Prevalence of pheochromocytoma and hyperparathyroidism in multiple endocrine neoplasia type 2A: results of long-term follow-up

J R Howe; J A Norton; S A Wells Jr

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Prevalence of pheochromocytoma and hyperparathyroidism in multiple endocrine neoplasia type 2A: results of long-term follow-up

Journal article

Peer reviewed

Prevalence of pheochromocytoma and hyperparathyroidism in multiple endocrine neoplasia type 2A: results of long-term follow-up

J R Howe, J A Norton and S A Wells Jr

Surgery, Vol.114(6), pp.1070-1077

12/1993

PMID: 7903003

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Abstract

Multiple endocrine neoplasia type 2A (MEN 2A) is an autosomal dominant condition in which virtually all affected kindred members have medullary thyroid carcinoma (MTC). However, the penetrance of pheochromocytoma and hyperparathyroidism in affected kindred members is variable, and the true prevalence of these neoplasms is unclear from previous studies. Members of MEN 2A kindreds with more than 10 years of follow-up screening for MTC, pheochromocytoma, or hyperparathyroidism were studied. The diagnosis of pheochromocytoma was based on histologic examination and hyperparathyroidism on both parathyroid hyperplasia plus preoperative elevation of serum calcium levels. Eighty-six patients operated on for MTC from 12 different MEN 2A kindreds were studied, with a mean follow-up of 12.9 years of screening for pheochromocytoma (79 patients) and 15.0 years for hyperparathyroidism (78 patients). Pheochromocytomas developed in 42% of patients with MTC, with a range of 6% to 100% in different kindreds. The prevalence of hyperparathyroidism was 35%, ranging from 0% to 53% between kindreds. The average age at diagnosis of MTC, pheochromocytoma, and hyperparathyroidism was 29, 37, and 36 years, respectively. We conclude that the penetrance of pheochromocytoma and hyperparathyroidism is variable in different kindreds with MEN 2A but that the overall prevalence of pheochromocytoma approximates 40% and that of hyperparathyroidism 35%.

Prevalence

Follow-Up Studies

Humans

Middle Aged

Child, Preschool

Thyroid Neoplasms - complications

Carcinoma, Medullary - complications

Hyperparathyroidism - complications

Hyperparathyroidism - epidemiology

Multiple Endocrine Neoplasia - complications

Pheochromocytoma - complications

Adrenal Gland Neoplasms - epidemiology

Adolescent

Adrenal Gland Neoplasms - complications

Adult

Aged

Child

Longitudinal Studies

Pheochromocytoma - epidemiology

Details

Title: Subtitle: Prevalence of pheochromocytoma and hyperparathyroidism in multiple endocrine neoplasia type 2A: results of long-term follow-up
Creators: J R Howe - Department of Surgery, Washington University School of Medicine, St. Louis, MO 63110
J A Norton
S A Wells Jr
Resource Type: Journal article
Publication Details: Surgery, Vol.114(6), pp.1070-1077
Publisher: United States
PMID: 7903003
ISSN: 0039-6060
eISSN: 1532-7361
Grant note: P01CA53524 / NCI NIH HHS 5T32 CA 09621 / NCI NIH HHS
Language: English
Date published: 12/1993
Academic Unit: Surgery
Record Identifier: 9984051988102771

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