DOCSLIB.ORG

Phaeochromocytoma-Investigation and Arch Dis Child: First Published As 10.1136/Adc.65.3.269 on 1 March 1990

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Phaeochromocytoma-Investigation and Arch Dis Child: First Published As 10.1136/Adc.65.3.269 on 1 March 1990 Archives ofDisease in Childhood 1990; 65: 269-274 269 Phaeochromocytoma-investigation and Arch Dis Child: first published as 10.1136/adc.65.3.269 on 1 March 1990. Downloaded from management of 10 cases J E Deal, P S Sever, T M Barratt, M J Dillon Abstract A system for the classification of tumours of Since 1960 we have diagnosed phaeochromo- the autonomic nervous system was proposed by cytoma (paraganglioma) in 10 children. The Glenner and Grimley in 1974 and is now widely cases include a 15 year old girl who over a accepted.3 Under this classification a tumour three year period presented with multiple arising from the adrenal medulla is termed a paragangliomata and an associated malignant phaeochromocytoma and chromaffin tumours carotid body tumour. AR children were hyper- arising outside the adrenals of paraganglionic tensive, eight of 10 presenting with severe origin are termed paragangliomata. Paragang- headaches. Diagnosis was based on finding a liomata are more commonly found at extra- raised urinary vaniliylmandelic acid excretion adrenal sites in children than in adults and can and plasma noradrenaline concentration. In occur anywhere in the autonomic nervous addition six of eight children were hyper- system. Tumours of the carotid body, in as- calcaemic with raised plasma calcitonin con- sociation with phaeochromocytoma, have been centrations; plasma parathyroid hormone frequently reported in adults,4 5 but there is concentrations were high in two of seven and only one report in the literature of an affected four out of eight children had raised plasma child, that of a 12 year old boy.' We report here renin activities on presentation. No child, 10 cases of paragangliomata, including one child however, was found to have a multiple endo- with multiple lesions and a malignant carotid crine neoplasia syndrome. Despite the intro- body tumour, that have been seen at the duction of newer techniques for the detection Hospital for Sick Children since 1960. of catecholamine producing tumours we found that selective arteriography and venous catecholamine sampling were superior for Patients tumour localisation compared with ultrasound REVIEW OF CASES scanning, computed tomography, and meta- Clinical details of all 10 children are summarised iodo-benzyl-guanidine (MIBG) scanning. in table 2; the cases are tabulated in chrono- logical order of presentation. They ranged in age from 3 5 months to 16 years, mean age 10-3 http://adc.bmj.com/ Phaeochromocytoma is a rare but treatable years. Only three children were less than 10 cause of hypertension in childhood.' At the years old at presentation. Hospital for Sick Children, Great Ormond The commonest presentation in the present Street, London, which is a tertiary referral series was severe headaches; varying in duration centre for childhood hypertension, 1-3% of from 11 days to 2-5 years. The youngest child cases referred over a 10 year period, for (case 3) presented with episodes of diarrhoea investigation of high blood pressure, were and sweating and two generalised fits, presumed on October 1, 2021 by guest. Protected copyright. found to have a phaeochromocytoma (table 1). to be due to her hypertension, at the age of In children there is a higher incidence (32%) of 3 months. All children were hypertensive; in multiple tumours and an increased occurrence eight children it was sustained and in only two of the multiple endocrine neoplasia syndromes was the blood pressure labile. One girl (case 10) when compared with that reported in adults.' was diagnosed fortuitously: although asympto- The incidence of malignancy is 2-4%, compared matic she was found to be hypertensive at an Renal Unit, with a malignancy rate of 10% in adults.2 outpatient clinic visit where she was being Hospital for Sick followed up for a stage IV Wilms' tumour that Children and Department seven ofPaediatric Nephrology, Table I Diagnoses of 454 cases of hypertension admitted had been diagnosed and treated years Institute of Child Health, to the renal unit at the Hospital for Sick Children, previously by chemotherapy, radiotherapy, and London 1975-85, including six of the children reported here a right nephrectomy. J E Deal Diagnosis No (%) of children Another girl (case 7) who presented with T M Barratt headaches, pallor, and tachycardia, was initially M J Dillon Coarse renal scarring: Department of Clinical Reflux nephropathy 84 (19) found to have two para-aortic paragangliomata. Pharmacology, Obstructive uropathy 72 (16) After surgical removal of these she presented Glomerular disease 123 (27) St Mary's Hospital, Renovascular disease 38 (8) over the subsequent three years with two London Haemolytic uraemic syndrome 32 (7) further extra-adrenal tumours, bilateral intra- P S Sever Polycystic kidney disease 22 (5) adrenal tumours, and a malignant carotid body Correspondence to: Idiopathic (essential) 18 (4) Dr J E Deal, Department of Renal dysplasia 13 (3) tumour, which were all surgically removed. Paediatric Nephrology, Wilms' tumour 9 (2) Two children had a positive family history: Institute of Child Health, Coarctation 7 (<2) 30 Guilford Street, Phaeochromocytoma 6 (<2) one (case 10) had an uncle who had been London WC1N IEH. Renal vein thrombosis 4 (1) Miscellaneous 26 (6) diagnosed some years before her presentation Accepted 9 October 1989 and the other (case 9) had a 17 year old sister 270 Deal, Sever, Barratt, Dillon Table 2 Clinical details of the 10 children with phaeochromocytoma in chronological order of presentation Case 1 Case 2 Case 3 Case 4 Case S Case 6 Age at presentation Arch Dis Child: first published as 10.1136/adc.65.3.269 on 1 March 1990. Downloaded from (years) 13-3 12-6 0 3 7-5 10-1 10-4 Sex Male Female Female Female Male Female Presentating symptoms Headaches, Headaches, Diarrhoea, Headaches Headaches, Headaches, convulsion pallor, sweating convulsion sweating abdominal pain Investigations: Urinary vanillylmandelic acid T T T T T T Plasma noradrenaline (pg/ml)' - - 1371 5130 18 345 27 700 Plasma renin activity - - N N T N Calcitonin - - T T T Parathyroid hormone - - T N - N Plasma calcium (mmol/l) - - 2-7 2-64 2-75 2-64 Preoperative treatment Phentolamine Phenoxybenzamine, Phenoxybenzamine, Phenoxybenzamine, Phenoxybenzamine Labetalol, propranolol propranolol propranolol phenoxybenzamine, propranolol Site and size of tumour (cm) Right adrenal Right adrenal Left adrenal Right adrenal Left adrenal Right adrenal 5x4 7x6 03x03 5x3 5x5 3x2-5 Other features N, normal; 1, concentration raised. 'To convert pg/ml to nmol/l: x0 005988. who was diagnosed at the same time as he was intravenous pyelography, which failed to localise being investigated. There were no positive the tumour, and then proceeded to an explora- family histories of multiple endocrine neoplasia tory laparotomy. syndromes. At presentation all 10 children had raised 24 hour urinary vanillylmandelic acid concentra- MANAGEMENT tions. Plasma noradrenaline concentrations All patients required treatment for their hyper- were also raised in the eight children measured. tension on presentation as shown in table 2. Case 7, the child with multiple paragangliomata, Six children were successfully treated with a had repeatedly normal urinary vanillylmandelic combination of phenoxybenzamine (1-4 mg/kg! acid concentrations after the removal of her first day) and propranolol (1-10 mg/kg/day) alone. two tumours but raised plasma noradrenaline Case 1 was prepared for surgery with phentola- http://adc.bmj.com/ concentrations. mine (100 [tg/kg intravenously). Treatment Plasma renin activity was raised in four out of with labetalol alone in cases 6, 8, and 10 did not eight patients on presentation and six of the produce adequate a blockade and so their eight had raised plasma calcitonin concentrations treatment was changed. Four of the present (normal <0-08 ,tg/l), but these were normal series were treated with nifedipine (0-5-4 mg/ when repeated postoperatively. Associated with kg/day). In one child (case 8) nifedipine was a raised plasma calcitonin concentration, the six added to the regime of propranolol and on October 1, 2021 by guest. Protected copyright. patients were noted to be hypercalcaemic at phenoxybenzamine to achieve adequate cate- presentation (normal <2-6 mmol/l corrected); cholamine blockade. In case 10 nifedipine alone postoperatively they were normocalcaemic. was insufficient to block the patient's symptoms Plasma concentrations of parathyroid hormone and control the blood pressure, this was only were high in two ofseven children at presentation achieved when phenoxybenzamine and labetalol but fell to normal after removal of their phaeo- were introduced. In two patients (cases 7 and 9) chromocytoma. adequate preoperative control of blood pressure Table 3 shows the localisation techniques that was achieved with nifedipine alone. were used in our patients. Case 1, who presented All 10 children were successfully treated by in 1960 for investigation, had a phaeochromo- surgical removal of their paragangliomata. cytoma clinically diagnosed and underwent There were no operative complications and no Table 3 Radiological techniques used to localise catecholamine secreting tumours in the 10 patients Case No 1 2 3 4 5 6 7 8 9 10 Intravenous pyelography - + Ultrasound -- - + - +* Computed tomography + + '231-MIBG scan +* + ± + Selective arteriography + + + + + + + + Serial catecholamine sampling + + + + + + +, Technique helped localise the tumour; -, technique failed to localise the tumour; +, equivocal result; * MIBG scan localised the tumours
Load more

Recommended publications
  • Hospital Medical School.)
    THE CONTROL OF THE SUPRARENAL GLANDS BY THE SPLANCHNIC NERVES'. BY T. R. ELLIOTT, M.D. (From the Research Laboratories of University College, Hospital Medical School.) THERE is no clear knowledge2 at present with regard to the share taken by the suprarenal glands in resisting various processes that are harmful to the body. For the last two years I have tried to gain some light on this question by analysing the state of exhaustion to which the human suprarenals are reduced in the different conditions leading to death in Hospital cases. Attention was paid chiefly to the loss of the normal load of cortical " lipoid " substance and of the adrenalin in the medulla, the gross total of the latter being measured quantitatively by physio- logical assay. Unfortunately the conditions of fatal disease in man were found to be too complex to permit of simple atialysis. Broadly summarising the results, it appeared that the glands suffered rapid eyhaustion in cases of any microbic fever, of repeated simple hawmorrhage, and of surgical shock: but to distinguish clearly the value and nature of each of these factors was impossible. I therefore tried to reproduce each separately on experimental animals, in which the relationship of the nervous system to the glands could at the same time be studied. Method. Cats were used in all the experiments. The lipoid in the cortex of the cat3 is never so abundant as in the human gland: changes in its distribution were observed histologically, but they did not seem to follow any special cause, and they will be referred to only incidentally in this paper.
    [Show full text]
  • Case Reports
    CASE REPORTS NONCHROMAFFIN PARAGANGLIOMA OF THE JUGULAR FORAMEN Jos~ G. ALBERNAZ,M.D.,* A:N'D PAUL C. BucY, M.D. Chicago Memorial Hospital and Department of Neurology and Neurological Surgery, University of Illinois, College of Medicine, Chicago, Illinois (Received for publication April 7, 1953) This paper deals with a carotid-body-like tumor found in the jugular foramen, similar to the tumors recently described by Lattes 12 as nouchromaffin paraganglio- mas. Kohn, 1~ in 1900, classified the carotid body as a paraganglion, in the belief that it was of sympathetic origin and part of the chromaffin system. Small masses of similar paraganglionic tissue have since been described in the following locations: 1) At the level of the middle ear. As early as 1840, Valentin 26 described a gangliolum tympanicum, associated with the tympanic branch of the 9th cranial nerve. Later, in 1879, Krause H showed that this structure resembles the carotid body and this has been confirmed recently by the studies of Guild s and Lattes and WaltnerY In addi- tion to the paraganglion tympanicum, Guild s described what he called the glomus jugularis "in the adventitia of the dome of the jugular bulb, immediately below the bony floor of the middle ear." ~) At the aortic-pulmonary region. Paraganglia related to the major vessels at the base of the heart were mentioned in 190~ by Biedl and Wiesel. 2 In the middle thirties, Palme, 2~ Seto, 24 Muratori ~9 and Nonidez 2~ described these structures in detail. From their contributions, well summarized by Boyd, 4 we learn that these "aortic bodies" are similar to the carotid bodies and that they can be found in four different locations: a) above the ductus arteriosus; b) on the trunk of the pulmonary artery, near the origin of the left coronary artery; c) near the root of the innominate artery; d) on the left part of the aortic arch.
    [Show full text]
  • A Xenograft and Cell Line Model of SDH-Deficient Pheochromocytoma Derived from Sdhb+/− Rats
    27 6 Endocrine-Related J F Powers et al. Rat model for Sdhb-mutated 27:6 337–354 Cancer paraganglioma RESEARCH A xenograft and cell line model of SDH-deficient pheochromocytoma derived from Sdhb+/− rats James F Powers1, Brent Cochran2, James D Baleja2, Hadley D Sikes3, Andrew D Pattison4, Xue Zhang2, Inna Lomakin1, Annette Shepard-Barry1, Karel Pacak5, Sun Jin Moon3, Troy F Langford3, Kassi Taylor Stein3, Richard W Tothill4,6, Yingbin Ouyang7 and Arthur S Tischler1 1Department of Pathology and Laboratory Medicine, Tufts Medical Center, Tufts University School of Medicine, Boston, Massachusetts, USA 2Department of Developmental, Molecular and Chemical Biology, Tufts University School of Medicine, Boston, Massachusetts, USA 3Department of Chemical Engineering, Massachusetts Institute of Technology, Cambridge, Massachusetts, USA 4Department of Clinical Pathology, University of Melbourne, Melbourne, Victoria, Australia 5Section on Medical Neuroendocrinology, Eunice Kennedy Shriver Division National Institute of Child Health and Human Development, Bethesda, Maryland, USA 6Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia 7Cyagen US Inc, Santa Clara, California, USA Correspondence should be addressed to J F Powers: [email protected] Abstract Tumors caused by loss-of-function mutations in genes encoding TCA cycle enzymes Key Words have been recently discovered and are now of great interest. Mutations in succinate f succinate dehydrogenase B dehydrogenase (SDH) subunits cause pheochromocytoma/paraganglioma (PCPG) and f paraganglioma syndromically associated tumors, which differ phenotypically and clinically from more f pheochromocytoma common SDH-intact tumors of the same types. Consequences of SDH deficiency include f cluster 1 rewired metabolism, pseudohypoxic signaling and altered redox balance. PCPG with f xenograft SDHB mutations are particularly aggressive, and development of treatments has been f cell culture hampered by lack of valid experimental models.
    [Show full text]
  • Opmaak 1 15/06/12 08:52 Pagina 124
    arain-_Opmaak 1 15/06/12 08:52 Pagina 124 JBR–BTR, 2012, 95: 124-125. PARAGANGLIOMA OF THE CAVERNOUS SINUS A. Arain, J. Vandevenne, B. Depeuter, J. Smits, F. Weyns, Y. Palmers 1 Key-word: Paraganglioma Background : A 15-year-old girl presented in a Dutch hospital with right-sided trigeminal neuralgia. MR-imaging showed a mass lesion in the right cavernous sinus. Differential diagnosis in this hospital was a meningioma or a schwannoma. The patient was referred to the neurosurgery department of our hospital, and resection of the lesion was planned. At surgery, the lesion pre - sented as a subdural bulge surrounded by swollen venous structures. The incision of the dura resulted in profuse hemorrhage of arterial origin, and hemostasis was obtained with difficulty. The tumor showed a fibrillar structure and a strong arterial vascularization which was not con - cordant with schwannoma. No further exploration of the lesion was performed, and no biopsy was obtained. To clarify the unexpected surgical findings and to reach a diagnosis without biopsy , pre-operative MR-images were reviewed. Digital subtraction angio graphy (DSA) was performed postoperatively. The lesion did not take up FDG on PET scan. Laboratory results showed increased catecholamines in the urine. AB CD E 1A 1B Fig. 1C 1D 1. Department of Medical Imaging, Campus Sint-Jan, Ziekenhuis Oost-Limburg, Genk, Belgium. 1E 2 arain-_Opmaak 1 15/06/12 08:52 Pagina 125 PARAGANGLIOMA OF THE CAVERNOUS SINUS — ARAIN et al 125 Work-up ly show a hyperintense signal on T2-weighted MR- images and a distinct contrast enhancement on T1- MRI of the brain (Fig.
    [Show full text]
  • Fernando De Castro and the Discovery of the Arterial Chemoreceptors
    REVIEW ARTICLE published: 12 May 2014 doi: 10.3389/fnana.2014.00025 Fernando de Castro and the discovery of the arterial chemoreceptors Constancio Gonzalez 1,2 *, Silvia V. Conde 1,2 ,Teresa Gallego-Martín 1,2 , Elena Olea 1,2 , Elvira Gonzalez-Obeso 1,2 , Maria Ramirez 1,2 , SaraYubero 1,2 , MariaT.Agapito 1,2 , Angela Gomez-Niño 1,2 , Ana Obeso 1,2 , Ricardo Rigual 1,2 and Asunción Rocher 1,2 1 Departamento de Bioquímica y Biología Molecular y Fisiología, Instituto de Biología y Genética Molecular, Consejo Superior de Investigaciones Científicas, Universidad de Valladolid, Valladolid, España 2 CIBER de Enfermedades Respiratorias, Instituto de Salud Carlos III, Facultad de Medicina, Universidad de Valladolid, Valladolid, España Edited by: When de Castro entered the carotid body (CB) field, the organ was considered to be a Fernando de Castro, Hospital Nacional small autonomic ganglion, a gland, a glomus or glomerulus, or a paraganglion. In his 1928 de Parapléjicos – Servicio de Salud de Castilla-La Mancha, Spain paper, de Castro concluded: “In sum, the Glomus caroticum is innervated by centripetal fibers, whose trophic centers are located in the sensory ganglia of the glossopharyngeal, Reviewed by: José A. Armengol, University Pablo de and not by centrifugal [efferent] or secretomotor fibers as is the case for glands; these are Olavide, Spain precisely the facts which lead to suppose that the Glomus caroticum is a sensory organ.” Ping Liu, University of Connecticut A few pages down, de Castro wrote: “The Glomus represents an organ with multiple
    [Show full text]
  • Chapter 13. Secondary Hypertension
    Hypertension Research (2014) 37, 349–361 & 2014 The Japanese Society of Hypertension All rights reserved 0916-9636/14 www.nature.com/hr GUIDELINES (JSH 2014) Chapter 13. Secondary hypertension Hypertension Research (2014) 37, 349–361; doi:10.1038/hr.2014.16 OVERVIEW AND SCREENING approximately 5–10% of hypertensive patients,984,985 and it is the most Hypertension related to a specific etiology is termed secondary frequent in endocrine hypertension. In addition, frequent etiological hypertension, markedly differing from essential hypertension, of factors for secondary hypertension include renal parenchymal hyper- which the etiology cannot be identified, in the condition and tension and renovascular hypertension. A study reported that sleep therapeutic strategies. Secondary hypertension is often resistant hyper- apnea syndrome was the most frequent factor for secondary hyper- tension, for which a target blood pressure is difficult to achieve by tension.517 The number of patients with secondary hypertension standard treatment. However, blood pressure can be effectively may further increase with the widespread diagnosis of sleep apnea reduced by identifying its etiology and treating the condition. There- syndrome. fore, it is important to suspect secondary hypertension and reach an Generally, the presence of severe or resistant hypertension, juvenile appropriate diagnosis. hypertension and the rapid onset of hypertension suggest the possi- Frequent etiological factors for secondary hypertension include bility of secondary hypertension. In such hypertensive patients, a close renal parenchymal hypertension, primary aldosteronism (PA), reno- inquiry on medical history, medical examination and adequate vascular hypertension and sleep apnea syndrome. Renal parenchymal examinations must be performed, considering the possibility of hypertension is caused by glomerular diseases, such as chronic secondary hypertension.
    [Show full text]
  • Paragangliomas of the Head and Neck: a Pictorial Essay
    Paragangliomas of the Head and Neck: A Pictorial Essay Jerry C. Lee, MD, Ajay Malhotra, MD, Henry Wang, MD, PhD, Per-Lennart Westesson, MD, PhD, DDS Division of Diagnostic and Interventional Neuroradiology Department of Imaging Sciences University of Rochester Medical Center Rochester, New York Presentation material is for education purposes only. All rights reserved. ©2007 URMC Radiology Page 1 of 25 Purpose Learn the common locations of paragangliomas of the head and neck and where they originate. Learn the common imaging findings of paragangliomas utilizing CT, MRI, and angiography. Presentation material is for education purposes only. All rights reserved. ©2007 URMC Radiology Page 2 of 25 J. Lee, MD et al Introduction Paragangliomas of the head and neck originate most commonly from the paraganglia within the carotid body, vagal nerve, middle ear, and jugular foramen. Also called glomus tumors, they arise from paraganglion cells of neuroectodermal origin frequently located near nerves and vessels. The function of most paraganglia in the head and neck is obscure; one exception is the carotid body, which is a chemoreceptor. Paragangliomas account for 0.6% of all neoplasms in the head and neck region, and about 80% of all paraganglioms are either carotid body tumors or glomus jugulare tumors. The classic manifestation of a carotid body tumor is a nontender, enlarging lateral neck mass which is mobile, pulsatile, and associated with a bruit. The jugulare and tympanicum tumors commonly cause pulsatile tinnitus and hearing loss and may cause cranial nerve compression. Vagal paraganglioms are the least common and present as a painless neck mass which may result in dysphagia and hoarseness.
    [Show full text]
  • Continuing Medical Education Article Anaesthesia for Phaeochromocytoma
    526 Continuing Medical Education Article Anaesthesia for John Pullerits ~D eacr'c, Sigmund Ein MD FRCSC, J. Wi~liamson Belle MD r~cvc phaeochromocytoma Phaeochromocytomas are functionally active cate- Type II-b (characterized by medullary carcinoma of the eholamine-seereting tumours of chromaffin tissue. These thyroid, Marf~oid appearance, mucosal adenomas and tumours are usually found in the adrenal medullae but phaeochromoeytoma). They can also be found as part of may occur anywhere where chromaffln tissue exists. von Hippel-Lindau syndrome which includes multiple Chromaffin tissue occurs in association with the celiac, phaeochromocytomas in association with haemangioblas- mesenteric, renal, adrenal, hypogastric, testicular and lomas of the retinae, cerebellum and other arcas of the paravertehral sympathetic nervous plexuses. Although central nervous system) most phaeochromocytomas are found in the adrenal Although most phaeochromocytoma8 are unilateral, medulla (in 90 per cent of cases), they can occur anywhere about nine per cent occur bilaterally. Bilateral tumours in the body where paraganglion ceils of the sympathetic also tend to be familial. Phaeochromocytomas may occur nervous system are found. Multiple and extra-adrenal at any age, but occur with the greatest frequency in the tumours are far more common in children (35 per cent) fourth and fifth decades of life. Ten per cent of these than in adults (eight per cent).t Although about 90 per tumours are found in children. In adults, the sex distribu- cent are benign and surgically curable, they are of special tion of the patient population shows a slightly higher interest because of the profound physiological effects female preponderance (55-60 per cent).
    [Show full text]
  • The Human Embryonic Heart in the Ninth Week
    THE HUMAN EMBRYONIC HEART IN THE NINTH WEEK RICHARD H. LICATA Department of Aqiatoniy, Univeisily of Michigan Medical School, Ann ArboT SIXTEEN I'IQURES Although observations on the developing heart go back to the time of Aristotle, it was not until late in the 19th century that the work of His (1885, 1886) and Born (1888, 1889) resulted in an understanding of heart development that was of sdlicient accuracy to be still of basic value by present standards. One of the crucial factors in their work was the development of adequate methods of reconstruction. Although His was the first to attempt sucli reconstructions, his efforts met with limited success and he had to resort to freehand modelling of embryonic hearts. These models, reproduced commercially by Ziegler, are fairly accurate, though they lack the detail of models made by the wax-plate method. In 1883 Born began to employ the wax-plate reconstruction method as a means of studying heart development. This technique was improved by Kastschenko (1886) and Strasser (1887). Later, the continued efforts of Born and Strasser brought this technique to a high degree of accuracy. A description of their methods was published in detail by Born in 1889. Tandler ('12, '13) greatly furthered knowledge of heart development by coordinating the results of earlier workers and supplementing them with extensive contributions of his own. Mall's work ('12) on the structure of the ventricles, and his observations on the development of the conduction sys- This paper represents x condensation of one section of a dissertation siili- rnitted in partial fulfillment of the requirements for the dqree of Doctor of Philosophy in the University of Michigan.
    [Show full text]
  • VU Research Portal
    VU Research Portal SDHB-linked Paraganglioma Rijken, J.A. 2020 document version Publisher's PDF, also known as Version of record Link to publication in VU Research Portal citation for published version (APA) Rijken, J. A. (2020). SDHB-linked Paraganglioma. General rights Copyright and moral rights for the publications made accessible in the public portal are retained by the authors and/or other copyright owners and it is a condition of accessing publications that users recognise and abide by the legal requirements associated with these rights. • Users may download and print one copy of any publication from the public portal for the purpose of private study or research. • You may not further distribute the material or use it for any profit-making activity or commercial gain • You may freely distribute the URL identifying the publication in the public portal ? Take down policy If you believe that this document breaches copyright please contact us providing details, and we will remove access to the work immediately and investigate your claim. E-mail address: [email protected] Download date: 08. Oct. 2021 SDHB-linked Paraganglioma COLOPHON SDHB-linked Paraganglioma Cover drawing: Johannes Rijken Cover layout: Rachel van Esschoten, DivingDuck Design, (www.divingduckdesign.nl) Lay-out inside: Rachel van Esschoten, DivingDuck Design Printed by: Ipskamp Printing, Enschede (www.ipskampprinting.nl) Anatomical drawings: Bourgery, Paris 1831-1844, Library University of Heidelberg. ISBN: 978-94-028-1905-2 The publication of this thesis was financially supported by: ALK, Allergy Therapeutics, DOS Medical BV, Rhino Horn Benelux BV, Specsavers International Healthcare BV. VRIJE UNIVERSITEIT SDHB-linked PARAGANGLIOMA ACADEMISCH PROEFSCHRIFT ter verkrijging van de graad Doctor aan de Vrije Universiteit Amsterdam, op gezag van de rector magnificus prof.dr.
    [Show full text]
  • Extra-Adrenal Paraganglioma: an Uncommon Cause of Secondary Hypertension in a Young Subject
    Case Report September-December, 2011/Vol 31/Issue 3 Extra-adrenal Paraganglioma: An Uncommon Cause of Secondary Hypertension in a Young Subject Kota SK1, Kota SK2, Jammula S3, Panda S4 1Dr. Sunil Kumar Kota, DNB Resident, Department of Endocrinology, Medwin Hospital, Hyderabad-500001, Andhra Pradesh, India. 2 Dr. Siva Krishna Kota Department of Anaesthesia, Central Security Hospital, RIYADH, Saudi Arabia. 3Dr. Sruti Jammula Department of Pharmaceutics, Roland Institute of Pharmaceutical Sciences, BERHAMPUR, Orissa, India. 4Dr.Sandip Panda Department of Cardiology, Jawaharlal Institute of Postgraduate Medical Education and Research, Pundicherry, India. Address for correspondence: Dr. Sunil Kumar Kota, E-mail: [email protected] Abstract A 8 year boy was admitted with features of tuberculous meningitis. He was detected to have hypertension. Further investigations revealed an extra-adrenal paraganglioma. Tuberculous meningitis and paraganglioma could be chance associations. Paraganglioma is a rare and potentially lethal cause of secondary hypertension, very uncommon at this age. If left untreated it leads to complications arising from unrelenting hypertension. We report a case report on this very rare disease of paraganglioma presenting in an unexpected way. Key words: Paraganglioma, Tuberculous meningitis, Secondary hypertension Introduction hemoglobin 13.8 g%, total leukocyte count 13,400 /mm3 The paraganglion system is formed by numerous with polymorphs 75% and lymphocytes 23%. His blood collections of neuroepithelial cells1. Paraganglioma are sugar, renal function tests, liver function tests, and serum tumors of paraganglia. The most common site is adrenal electrolytes were within normal limits. Cerebrospinal medulla, where it is known as pheochromocytomas. fl uid analysis revealed lymphocytic pleocytosis in the Those located outside the adrenal gland as extra-adrenal cerebrospinal fl uid with protein content of 134 mg% paragangliomas (EAPs).
    [Show full text]
  • Torsades De Pointes: a Rare Complication of an Extra-Adrenal Pheochromocytoma
    1263 Hypertens Res Vol.30 (2007) No.12 p.1263-1266 Case Report Torsades de Pointes: A Rare Complication of an Extra-Adrenal Pheochromocytoma Heiko METHE1), Martin HINTERSEER1), Ute WILBERT-LAMPEN1), Britt M. BECKMANN1), Gerhard STEINBECK1), and Stefan KÄÄB1) Pheochromocytoma is an infrequent secondary cause of arterial hypertension, often associated with parox- ysmal headache, sweating, weight loss, and palpitations. Cardiovascular complications of pheochromocy- toma include sudden death, heart failure due to toxic cardiomyopathy, and hypertensive encephalopathy. Here we report the case of a female with an acquired long-QT-syndrome as a rare complication of an extra- adrenal pheochromocytoma. Diagnosis was made after sotalol-induced Torsades de Pointes. (Hypertens Res 2007; 30: 1263–1266) Key Words: pheochromocytoma, long-QT-syndrome, Torsades de Pointes There was no family history of sudden death. Serum electro- Introduction lytes were as follows: sodium 139 mmol/L, potassium 5.1 mmol/L, calcium 2.7 mmol/L, anorganic phosphorus 4.1 mg/ Pheochromocytoma is a rare cause of sustained hypertension dL, magnesium 1.97 mg/dL. Fasting blood glucose was ele- or hypertensive crisis and many cardiac complications have vated to 124 mg/dL. Renal, liver and thyroid tests were all been reported with this neuroendocrine tumor. Development normal. of long-QT-syndrome (LQTS) due to myocardial hypertro- Her baseline ECG showed sinus rhythm and deep T-wave phy in this patient group is an occasional but potentially life- inversion in V2–4. Most remarkably it showed a QTc interval threatening complication increasing the risk for arrhythmias. of 480 ms (Fig. 1A). Sotalol-treatment was discontinued immediately but during intensive care unit monitoring, a Tor- Case Report sades de Pointes tachycardia was recorded (Fig.
    [Show full text]