Japanese Journal of Clinical Oncology, Vol 27, Issue 3 128-134, Copyright © 1997 by Foundation for Promotion of Cancer Research
M Iihara, T Yamashita, T Okamoto, M Kanbe, K Yamazaki, S Egawa, K Yamaguchi and T Obara
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.
ORIGINAL ARTICLE
A nationwide clinical survey of patients with multiple endocrine neoplasia type 2 and familial medullary thyroid carcinoma in Japan
Department of Endocrine Surgery, Tokyo Women's Medical College, Japan.
CiteULike 
Connotea 
Del.icio.us What's this?
This article has been cited by other articles:
|
|
 |
|
  A. Machens, M. Brauckhoff, H.-J. Holzhausen, P. N. Thanh, H. Lehnert, and H. Dralle Codon-Specific Development of Pheochromocytoma in Multiple Endocrine Neoplasia Type 2 J. Clin. Endocrinol. Metab., July 1, 2005; 90(7): 3999 - 4003. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 G. Szinnai, C. Meier, P. Komminoth, and U. W. Zumsteg Review of Multiple Endocrine Neoplasia Type 2A in Children: Therapeutic Results of Early Thyroidectomy and Prognostic Value of Codon Analysis Pediatrics, February 1, 2003; 111(2): e132 - 139. [Abstract] [Full Text] [PDF]
|
|