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LONG‐TERM FOLLOW‐UP OF A KINDRED WITH MULTIPLE ENDOCRINE NEOPLASIA TYPE IIA
Author(s) -
Munn StephenR.,
Heerden JonA. Van,
Sizemore GlenW.,
Carney J. Aidan,
Paloyan Edward,
Gau FrederickC. J.
Publication year - 1989
Publication title -
australian and new zealand journal of surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.111
H-Index - 51
eISSN - 1445-2197
pISSN - 0004-8682
DOI - 10.1111/j.1445-2197.1989.tb01659.x
Subject(s) - medicine , medullary cavity , multiple endocrine neoplasia , thyroid carcinoma , pheochromocytoma , endocrine system , disease , endocrine disease , presentation (obstetrics) , medullary carcinoma , metastasis , thyroid , pediatrics , surgery , cancer , hormone , biochemistry , chemistry , gene
A kindred with multiple endocrine neoplasia type ha was studied. In four of five fully documented cases, patients presented with adrenal medullary disease during the second or third decade; in three cases, medullary thyroid carcinoma was not diagnosed until the fourth decade. This kindred seems to have an aggressive variant of adrenal medullary disease in which clinical presentation is early, metastasis occurs, and oncological cure is uncommon. Early detection and operation were important, and symptoms were controlled with operations and α and β‐adrenergic blockade in uncured members.