A Unique case of Multiple Endocrine Neoplasm-1 Syndrome satisfying all six WHO criteria

  • Rushabh Jitendra Shah Seth GS Medical College and KEM Hospital, Parel , Mumbai
  • Samruddhi Dilip Rajpurkar Seth GS Medical College and KEM Hospital, Parel , Mumbai
Keywords: Multiple Endocrine Neoplasia type 1, parathyroid hyperplasia, thymic carcinoid, pituitary adenoma

Abstract

Multiple Endocrine Neoplasia (MEN) syndromes are a group of genetically inherited diseases resulting in proliferative lesions (hyperplasia, adenomas and carcinomas) of the endocrine organs. MEN 1 syndrome principally includes tumors of parathyroid, pancreas and pituitary. Our case is a 45 year old male, previously operated for thymic carcinoma, subsequently presented with radiologically and pathologically confirmed neuroendocrine lesions involving pituitary, parathyroid, adrenal, pancreas, stomach and duodenum. This patient had a positive family history for MEN 1 syndrome thus satisfying all six WHO criteria for MEN 1 syndrome which is a rare observation with no previous published reports. Tumors in this syndrome are more aggressive and recur in higher proportion of cases than do similar tumors occurring sporadically in non-syndromic patients.

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Published
2016-10-03
Section
Case Report