Children with multiple endocrine neoplasia type 2B: Not tall and marfanoid, but short with normal body proportions.

ABSTRACT:

Objective

Multiple endocrine neoplasia 2B (MEN2B) is characterised by early-onset medullary thyroid carcinoma (MTC), pheochromocytoma and several nonendocrine manifestations. Unfortunately, MEN2B is often diagnosed late, after the development of clinically significant MTC. Marfanoid habitus is considered an important related feature, which may lead to the assumption that patients with MEN2B have tall stature. Here, we describe the longitudinal growth and body proportions of eight MEN2B patients during childhood.

Design

It is a retrospective case series.

Methods

Patients were under the care of a Dutch MEN expertise centre. Growth patterns were assessed and interpreted in relation to body mass index (BMI), age at diagnosis and at thyroidectomy, extensiveness of disease

SUBMITTER: van den Broek MFM 

PROVIDER: S-EPMC8456974 | biostudies-literature | 2021 Sep

REPOSITORIES: biostudies-literature