Complex Endocrinopathies in MEN-1: Diagnostic Dilemmas in Endocrine Oncology
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CASE REPORT
Complex Endocrinopathies in MEN-1: Diagnostic Dilemmas in Endocrine Oncology Nabeel Y. Y. Al Brahim & Gloria Rambaldini & Shereen Ezzat & Sylvia L. Asa
Published online: 5 April 2007 # Humana Press Inc. 2007
Abstract Endocrine oncology is a complex area that must determine the site of a neoplastic process and the hormonal dysregulation that ensues. Patients with endocrine tumors often have delayed diagnosis because of the nonspecific and often subtle signs and symptoms. In patients with multiple endocrine neoplasia syndromes, diagnosis and clinicopathologic correlations can be even more challenging. We report a patient with multiple endocrine neoplasia type 1 (MEN-1) and a highly complex clinical story associated with multiple atypical lesions including two pituitary adenomas, a gonadotroph macroadenoma and a corticotroph microadenoma with Crooke’s hyaline change and ectopic production of corticotropin-releasing hormone (CRH) from a thymic endocrine carcinoma. These lesions resulted in a highly complex clinical story, difficult diagnoses and questions about management. This case illustrates a number of clinically relevant challenges, including the diagnosis of pituitary adenomas in MEN-1, the difficulty in diagnosing Cushing’s disease, and the large differential of pituitary pathologies in this disorder, double pituitary adenomas and other decoy lesions in Cushing’s disease, the pathophysiolN. Y. Y. Al Brahim : S. L. Asa (*) Department of Pathology, University Health Network, 200 Elizabeth Street, 11th Floor, Toronto, Ontario M5G 2C4, Canada e-mail: [email protected] G. Rambaldini : S. Ezzat Department of Medicine (Endocrinology), Mount Sinai Hospital, Toronto, Ontario, Canada N. Y. Y. Al Brahim : G. Rambaldini : S. Ezzat : S. L. Asa The Ontario Cancer Institute, Toronto, Ontario, Canada N. Y. Y. Al Brahim : G. Rambaldini : S. Ezzat : S. L. Asa University of Toronto, Toronto, Ontario, Canada
ogy of Crooke’s hyaline change in the pituitary, and the various causes of Cushing’s syndrome associated with MEN-1. Keywords Cushing's syndrome . multiple endocrine neoplasia . Crooke's hyaline . ectopic hormone production
Introduction Cushing’s syndrome is a highly complex pathophysiological entity. It may be caused by primary adrenal disorders, pituitary adrenocorticotropic hormone (ACTH) hypersecretion, or ectopic ACTH. It represents one of the most challenging endocrine diagnoses. Multiple endocrine neoplasia type 1 (MEN-1) represents another challenge to the endocrinologist. Patients can develop hormonally active neoplasms in pituitary, parathyroid, pancreas, adrenal, thymus, and other sites. The evaluation and management of a patient with this disorder requires astute knowledge of the multiple possible manifestations of this disorder. We present an unusual case of Cushing’s syndrome in a patient with MEN-1 that illustrates many aspects of endocrine pathology.
Case Report A 54-year-old man was referred for investigation of Cushing’s syndrome. His past medical history was remarkable for a constellation o
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