DOCSLIB.ORG

Expanding the Cause of Hypopituitarism

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Expanding the Cause of Hypopituitarism 176:6 S Pekic and V Popovic Unusual causes of 176:6 R269–R282 Review hypopituitarism PROOF ONLY DIAGNOSIS OF ENDOCRINE DISEASE Expanding the cause of hypopituitarism Sandra Pekic1,2 and Vera Popovic1 Correspondence 1School of Medicine, University of Belgrade, Belgrade, Serbia and 2Clinic of Endocrinology, Diabetes and should be addressed Metabolic Diseases, University Clinical Center Belgrade, Belgrade, Serbia to V Popovic Email [email protected] Abstract Hypopituitarism is defined as one or more pituitary hormone deficits due to a lesion in the hypothalamic–pituitary region. By far, the most common cause of hypopituitarism associated with a sellar mass is a pituitary adenoma. A high index of suspicion is required for diagnosing hypopituitarism in several other conditions such as other massess in the sellar and parasellar region, brain damage caused by radiation and by traumatic brain injury, vascular lesions, infiltrative/immunological/inflammatory diseases (lymphocytic hypophysitis, sarcoidosis and hemochromatosis), infectious diseases and genetic disorders. Hypopituitarism may be permanent and progressive with sequential pattern of hormone deficiencies (radiation-induced hypopituitarism) or transient after traumatic brain injury with possible recovery occurring years from the initial event. In recent years, there is increased reporting of less common and less reported causes of hypopituitarism with its delayed diagnosis. The aim of this review is to summarize the published data and to allow earlier identification of populations at risk of hypopituitarism as optimal hormonal replacement may significantly improve their quality of life and life expectancy. European Journal of Endocrinology (2017) 176, R269–R282 European Journal European of Endocrinology Introduction Hypopituitarism is an uncommon condition with of underlying etiology of hypopituitarism is the a prevalence of ~46 per 1 00 000 (1). It is defined as challenge. The most common cause of hypopituitarism one or more anterior pituitary hormone deficiencies associated with a sellar mass is nonfunctioning pituitary caused by a variety of structural lesions or trauma macroadenoma. Hypopituitarism may occur as a in the hypothalamic–pituitary region. The diversity consequence of treatment of pituitary adenomas with Invited Authors’ profile Prof Dr Vera Popovic-Brkic MD, PhD, FRCP is a Professor of Internal Medicine at Belgrade University School of Medicine, Belgrade, Serbia. She is principal investigator with a research grant funded by the Ministry of Science, Serbia since 1985. Her research studies include investigations in the field of new molecules in the regulation of GH secretion (GHRP-6 and ghrelin), long-acting GH molecules for GH replacement, and new molecules in energy homeostasis (leptin and ghrelin). Her special clinical interests are hypopituitarism and the management of pituitary adenomas. Vera Popovic-Brkic Sandra Pekic MD, PhD is currently Associate Professor of Internal Medicine and Endocrinology, University of Belgrade, Serbia. Her clinical research is mainly devoted to pituitary adenomas, hypopituitarism and growth hormone disorders. Sandra Pekic www.eje-www.eje-online.orgonline.org © 2017 European Society of Endocrinology Published by Bioscientifica Ltd. DOI: 10.1530/EJE-16-1065 Printed in Great Britain Downloaded from Bioscientifica.com at 09/25/2021 12:25:44PM via free access 10.1530/EJE-16-1065 Review S Pekic and V Popovic Unusual causes of 176:6 R270 hypopituitarism pituitary surgery and/orPROOF radiotherapy. Other less prevalent Neoplastic ONLY lesions in the sellar and causes of hypopituitarism such as radiation-induced and suprasellar region traumatic brain injury-induced hypopituitarism have been studied more extensively in the recent years. More Hypopituitarism may be the consequence of many rarely, vascular causes (pituitary apoplexy, Sheehan’s sellar and parasellar masses that include meningioma, syndrome, subarachnoid hemorrhage and aneurysm), germinoma, glioma, astrocytoma, chordoma, gang- infiltrative/immune/inflammatory processes such as lioneuroma, pituicytoma, pituitary lymphoma, plasma- lymphocytic hypophysitis, sarcoidosis, hemochromatosis cytoma and metastases (6). and infectious diseases have been associated with hypopituitarism (Table 1). Pituitary lymphoma Whatever the cause, hypopituitary patients usually exhibit nonspecific symptoms/signs. For the clinician, it Recently published systematic review of primary sellar remains challenging to ascribe specific features for each lymphoma reported a total of 33 cases of this isolated pituitary hormone deficiency. The recently published localization of lymphoma in the sella (7). The vast majority guidelines on hormonal replacement in hypopituitarism of CNS lymphomas are non-Hodgkin B-cell lymphomas. in adults discuss in great detail the diagnosis and T-cell lymphoma and NK/T-cell lymphoma are much management of hypopituitarism (2). more rare. Patients do not have systemic lymphoma or Hypopituitarism is associated with increased other brain lesions. Even rarer is an intravascular large mortality (3). Highest mortality is among younger B-cell lymphoma characterized by massive proliferation patients, women and patients with diabetes insipidus (4). of lymphoid cells within the small and medium blood A recent study from Sweden shows a decline in mortality vessels. We reported a rare case of hypopituitarism in in patiens optimally replaced including GH replacement a patient with intravascular large B-cell lymphoma therapy (5). who after complete hematological remission, reversed Given the complexity of hypopituitarism, its insidious hypopituitarism after more than two years (8). Anterior onset with a delay in the diagnosis by decades, here pituitary deficiencies are present in 70% of cases (most we review contemporary studies on the less common/ have panhypopituitarism), diabetes insipidus is frequent unusual causes of hypopituitarism in adults. (in 36% of cases), unlike in pituitary adenomas (7). MRI usually shows diffusely enlarged pituitary gland with invasion of both cavernous sinuses mimicking a pituitary European Journal European of Endocrinology Table 1 Causes of hypopituitarism. adenoma. Usually a preoperative presumptive diagnosis of pituitary adenoma is made and only pathologic evaluation Neoplastic Pituitary and non-pituitary tumors Metastases reveals the diagnosis of the pituitary lymphoma. Invasive Hematological malignancy sellar mass with rapid growth pattern and cranial nerve Langerhans cell histiocytosis palsies in an elderly patient (60–70 years) may be Brain damage Traumatic brain injury Cranial irradiation discriminative and suggestive of lymphoma. Congenital causes Isolated or multiple pituitary hormone deficiencies Vascular Postpartal pituitary necrosis Intrasellar plasmacytoma (Sheehan’s syndrome) Aneurysm Intrasellar plasma cell tumors are rare, may mimic Pituitary apoplexy nonfunctioning pituitary tumors and may present as Subarachnoid hemorrhage Snake bite the first manifestation of multiple myeloma 9( ). The Inflammatory/ Sarcoidosis radiological and clinical features of sellar plasmacytoma infiltrative are nonspecific. Cranial nerve affection and intact Lymphocytic hypophysitis (primary, secondary) pituitary function are usually present. The diagnosis Haemochromatosis is made histologically. The diagnosis of intrasellar Amiloidosis plasmacytoma should be suspected in patients with Wegener’s granulomatosis invasive sellar mass, intact anterior pituitary function and Infectious diseases Bacterial, viral, fungal, parasital Other causes Congenital chronic hydrocephalus cranial nerve palsies in the presence of significant sellar Primary empty sella destruction (Fig. 1). www.eje-online.org Downloaded from Bioscientifica.com at 09/25/2021 12:25:44PM via free access Review S Pekic and V Popovic Unusual causes of 176:6 R271 hypopituitarism PROOF ONLY Figure 2 Figure 1 (A) A 62-year-old woman with euthyroid multinodular goiter (A and B) A 57-year-old woman presenting with headache, presenting with bitemporal hemianopsia and moderate dyplopia and ptosis. Sellar MRI ((A) sagittal and (B) coronal hypeprolactinemia. (B) Sellar MRI shows a large suprasellar views) shows an invasive sellar mass with propagation to the mass. (C) Pathology report: Metastasis from papillary sphenoid sinus and clivus. Pathology report: Pituitary carcinoma of thyroid not previously diagnosed. From: plasmacytoma IgA kappa+. Stojanovic M et al. European Thyroid Journal 2013 1 277–284. Pituitary metastasis (reproduced with permission). from myeloid progenitor cells that express CD34 Metastatic tumors represent a small fraction of sellar surface antigen. Recently, somatic oncogenic BRAFV600E masses. A rise in incidence is due to longer life expectancy mutations were detected in 25–60% of LCH patients, in patients with malignant disease and advances in suggesting that LCH is likely a neoplastic disorder (12). diagnostic imaging. In most cases, the nature of a metastasis Positive response to a BRAF inhibitor vemurafenib in to the sellar region is suggested by the primary tumor, but patients with BRAFV600E-mutated LCH is described (13). when the primary tumor is unknown, sellar metastasis is Central nervous system is affected in 6% of patients diagnosed postmortem. Breast and lung cancers are the with LHC, mostly the hypothalamopituitary region and European Journal European of Endocrinology most common sources of metastases (10, 11). Pituitary pineal gland, cerebellum and basal ganglia (14). Patients metastases
Load more

Recommended publications
  • Lymphocytic Hypophysitis Successfully Treated with Azathioprine
    1581 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.74.11.1581 on 14 November 2003. Downloaded from SHORT REPORT Lymphocytic hypophysitis successfully treated with azathioprine: first case report A Lecube, G Francisco, D Rodrı´guez, A Ortega, A Codina, C Herna´ndez, R Simo´ ............................................................................................................................... J Neurol Neurosurg Psychiatry 2003;74:1581–1583 is not well established, but corticosteroids have been An aggressive case of lymphocytic hypophysitis is described proposed as first line treatment.10–12 Trans-sphenoidal surgery which was successfully treated with azathioprine after failure should be undertaken in cases associated with progressive of corticosteroids. The patient, aged 53, had frontal head- mass effect, in those in whom radiographic or neurological ache, diplopia, and diabetes insipidus. Cranial magnetic deterioration is observed during treatment with corticoster- resonance imaging (MRI) showed an intrasellar and supra- oids, or when it is impossible to establish the diagnosis of sellar contrast enhancing mass with involvement of the left lymphocytic hypophysitis with sufficient certainty.25 cavernous sinus and an enlarged pituitary stalk. A putative We describe an unusually aggressive case of pseudotumor- diagnosis of lymphocytic hypophysitis was made and ous lymphocytic hypophysitis successfully treated with prednisone was prescribed. Symptoms improved but azathioprine. This treatment was applied empirically because recurred after the dose was reduced. Trans-sphenoidal of the failure of corticosteroids. To the best to our knowledge, surgery was attempted but the suprasellar portion of the this is the first case of lymphocytic hypophysitis in which mass could not be pulled through the pituitary fossa. such treatment has been attempted. The positive response to Histological examination confirmed the diagnosis of lympho- azathioprine suggests that further studies should be done to cytic hypophysitis.
    [Show full text]
  • A Case of Malignant Insulinoma Responsive to Somatostatin Analogs
    Caliri et al. BMC Endocrine Disorders (2018) 18:98 https://doi.org/10.1186/s12902-018-0325-4 CASE REPORT Open Access A case of malignant insulinoma responsive to somatostatin analogs treatment Mariasmeralda Caliri1†, Valentina Verdiani1†, Edoardo Mannucci2, Vittorio Briganti3, Luca Landoni4, Alessandro Esposito4, Giulia Burato5, Carlo Maria Rotella2, Massimo Mannelli1 and Alessandro Peri1* Abstract Background: Insulinoma is a rare tumour representing 1–2% of all pancreatic neoplasms and it is malignant in only 10% of cases. Locoregional invasion or metastases define malignancy, whereas the dimension (> 2 cm), CK19 status, the tumor staging and grading (Ki67 > 2%), and the age of onset (> 50 years) can be considered elements of suspect. Case presentation: We describe the case of a 68-year-old man presenting symptoms compatible with hypoglycemia. The symptoms regressed with food intake. These episodes initially occurred during physical activity, later also during fasting. The fasting test was performed and the laboratory results showed endogenous hyperinsulinemia compatible with insulinoma. The patient appeared responsive to somatostatin analogs and so he was treated with short acting octreotide, obtaining a good control of glycemia. Imaging investigations showed the presence of a lesion of the uncinate pancreatic process of about 4 cm with a high sst2 receptor density. The patient underwent exploratory laparotomy and duodenocephalopancreasectomy after one month. The definitive histological examination revealed an insulinoma (T3N1MO, AGCC VII G1) with a low replicative index (Ki67: 2%). Conclusions: This report describes a case of malignant insulinoma responsive to octreotide analogs administered pre- operatively in order to try to prevent hypoglycemia. The response to octreotide analogs is not predictable and should be initially assessed under strict clinical surveillance.
    [Show full text]
  • HYPOPITUITARISM YOUR QUESTIONS ANSWERED Contents
    PATIENT INFORMATION HYPOPITUITARISM YOUR QUESTIONS ANSWERED Contents What is hypopituitarism? What is hypopituitarism? 1 What causes hypopituitarism? 2 The pituitary gland is a small gland attached to the base of the brain. Hypopituitarism refers to loss of pituitary gland hormone production. The What are the symptoms and signs of hypopituitarism? 4 pituitary gland produces a variety of different hormones: 1. Adrenocorticotropic hormone (ACTH): controls production of How is hypopituitarism diagnosed? 6 the adrenal gland hormones cortisol and dehydroepiandrosterone (DHEA). What tests are necessary? 8 2. Thyroid-stimulating hormone (TSH): controls thyroid hormone production from the thyroid gland. How is hypopituitarism treated? 9 3. Luteinizing hormone (LH) and follicle-stimulating hormone (FSH): LH and FSH together control fertility in both sexes and What are the benefits of hormone treatment(s)? 12 the secretion of sex hormones (estrogen and progesterone from the ovaries in women and testosterone from the testes in men). What are the risks of hormone treatment(s)? 13 4. Growth hormone (GH): required for growth in childhood and has effects on the entire body throughout life. Is life-long treatment necessary and what precautions are necessary? 13 5. Prolactin (PRL): required for breast feeding. How is treatment followed? 14 6. Oxytocin: required during labor and delivery and for lactation and breast feeding. Is fertility possible if I have hypopituitarism? 15 7. Antidiuretic hormone (also known as vasopressin): helps maintain normal water Summary 15 balance. What do I need to do if I have a pituitary hormone deficiency? 16 Glossary inside back cover “Hypo” is Greek for “below normal” or “deficient” Hypopituitarism may involve the loss of one, several or all of the pituitary hormones.
    [Show full text]
  • Hypopituitarism Due to Lymphocytic Hypophysitis in a Patient with Retroperitoneal Fibrosis
    732 Alvarez, Cordido, Sacriscin Postgrad Med J: first published as 10.1136/pgmj.73.865.732 on 1 November 1997. Downloaded from Hypopituitarism due to lymphocytic hypophysitis in a patient with retroperitoneal fibrosis A Alvarez, F Cordido, F Sacristan Summary of 130/70 mmHg and body temperature of We present a 78-year-old woman with 37°C. Cardiopulmonary auscultation showed clinical acute hypopituitarism in whom a systolic murmur and bilateral rales. There pathologic findings showed lymphocytic was marked tenderness after percussion of the hypophysitis and retroperitoneal fibrosis. right costovertebral area. Laboratory blood Lymphocytic hypophysitis should be in- tests revealed an erythrocyte count of cluded in the differential diagnosis of 3.1 x 1012/1, haemoglobin 6.0 mmol/l, haema- hypopituitarism at any age. The associa- tocrit 0.267, leucocyte count 8.7 x 109/1, urea tion with idiopathic retroperitoneal fibro- 34.1 mmol/l, creatinine 335.9 mmol/l, potas- sis suggest an autoimmune origin for this sium 5.5 mmol/l, sodium 135 mmol/l and entity. glucose 6.1 mmol/l. The urinary sediment presented abundant white blood cells and Keywords: hypopituitarism, lymphocytic hypophysi- bacteria. Chest X-ray showed a right pleural tis, retroperitoneal fibrosis effusion. Renal sonogram showed bilateral nephrolithiasis, high-grade right obstructive uropathy, and atrophic right kidney. The Hypopituitarism is most frequently due to a initial diagnosis was urinary tract infection pituitary tumour, other common causes being complicating obstructive uropathy. Empiric surgery, pituitary radiation therapy and pitui- antibacterial treatment was prescribed (aztreo- tary apoplexy. Less common causes ofpituitary nam). Her condition worsened with vomiting, insufficiency include empty sella syndrome, progressive decline in mental status and head trauma, internal carotid artery aneurysm hypotension.
    [Show full text]
  • A Radiologic Score to Distinguish Autoimmune Hypophysitis from Nonsecreting Pituitary ORIGINAL RESEARCH Adenoma Preoperatively
    A Radiologic Score to Distinguish Autoimmune Hypophysitis from Nonsecreting Pituitary ORIGINAL RESEARCH Adenoma Preoperatively A. Gutenberg BACKGROUND AND PURPOSE: Autoimmune hypophysitis (AH) mimics the more common nonsecret- J. Larsen ing pituitary adenomas and can be diagnosed with certainty only histologically. Approximately 40% of patients with AH are still misdiagnosed as having pituitary macroadenoma and undergo unnecessary I. Lupi surgery. MR imaging is currently the best noninvasive diagnostic tool to differentiate AH from V. Rohde nonsecreting adenomas, though no single radiologic sign is diagnostically accurate. The purpose of this P. Caturegli study was to develop a scoring system that summarizes numerous MR imaging signs to increase the probability of diagnosing AH before surgery. MATERIALS AND METHODS: This was a case-control study of 402 patients, which compared the presurgical pituitary MR imaging features of patients with nonsecreting pituitary adenoma and controls with AH. MR images were compared on the basis of 16 morphologic features besides sex, age, and relation to pregnancy. RESULTS: Only 2 of the 19 proposed features tested lacked prognostic value. When the other 17 predictors were analyzed jointly in a multiple logistic regression model, 8 (relation to pregnancy, pituitary mass volume and symmetry, signal intensity and signal intensity homogeneity after gadolin- ium administration, posterior pituitary bright spot presence, stalk size, and mucosal swelling) remained significant predictors of a correct classification. The diagnostic score had a global performance of 0.9917 and correctly classified 97% of the patients, with a sensitivity of 92%, a specificity of 99%, a positive predictive value of 97%, and a negative predictive value of 97% for the diagnosis of AH.
    [Show full text]
  • Synchronous Morphologically Distinct Craniopharyngioma and Pituitary
    orders & is T D h e n r Bhatoe et al., Brain Disord Ther 2016, 5:1 i a a p r y B Brain Disorders & Therapy DOI: 10.4172/2168-975X.1000207 ISSN: 2168-975X Case Report Open Access Synchronous Morphologically Distinct Craniopharyngioma and Pituitary Adenoma: A Rare Collision Entity Harjinder S Bhatoe*, Prabal Deb and Sudip Kumar Sengupta Institute of Neuroscience, Max Super Speciality Hospital, New Delhi, India Abstract While pituitary tumors and craniopharyngiomas share a common lineage, their simultaneous occurrence is distinctly rare. We present one such patient, an adult male with two distinct tumors, that were excised by two different approaches. Relevant literature is briefly reviewed. Keywords: Brain tumor; Collision tumor; Craniopharyngioma; Pituitary tumor Introduction Simultaneous occurrence of morphological distinct, discreet intracranial tumors sharing the same cell lineage is a rarity. Pituitary tumors and craniopharyngiomas share a common lineage. Simultaneous occurrence of these two tumors in the same patient is rare and has been reported only nine times so far (Table 1). While pituitary tumours are centred in the sella, craniopharyngiomas may occur anywhere from the pituitary gland to the third ventricle. Association of intra-third ventricular craniopharyngioma and growth hormone- Figure 1: Contrast MRI (T1-weighted sagittal) showing intra-third-ventricular secreting pituitary macroadenoma as two distinct, unconnected tumors craniopharyngioma and pituitary adenoma. occurring synchronously has not been reported so far. Case Report A 35-year-old male was admitted with six-month-history of generalized headache, gradual loss of vision and intermittent generalized tonic clonic seizures. Clinically, he had acromegaly and optic atrophy with no perception of light.
    [Show full text]
  • From Isolated GH Deficiency to Multiple Pituitary Hormone
    European Journal of Endocrinology (2009) 161 S75–S83 ISSN 0804-4643 From isolated GH deficiency to multiple pituitary hormone deficiency: an evolving continuum – a KIMS analysis M Klose, B Jonsson1, R Abs2, V Popovic3, M Koltowska-Ha¨ggstro¨m4, B Saller5, U Feldt-Rasmussen and I Kourides6 Department of Medical Endocrinology, Copenhagen University Hospital, PE2131, Rigshospitalet, Blegdamsvej 9, DK-2100 Copenhagen, Denmark, 1Department of Women’s and Children’s Health, Uppsala University, SE-75185 Uppsala, Sweden, 2Department of Endocrinology, University of Antwerp, Antwerp, Belgium, 3Neuroendocrine Unit, Institute of Endocrinology, University Clinical Center Belgrade, Belgrade, Serbia, 4KIMS Medical Outcomes, Pfizer Endocrine Care, Sollentuna, Sweden, 5Pfizer Endocrine Care Europe, Tadworth, UK and 6Global Endocrine Care, Pfizer Inc., New York, New York 10017, USA (Correspondence should be addressed to M Klose; Email: [email protected]) Abstract Objective: To describe baseline clinical presentation, treatment effects and evolution of isolated GH deficiency (IGHD) to multiple pituitary hormone deficiency (MPHD) in adult-onset (AO) GHD. Design: Observational prospective study. Methods: Baseline characteristics were recorded in 4110 patients with organic AO-GHD, who were GH naı¨ve prior to entry into the Pfizer International Metabolic Database (KIMS; 283 (7%) IGHD, 3827 MPHD). The effect of GH replacement after 2 years was assessed in those with available follow-up data (133 IGHD, 2207 MPHD), and development of new deficiencies in those with available data on concomitant medication (165 IGHD, 3006 MPHD). Results: IGHD and MPHD patients had similar baseline clinical presentation, and both groups responded similarly to 2 years of GH therapy, with favourable changes in lipid profile and improved quality of life.
    [Show full text]
  • Current Medicine: Current Aspects of Thyroid Disease
    CURRENT MEDICINE CM CURRENT ASPECTS OF THYROID DISEASE J.H. Lazarus, Department of Medicine, University of Wales College of Medicine, Cardiff INTRODUCTION solved) the details of the immune dysfunction in these During the past two decades or so, advances in immunology conditions. Since the description of the HLA system and and molecular biology have contributed to a substantial the association of certain haplotypes with autoimmune increase in our understanding of many aspects of the endocrine disease, including Graves’ disease and Hashimoto’s pathophysiology of thyroid disease.1 This review will thyroiditis, it was anticipated that the immunogenetics of indicate some of these advances prior to illustrating their these diseases would shed definitive light on their significance in clinical practice. Emphasis is placed on pathogenesis. This hope has only been partially achieved, recent developments and it is not intended to provide full and the relative risk of the HLA haplotype has been low; clinical descriptions. other factors must be sought. Developments in the understanding of the interaction between the T lymphocyte THYROID HORMONE ACTION and the presentation of antigen (e.g. co-stimulatory signals) Since the discovery that the thyroid hormone receptor is have enabled further immunological insight.5 However, derived from the erb c oncogene, it has been realised that the role of environmental factors should not be overlooked. there are two receptor types, alpha and beta, whose genes The ambient iodine concentration may affect the incidence are located on chromosomes 3 and 17 respectively.2 There of autoimmune thyroid disease, and iodine has documented is now known to be differential tissue distribution of the immune effects in the thyroid gland both in vitro and in active TRs alpha 1 and beta 1 and 2.
    [Show full text]
  • Clinical Characteristics of Pain in Patients with Pituitary Adenomas
    C Dimopoulou and others Pain in patients with 171:5 581–591 Clinical Study pituitary adenomas Clinical characteristics of pain in patients with pituitary adenomas C Dimopoulou1, A P Athanasoulia1,3, E Hanisch1, S Held1, T Sprenger2,4,5, T R Toelle2, J Roemmler-Zehrer3, J Schopohl3, G K Stalla1 and C Sievers1 1Department of Endocrinology, Max Planck Institute of Psychiatry, Kraepelinstrasse 2-10, 80804 Munich, Germany, Correspondence 2Department of Neurology, Technische Universita¨ tMu¨ nchen, Munich, Germany, 3Medizinische Klinik und Poliklinik should be addressed IV, Ludwig-Maximilians-University, Munich, Germany, 4Department of Neurology, University Hospital Basel, Basel, to C Sievers Switzerland and 5Division of Neuroradiology, Department of Radiology, University Hospital Basel, Basel, Email Switzerland [email protected] Abstract Objective: Clinical presentation of pituitary adenomas frequently involves pain, particularly headache, due to structural and functional properties of the tumour. Our aim was to investigate the clinical characteristics of pain in a large cohort of patients with pituitary disease. Design: In a cross-sectional study, we assessed 278 patients with pituitary disease (nZ81 acromegaly; nZ45 Cushing’s disease; nZ92 prolactinoma; nZ60 non-functioning pituitary adenoma). Methods: Pain was studied using validated questionnaires to screen for nociceptive vs neuropathic pain components (painDETECT), determine pain severity, quality, duration and location (German pain questionnaire) and to assess the impact of pain on disability (migraine disability assessment, MIDAS) and quality of life (QoL). Results: We recorded a high prevalence of bodily pain (nZ180, 65%) and headache (nZ178, 64%); adrenocorticotropic adenomas were most frequently associated with pain (nZ34, 76%). Headache was equally frequent in patients with macro- and microadenomas (68 vs 60%; PZ0.266).
    [Show full text]
  • Delayed Diagnosis of Pituitary Stalk Interruption Syndrome with Severe Recurrent Hyponatremia Caused by Adrenal Insufficiency
    Case report https://doi.org/10.6065/apem.2017.22.3.208 Ann Pediatr Endocrinol Metab 2017;22:208-212 Delayed diagnosis of pituitary stalk interruption syndrome with severe recurrent hyponatremia caused by adrenal insufficiency Kyung Mi Jang, MD1, Pituitary stalk interruption syndrome (PSIS) involves the occurrence of a thin Cheol Woo Ko, MD, PhD2 or absent pituitary stalk, hypoplasia of the adenohypophysis, and ectopic neurohypophysis. Diagnosis is confirmed using magnetic resonance imaging. 1Department of Pediatrics, Yeungnam Patients with PSIS have a variable degree of pituitary hormone deficiency and a University College of Medicine, 2 wide spectrum of clinical manifestations. The clinical course of the disease in our Daegu, Department of Pediatric patient is similar to that of a syndrome of inappropriate antidiuretic hormone Endocrinology, Kyungpook National University Children’s Hospital, Daegu, secretion. This is thought to be caused by failure in the suppression of vasopressin Korea secretion due to hypocortisolism. To the best of our knowledge, there is no case report of a patient with PSIS presenting with hyponatremia as the first symptom in Korean children. Herein, we report a patient with PSIS presenting severe recurrent hyponatremia as the first symptom, during adolescence and explain the pathophysiology of hyponatremia with secondary adrenal insufficiency. Keywords: Pituitary stalk interruption syndrome, Hyponatremia, Hypopituitarism, Inappropriate ADH syndrome, Adrenal insufficiency Introduction Pituitary stalk interruption syndrome (PSIS) involves the occurrence of a thin or absent pituitary stalk, hypoplasia of the adenohypophysis, and ectopic neurohypophysis1). Diagnosis of this disease is confirmed using magnetic resonance imaging (MRI). PSIS was first reported in 1987, based on MRI results1).
    [Show full text]
  • Incidental Pituitary Adenomas
    Neurosurg Focus 31 (6):E18, 2011 Incidental pituitary adenomas WALAVAN SIVAKUMAR, M.D.,1 ROUKOZ CHAMOUN, M.D.,1 VINH NGUYEN, M.D.,2 AND WILLIAM T. COULdwELL, M.D., PH.D.1 Departments of 1Neurosurgery and 2Radiology, University of Utah, Salt Lake City, Utah Object. Pituitary incidentalomas are a common finding with a poorly understood natural history. Over the last few decades, numerous studies have sought to decipher the optimal evaluation and treatment of these lesions. This paper aims to elucidate the current evidence regarding their prevalence, natural history, evaluation, and management. Methods. A search of articles on PubMed (National Library of Medicine) and reference lists of all relevant ar- ticles was conducted to identify all studies pertaining to the incidence, natural history, workup, treatment, and follow- up of incidental pituitary and sellar lesions, nonfunctioning pituitary adenomas, and incidentalomas. Results. The reported prevalence of pituitary incidentalomas has increased significantly in recent years. A com- plete history, physical, and endocrinological workup with formal visual field testing in the event of optic apparatus involvement constitutes the basics of the initial evaluation. Although data regarding the natural history of pituitary incidentalomas remain sparse, they seem to suggest that progression to pituitary apoplexy (0.6/100 patient-years), visual field deficits (0.6/100 patient-years), and endocrine dysfunction (0.8/100 patient-years) remains low. In larger lesions, apoplexy risk may be higher. Conclusions. While the majority of pituitary incidentalomas can be managed conservatively, involvement of the optic apparatus, endocrine dysfunction, ophthalmological symptoms, and progressive increase in size represent the main indications for surgery.
    [Show full text]
  • Multiple Endocrine Neoplasia Type 1 (MEN1)
    Lab Management Guidelines v2.0.2019 Multiple Endocrine Neoplasia Type 1 (MEN1) MOL.TS.285.A v2.0.2019 Introduction Multiple Endocrine Neoplasia Type 1 (MEN1) is addressed by this guideline. Procedures addressed The inclusion of any procedure code in this table does not imply that the code is under management or requires prior authorization. Refer to the specific Health Plan's procedure code list for management requirements. Procedures addressed by this Procedure codes guideline MEN1 Known Familial Mutation Analysis 81403 MEN1 Deletion/Duplication Analysis 81404 MEN1 Full Gene Sequencing 81405 What is Multiple Endocrine Neoplasia Type 1 Definition Multiple Endocrine Neoplasia Type 1 (MEN1) is an inherited form of tumor predisposition characterized by multiple tumors of the endocrine system. Incidence or Prevalence MEN1 has a prevalence of 1/10,000 to 1/100,000 individuals.1 Symptoms The presenting symptom in 90% of individuals with MEN1 is primary hyperparathyroidism (PHPT). Parathyroid tumors cause overproduction of parathyroid hormone which leads to hypercalcemia. The average age of onset is 20-25 years. Parathyroid carcinomas are rare in individuals with MEN1.2,3,4 Pituitary tumors are seen in 30-40% of individuals and are the first clinical manifestation in 10% of familial cases and 25% of simplex cases. Tumors are typically solitary and there is no increased prevalence of pituitary carcinoma in individuals with MEN1.2,5 © eviCore healthcare. All Rights Reserved. 1 of 9 400 Buckwalter Place Boulevard, Bluffton, SC 29910 (800) 918-8924 www.eviCore.com Lab Management Guidelines v2.0.2019 Prolactinomas are the most commonly seen pituitary subtype and account for 60% of pituitary adenomas.
    [Show full text]