containing 400 IU of Vitamin D(D2).Her blood biochemistry revealed a dramatic improvement in symptoms. He now opens his bowels once or twice aday. markedly raised corrected calcium at 3.57 mmol/l(2.15–2.55), inappropriately His symptoms worsened when Octreotide was withdrawn. He also noticed two [ PTH at 17.6 pmol/l (1.6–6.9), Y Phosphate 0.7 mmol/l(0.87–1.45), [ Vitamin D days prior to the lanreotide injection be opens his bowels more frequently. 140 nmol/l (50–80). Fractional excretion of Calcium on a24hurine collection Discussion was 0.03 ( O 0.01) excluding familial hypocalciuric hypercalcaemia. There are 5subtypes of somatostatin receptors (SSTR). All five SSTRs bind to the She was treated with intravenousnormal saline (4 l/day) for 4days but natural SST. Two different SST analogues are used clinically, octreotide and unfortunately her calcium only marginally decreased to 3.17 nmol/l. She was lanreotide. These analogues bind principally to the receptor subtype 2and 5. reviewed by the obstetriciansand no foetal complications were detected. An Although most studieshave shown no major difference betweenoctreotide and ultrasound of her parathyroid glands revealed aright inferior pole parathyroid lanreotide in terms of receptor affinity or biological activity, this case illustrates adenoma 1.5 by 1cm. At 20 weeks gestation,she was referred to aspecialist that combination therapy may have clinical benefits. endocrine surgeon and underwentaminimally invasive parathyroidectomy with rapid intraoperative PTH measurement.The operationwas successful with a postoperativePTH of 3pmol/l and cCa 2.23 with no complications. Given that she has presented with hyperparathyroidism at such ayoung age, she will clearly require screening for MEN-1. The prevalence of primary hyperparathyroidism in the general population is 0.15% and aquarter of these cases occur in women of childbearing age, however there are only an estimated 200 cases reported in the literature thus far. Symptomatic hyperparathyroidism in pregnancy is ideally managed with surgery P31 in the second trimester. Regardless of whether the hyperparathyroidism is Diagnostic cut-offfor calcitonin: is 10 ng/l still valid? managed medically or surgically, the pregnancy is regarded as high risk and a Radha Ramachandran1 ,Patricia Benfield2 ,Sara White1 ,Richard Chapman2 , high degree of vigilance is indicated with aclear multidisciplinary approach. Had Karim Meeran1 ,Mandy Donaldson2 &Niamh Martin1 the ultrasoundscan been negative(sensitivity! 50%) in this lady with no clear 1 Department of Investigative Sciences, Imperial College Healthcare NHS surgical target,the management may have beenevenmore complicated, Trust, London, UK; 2 Supraregional Assay Service, Imperial College especially since sestamibiscanning is contraindicated in pregnancy. Healthcare NHS Trust, London, UK.
Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008
P33 Pituitary stalk haemorrhage Varunika Lecamwasam, Monica Whittle &Mark Edwards Hillingdon Hospital, London, UK.
We report the case of a59-year-old male who presented complaining of several weeks of tiredness, poor appetite and dizzinessonstanding. He had been treated with flucloxacillin by his GP for otitis externaone week earlier. In 1982, he was diagnosed with nasopharyngeal carcinoma in Hong Kong, which was treated by radical dissection and radiotherapy. On examination, his standing and lying blood pressure were 150/100 and 110/90 respectively. There was no mastoid or sinus tenderness. Hormonal evaluation showed afree thyroxine17.2 pmol/l (normal range 9–22.7 pmol/l), TSH 0.2 mU/l (NR 0.35–5), random cortisol ! 50 nmol/l (NRO 100), ACTH! 5ng/l, FSH 1.7 U/l (NR 3–20), LH 0.1 U/l (NR 1.2–8.6), testosterone ! 0.6 nmol/l (NR 6.1– 27.1) and aprolactin level of 474 mU/l (NR! 100). He was immediately commenced on hydrocortisone followed by thyroxinefor treatmentof hypopituitarism. MRIofthe head on admission revealed anasopharyngeal mucocoele and a haemorrhagic lesion in the pituitary stalk. He was then transferred to a Figure 2 Meningioma arising from the apex adjacenttothe sinus. neurosurgical unit where he underwent biopsy of this lesion via an endoscopic sphenoidectomy. Thisrevealednorecurrence of previousnasopharyngeal tumour. He also had awhole body CT scan which only revealed asmall nodule on the left lobe of thyroid gland. Arepeat Pituitary MRI four months later revealed the pituitary stalk haemorrhage In 2007, she complained of right facial neuropathic pain. On clinical examination had completely resolved. she had reduced sensation of the ophthalmic and maxillary divisionof5th cranial Pituitary apoplexyiscommonly due to ischaemia or necrosis for example nerve with loss of corneal reflex &loss of right tongue sensation. Repeat MRI infarction of apituitary adenoma. We believe this is the first report and MRI imaging showedanew lesion arising lateral to the cavernous sinus from the dural images of pituitary stalk haemorrhage. tail of the petrous bone (Fig. 2). She underwent an orbito-zygomatic craniotomy and excision of this lesion.Histology confirmed an atypical meningothelial meningioma with brisk mitotic activity with 80% of cells expressing progesterone receptor. Clinical learning point Hypopituitarism secondary to radiotherapy is common and well described. It is likely that this patient had radiotherapy-related meningiomas since they occurred P34 within the field of the radiotherapy beam and at an appropriate time interval. As of yet, there are no conclusive prospective studies with long term follow up Apotential complication of radiotherapy for acromegaly and adequate patient numbers to fully assess the excess risk of non-pituitary Varunika Lecamwasam, Ali Abbara, Richard Bell, Kevin Baynes & cerebral malignanciessecondary to radiotherapy. Akkib Rafique This potential late occurrence should be considered for patients who have had Ealing Hospital, London, UK. radiotherapy for pituitary disease.
Case report A40-year-old lady was diagnosed with acromegaly in 1993 after presentation with clinical symptoms. Imaging showed a1cm pituitary lesion and she underwenttransphenoidal hypophysectomy in 1993. Post-operatively she was rendered hypopituitary, but still had biochemical evidence of active acromegaly. She proceeded to externalbeam radiotherapy and was started on bromocriptine P35 medical therapy. An unusual case of primary infertility On routine review in 2006, her acromegaly was biochemically quiescent, but a Anjali Amin, Stephen Robinson &Lisa Webber surveillance pituitary MRI revealed an asymptomatic incidental 1.5 cm right St Mary’s Hospital, London, UK. parafalcine tumour (Fig. 1). This had features consistentwith ameningioma and was treated conservatively. We present acase of a29-year-oldlady who presented with subfertility. She had had a2years history of oligomenorrhoea with highly irregular menses.She had symptoms of depression and described tunnel vision. Clinically, she was euendocrine with no features of hormonal excess or deficiency. Shewas not hirsute. Visual fieldtesting demonstrated asevere left superior temporal quadrantopia. Laboratory investigations demonstrated an oestriadol level ranging between5000 and 6000 pmol/l, in the context of aFSH of 15 U/l. Her prolactin was mildly elevated at 600 mU/l. Ashort synacthen test was consistentwith pituitary adrenal failure (0 min 219, 30 min 411, 60 min 444). Her free T 4 was 10.3. AMRI of the pituitary glandrevealed alarge homogenousadenomawith chiasmal compression. Adiagnosisofapituitary adenoma secreting FSH with ovarian hyperstimulation was made. She was commenced on hydrocortisone 30mg daily. It was felt that any tissue secreting FSH should be removed through surgery in order improve her chances of fertility. She elected to have her pituitary surgery abroad. She had a successful transphenoidalresection of thepituitarytumour, whichwas complicated by apost-operative CSF leak. Followingher surgery, her FSH had dropped to 4.9 U/l with an undetectable oestradiol level.Aninsulin stress test showed good hypoglycaemia with apeak cortisol of 472 nmol/l at 90 min and peak growth hormone of 1.5 mU/l at 60 min. She did not have areturn of her menses, which was consistent with her hypopituitary status. She is still trying to conceive and is being considered for Figure1Gadolinium enhancedMRI Brain 1.5 cm right petrous right fertility treatment with GnRH treatment, however there are several factors which parafalcine tumour cavernous posteriorly to abut the anterior surface of the have made this problematic. Firstly, her BMI is over 35 and secondly her partner pons/mid-brain. lives abroad.
Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008
In summary, this is a29-year-old lady with aFSH secreting pituitary tumour, who P38 presents as an unusual cause of primary infertility. Rapid managementofhypercalcaemic crisis: amultidisciplinary approach Alero Adjene, James Donaldson &Keith Steer North West London Hospitals NHS Trust, London, UK.
A69-year-old man with longstanding ulcerative colitis presented for annual P36 review by the gastroenterologists. He complained of lower back pain for six Acase of insulin allergy: something to zinc about months and arecenthistoryofanorexia, constipation, thirst and urinary Katherine Simpson, Michael Clements, Arla Ogilvie, Sharon Carter, frequency. Chris Feben, Kapila Batta &Alan Rubin His serum calcium was 5.12 mmol/l, phosphate 1.77 mmol/l, urea 19.1 mmol/l Watford General Hospital, Watford, Hertfordshire, UK. and creatinine 252 m mol/l. He was admitted, rehydrated with 5lof intravenousnormal saline over 24 hand given pamidronate 90 mg, but remained unwell and hypercalcaemic. A50-year-old man with poorly controlled type 2diabetes mellitus on oral The endocrinologists were informed of his admission. On examination, amass hypoglycaemics was under review as adiabeticoutpatient. In April 2007, he was palpable in the right anterior cervical triangleand an urgentparathyroid developed idiopathic thrombocytopaenic purpura which, on abackground of hormone (PTH) level requested. His PTH was 192.9 pmol/l and aneck ultrasound probable diabeticnephropathy, precipitated end-stage renal failure. He was was performed that morning; this identified aheterogeneous nodule (4.1! 3.8! treated with oral steroids and was established on haemodialysis. To achieve 2.3 cm), separatetothe thyroid, thought to represent aright superior parathyroid better glycaemic control, he was converted to twice daily insulin. His treatment adenoma. The size of the nodule and the PTH level raised concerns of a dose of prednisolone was decreased to 10 mg in August 2007. In October 2007, parathyroid carcinoma. After liaising with the surgeons, he had aparathyr- he started to notice discrete firm nodulesappearing at the sites of insulin oidectomy that afternoon and required intensive care post-operatively for renal injectionand his insulin requirements increased. In December2007, he support. discontinued his prednisolone and following each insulin injection he noticed His recovery was uneventful with no significant hypocalcaemia. The pathologists alarge wheal appearing within afew minutes at the injection site. His eosinophil reported aparathyroid neoplasmofuncertain malignant potential. 9 count was noted to be high, with apeak of 12.01! 10 /l and an IgE RAST test to Following the diagnosis of parathyroid carcinoma, he is seen regularly. His insulin was strongly positive. Abiopsy of anodule was performed which calciumlevelsare within normal limits,but hisPTH remainselevated revealed dense eosinophilic infiltrates. He was subsequently reviewed by the (29.8 pmol/l at his last visit) and he is vitamin Ddeficient. diabetes team with apreliminary diagnosis of insulin allergy. Different insulin Parathyroid carcinomaisarare cause of PTH-related hypercalcaemia. preparations were tried as well as exenatide. His eosinophil counts are charted Histopathological distinction between benign and malignant parathyroid tumours and clearly demonstrate aresponse to the different diabetes treatments and is difficult. reducing steroid dose. Since the insulin types and their additives were easily Surgery is the most effective treatment in cases of severe hypercalcaemia and the determined, we were able to try alternative insulin regimes until the reactions timely collaboration betweenthe different specialties in this case was essential to stopped. From these observations,wewere able to deduce that this patient this man receiving prompt and appropriate treatment. suffers from azinc allergy, acommon additive used to form hexamers in commercial insulins.
P39 Renal artery stenosis and possible coexisting Conn’s adenoma P37 KOShaafi &SRussell An unusual case of hypercalcaemia Chase Farm Hospital, Middlesex, UK. AnjaliBala, Belinda Sandler, Felicity Kaplan &Sunil Kaniyur Lister Hospital, Stevenage, UK. A47-year-old lady presented with along history of resistanthypertension. Her GP referred her for further investigation to acardiologist who found asmaller We describe the case of a70-year-old lady presenting with marked, symptomatic right kidney on ultrasound scan and an elevated rennin level.Renal artery MRA hypercalcaemia. revealed right renal artery stenosis. On September 2008, she underwent right renal Past medical history was of insulin-treated Type 2Diabetes, macrovascular artery stenting and was advised to stop taking the antihypertensive medications disease and chronic renal impairment. (doxazosin and amlodipine). Aweek later she was admitted with headache, On admission, corrected calcium was noted to be 3.53 mmol/l with apaired PTH vomiting and severe hypertension. She was referred to our endocrine department withinnormal range (4.3 pmol/l, range 1.6–9.3). Further questioningrevealed a and on review of her notes it was clear that she had very long standing historyofweight loss but no other clinical features specifically suggestive of hypokalaemia with potassium ranging between2.5 and 3.5 mmol/lover the past 5 malignancy. There was no personal or family history of endocrinopathy, and the years. Despite recommencement of her antihypertensive (doxazosin 8mgb.d) her patient was not taking any relevantmedication. blood pressure remained difficult to control (220/124). ArepeatCTwith Doppler Aside from clinical evidence of dehydration, examination was unremarkable and flow examination post stent insertion revealed that the proximal segmentofthe in particular, there was no evidence of finger clubbing, lymphadenopathy, breast right main renal artery was patent and appeared to show contrast. However, there or abdominal masses. was no obvious contrast seen within the stent. Distal to the stent the contrast was Investigations conducted including liver functiontests, tumour markers, myeloma seen in the vessel and the right kidney showeduniform complete enhancement. screenand bone scan were normal.Aserum ACE was found to be moderately The Doppler examination showed forward diastolic flow with patentDoppler elevated at 113 IU/l (10–70) in the absence of overt respiratory, cutaneous or signal. Incidentally there was a10mmlesion in the left adrenal gland. musculoskeletal pathology. Her plasma renin activity remains high and aposturalAldosterone Renin ratios Following hydration and intravenous bisphosphonate therapy, corrected calcium were as follow: 09:19 am renin 10 aldosterone 330, 10:10 am renin 15 aldosterone remained elevated. 1210 and 12:55 pm renin 22 aldosterone 370. UE and LFT were normal as well as Although PTH was not elevated or indeedatthe upper end of normal, alack of the rest of routine blood tests. clearmalignant aetiology prompted arequestfor an ultrasoundscan of the She was recently commenced on spironolactone (in addition to doxazosin 8mg parathyroids. This displayed aright parathyroid lesion which corresponded to an b.d) titrating the dose gradually to 100 mg b.d. This has resulted in improvement area of increased uptake on subsequent Sestamibi imaging. The patient was in the BP readings. We are contemplating adrenal venous samplingfor Conn’s referred for surgery in view of symptomology and degree of hypercalcaemia. adenoma and possible MRI of the adrenal gland. Also she needs arepeat renal Surprisingly, histology showedaparathyroid adenomawithgranuloma artery MRA to rule out possible blockageofthe stent (possible 6weeks post stent suggestive of sarcoidosis, as well as an area of thyroid tissue with features of insertion). granulomatous thyroiditis, also favouringsarcoidosis. These findings correlated This is acase of possible adrenal aldosterone-secreting adenoma with coexisting with the elevated ACE and asubsequent high-resolutionCTchest which showed active renalarterystenosis in ahypertensive middle-agedwoman.The evidence of nodular interstitial disease predominantly in the mid and upper zones. concomitanceofthe two lesions was previouslyreported in the literature only The final diagnosis was therefore unexpected –sarcoidosis with granulomatous in five patients. Therefore we would like to present this interesting case to the infiltration of the parathyroid gland resulting in hypercalcaemia. ThirdHammersmith MultidisciplinaryMeeting principally to discussthe following points:
Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008
1. Is this likely to be Conn’s coexistingwith RA stenosis (is hypokalaemia is A63-year-old type 2diabetic gentleman with paranoid schizophrenia presented in feature of RA stenosis) October 2008 with systemic sepsis arising from multiple neuropathicfoot ulcers. 2. Shall we proceed with adrenal venous sampling. Foot MRIconfirmed extensive osteomyelitic change.This admission followed 3. The confounding effects on biochemistry when both conditions coexist. two previous similar episodes which culminated in surgical debridement of the ulcers and amputation of the second and third toe of his left foot. Post-operatively, as his condition improved he was unable to comply with IV antibiotics, VAC dressings andoff-loading of hisfeetdue to his schizophrenic delusions re-emerging. P40 On his admission,hetolerated IV antibiotics fleetingly during the acute phase of his illness. However as IV access became problematic, he was subsequently switched to oral Ciprofloxacin and Clindamycin. He was discussed at the Diabetic Foot MDT. It was felt that bilateral below knee amputations would be an option Thisabstract appears as OC5. which, given our patient’s lack of capacity, would need furtherdiscussion with the next of kin and his psychiatrist to ascertainthe patient’s best interest. In the interim, we continued with oral antibiotics and closely monitored his clinical state. His condition deteriorated and he was discussed in further MDT and case conference settingswith aConsultant Diabetologist, VascularSurgeon, Microbiologist, Psychiatrist, Next of Kin, Community Psychiatric Nurse and Nursing Home Manager present. It was concluded that due to this patient’s inability to tolerate IV antibiotics and his poor rehabilitation potential combined P41 with his poor quality of life and prognosis, it was in his best interest to be treated Adiabetic foot dilemma resolved through the use of the diabetic foot palliatively. To this end we withdrew antibiotics and dischargedhim to his MDT nursing home with community palliative support. KFStyles, CMBurns, HShaikh &JTurner This case highlights the vital importance of regular diabeticfoot multidisciplinary Endocrine Unit, Department of Investigative Medicine, Imperial College meetings in tailoring management to meet individual patient needs. NHS Trust, London, UK.
Endocrine Abstracts (2008) Vol 18 3rd Hammersmith Multidisciplinary Endocrine Symposium 2008
Author index (2008)
Abbara, AP32 &P34 Farquhar-Thomson, DP5 Lecamwasam, VP32, P33 Roberts, S. OC5 Adjene, AP38 Feben, Ch P36 &P34 Robinson, SP8&P35 Ahlquist, JOC7 Field, BCTP9&P22 Linton, NP17 &P28 Roncaroli, FP20 Amin, AP17, P28 &P35 Fleming, BP4 Liu, Y-FP24 Royle,GP25 Flood, JP23 Rubin, Al P36 Bala, AP37 Fountain, AECP9 Martineau, MP3 Russell, SOC5 &P39 Banerjee, AP4&P16 Fountain, AOC4, P10 Martin, NOC1, OC4, P9, Saha, SP9 Bassett, DP4 &P8 P19, P22 &P31 Batta, KP36 May, SP1, P2 Sandler,BP37 Baynes, KP32 &P34 Ghaffar,AOC2 McGowan, BMCOC4, Shaafi, KOOC5 &P39 Bell, RP32 &P34 Goldstone,AOC6 P4 &P9 Shaikh, HOC1, P22 &P41 Benfield, PP31 Goldstone,APP9 Meeran, KOC1, OC4, OC6, Sharma, SP13 Bhojak, MP20 Grant, PP11 P4, P8, P9, P16, P19, Siah, TWP15 Simpson, KP36 Bingham, EOC3 &P15 Haq, MP3 P20, P22, P30 &P31 Skennerton, SP19 Burns, CMP41 Hatfield, EOC1, OC4, P20 Mehta, AP20 Sligo, MP5 &P22 Mehta, POC4 &P20 Carroll, PP24 Steer,KP38 Hui, EP17 &P28 Mehta, SROC1 &P22 Carter,SP36 Styles, KFP41 Hussain, SP23 Min, LP4 Chapman, RP31 Miras, AP12 Swords, FP13 Charles, D-A P24 Imtiaz, FP26 Morganstein, DLOC2, Tan, TOC1, OC4, P4, Chaudhri, OBOC1, OC4, Jackson, JP26 P19, P22 &P30 P9 &P16 P22 &P27 Jaques, AP24 Mortier,AP25 Tharakan, GP19 Chaudhuri OP9 Mukherjee, LP23 Todd, JFOC2, OC4, OC6, Chiti-Batelli, SP17 &P28 Kaniyur,SP37 P8 &P9 Clements, MP36 Naqvi, SP12 Kaplan, FP37 Tringham, JP15 Cove,DP5 Neila, MP32 Kaushal, RP23 Turner,JP41 Kirkby-Bott, JOC2, P16, Ogilvie, AP36 Dassan, PP28 P25 &P26 Vaks, VP10 Dewbury, KP25 Krishnan, BOC3 &P15 Palazzo, FOC1, OC2, OC6, Valabhji, JP17 &P28 Dhillo, WP9&P20 Krishnan, LOC7 P8, P12, P16 &P26 Webber,LP35 Donaldson, JP38 Kubba, FP32 Patel, PP17 White, SP31 Donaldson, MP31 Kurzawinski, TP32 Peaston, RP1&P2 Whittle, MP33 Dozio, NP13 Perros, PP1&P2 Lagattolla, NP5 Williamson, CP4&P16 Edwards, MP33 Lake, EP26 Rafique,AP32 &P34 Wren, AP10 El-Gayar, HP27 &P30 Lawrence, JP20 Ramachandran, RP31 Wynne,KOC1 &OC6
3rd Hammersmith Multidisciplinary Endocrine Symposium 2008 12 December 2008, London, UK Endocrine Abstracts December 2008 Volume 18 ISSN 1470-3947 (print) ISSN 1479-6848 (online)
3rd Hammersmith Multidisciplinary Endocrine Symposium 2008 12 December 2008, London, UK
Online version available at 1470-3947(200812)18;1-K www.endocrine-abstracts.org
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