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Expanding the Cause of Hypopituitarism

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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
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