Document Detail


A nationwide clinical survey of patients with multiple endocrine neoplasia type 2 and familial medullary thyroid carcinoma in Japan.
MedLine Citation:
PMID:  9255265     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
MEN (multiple endocrine neoplasia) type 2 syndrome is an inherited disease characterized by medullary thyroid carcinoma, pheochromocytoma, hyperparathyroidism and/or developmental anomalies. Germ-line mutations of the RET proto-oncogene have recently been identified as the underlying cause of the syndrome. Accordingly, several investigators have advocated prophylactic total thyroidectomy for medullary thyroid carcinoma at an early age in MEN 2 gene carriers identified by DNA analysis. Before applying this strategy in Japan, the biological behavior of each category of tumor in MEN 2 syndrome, and medullary thyroid carcinoma in particular, should be well understood. We conducted a nationwide questionnaire survey to clarify the clinicopathological features of MEN 2 in Japan, obtaining data for 230 patients diagnosed as having MEN 2. They included 84 males and 146 females, with a median age of 37.5 years (range 5-83). Patients were categorized as 179 with MEN 2A, 17 with MEN 2B, 12 with familial medullary thyroid carcinoma and 22 'other'. Medullary thyroid carcinoma, pheochromocytoma and parathyroid lesions occurred in 224 (97%), 132 (57%) and 25 (11%) patients respectively. Twelve patients (5.2%) died of medullary thyroid carcinoma and 11 patients died of other or unknown causes. Of 163 patients for whom follow-up data were obtained, 82 (50%) experienced recurrences of medullary thyroid carcinoma, including symptomatic recurrent tumors in 24 patients and elevated calcitonin levels alone in 54. In the era of RET mutational analysis for screening relatives of patients with MEN 2, these data provide useful information about surgical management for patients with MEN 2 in Japan.
Authors:
M Iihara; T Yamashita; T Okamoto; M Kanbe; K Yamazaki; S Egawa; K Yamaguchi; T Obara
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Japanese journal of clinical oncology     Volume:  27     ISSN:  0368-2811     ISO Abbreviation:  Jpn. J. Clin. Oncol.     Publication Date:  1997 Jun 
Date Detail:
Created Date:  1997-08-29     Completed Date:  1997-08-29     Revised Date:  2012-06-04    
Medline Journal Info:
Nlm Unique ID:  0313225     Medline TA:  Jpn J Clin Oncol     Country:  JAPAN    
Other Details:
Languages:  eng     Pagination:  128-34     Citation Subset:  IM    
Affiliation:
Department of Endocrine Surgery, Tokyo Women's Medical College, Japan.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Aged
Aged, 80 and over
Carcinoma, Medullary / epidemiology*,  genetics,  surgery
Child
Child, Preschool
Drosophila Proteins*
Female
Germ-Line Mutation*
Humans
Japan / epidemiology
Male
Middle Aged
Multiple Endocrine Neoplasia Type 2a / epidemiology*,  genetics,  surgery
Multiple Endocrine Neoplasia Type 2b / epidemiology*,  genetics,  surgery
Parathyroid Neoplasms / etiology,  genetics
Pheochromocytoma / epidemiology,  genetics
Proto-Oncogene Proteins / genetics*
Proto-Oncogene Proteins c-ret
Receptor Protein-Tyrosine Kinases / genetics*
Thyroid Neoplasms / epidemiology*,  genetics,  surgery
Thyroidectomy
Chemical
Reg. No./Substance:
0/Drosophila Proteins; 0/Proto-Oncogene Proteins; EC 2.7.10.1/Proto-Oncogene Proteins c-ret; EC 2.7.10.1/Receptor Protein-Tyrosine Kinases; EC 2.7.10.1/Ret oncogene protein, Drosophila

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