1. [Multiple endocrine adenomatosis IIB].
- Author
-
Dohán O, Höppner W, Salamon F, Góth M, Kovács L, Szilágyi G, and Szabolcs I
- Subjects
- Adolescent, Humans, Male, Proto-Oncogenes, Thyroid Neoplasms diagnostic imaging, Thyroid Neoplasms pathology, Thyroid Neoplasms surgery, Ultrasonography, Multiple Endocrine Neoplasia Type 2b diagnostic imaging, Multiple Endocrine Neoplasia Type 2b genetics, Multiple Endocrine Neoplasia Type 2b pathology, Multiple Endocrine Neoplasia Type 2b surgery, Thyroid Neoplasms genetics
- Abstract
The first Hungarian MEN IIB (multiplex endocrine neoplasia) syndrome is reported with the short summary of the literature about the pathogenesis and diagnosis of medullary thyroid carcinoma, presenting 80% in sporadic, 20% in hereditary form. The appearance of the patients alone (marfanoid stature, bulky lips, and ganglioneuromatosis of the tongue) may be almost enough for the presumption for the diagnosis of MEN IIB: For screening and preventing the clinical manifestation of the very aggressive medullary carcinoma in the relatives of the patient, the genetic screening is indispensable. The costs of the genetic screening and early treatment of the patients are much lower than the expenses of the traditional annual biochemical screening and the--delayed, often only supportive--treatment of the clinically manifested illness.
- Published
- 1998