[Multiple endocrine neoplasias in 3 generations].

Authors
Category Primary study
JournalLangenbecks Archiv fur Chirurgie
Year 1987
Typical in MEA IIb is a combination of medullary carcinoma of the thyroid gland and a pheochromocytoma with neurocutaneous and skeletal anomalies and a hyperplasia of the sympathetic nervous system of the GI tract with megacolon. Two patients of a family with MEA II are described. The family history reveals 1) an autosomal inheritance with high penetrance, 2) a changing pattern of anomalies in different generations, 3) no marfanoid traits as typical in MEA IIb, 4) only males affected by megacolon. This may be an uncommon variation or an additional type: IIc. Further investigation is required.
Epistemonikos ID: cf2742e1a316b85566a720024b77cfa4c09a6e95
First added on: Apr 14, 2022