Medullary carcinoma of the thyroid gland

Abstract

One hundred thirty-nine surgically treated patients with medullary carcinoma of the thyroid gland (MTC) were seen in our institution between January, 1926 and December, 1973. The incidence of this tumor among all thyroid cancers was 8%. Twenty-nine patients had the familial form of MTC; they were subclassified, on the basis of the phenotype, into a group of 15 patients with Sipple syndrome (or multiple endocrine neoplasia (MEN) Type 2A) and a group of 14 patients with mucosal-neuroma phenotype (or MEN type 2B). Better survivorship was seen in the younger patients, in those with bilateral tumors, in familial MTC, and in patients whose tumor was confined to the thyroid gland at the initial surgery. The incidence of tumor recurrence was 34%. However, in those with adequate surgical treatment, the recurrence was only 23%. The 5- and 10-year survivorships were 80% and 67%, respectively. The best chance of cure lies in early diagnosis through the use of immunoreactive calcitonin measurement in family members at risk, and an aggressive surgical attack on the primary tumor and any cervical metastases.

Management of Rare Thyroid Malignancies

“…It is important to note that the parafollicular c-cells also produce carcinoembryonic antigen (CEA), prostaglandin, and serotonin of which excess levels could lead to symptoms [10]. Males and females are equally affected, typically over the age of 50 [51,52].…”

“…The majority of MTC, approximately 75%, occurs in a sporadic fashion and generally are unilateral and unifocal [50,52]. The remaining MTC is derived from an autosomal dominant hereditary pattern associated with the RET proto-oncogene.…”

Multiple endocrine neoplasia type 2: recent progress in diagnosis and management

“…MEN-2A is characterized by the triad of C-cell disease (C-cell hyperplasia or MTC), pheochromocytoma, and hyperparathyroidism (6).…”

“…Medullary thyroid carcinoma (MTC) is the key component of MEN-2 and largely determines its mortality (2)(3)(4)(5)(6). MEN-2 is caused by germline mutations in the RET proto-oncogene and is transmitted to the offspring in an autosomal dominant fashion (7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17).…”

A comprehensive review on MEN2B

“…The term multiple endocrine neoplasia type 2 was proposed by Steiner a in 1968 (Steiner et al 1968) and the rare association of hereditary MTC with a mucosal neuroma phenotype was eventually named multiple endocrine neoplasia type 2B (MEN2B) by Chong andcoworkers in 1975 (Chong et al 1975). The phenotypic specificities of MEN2B compared with MEN2A include prominent extra-endocrine features, a more aggressive presentation of MTC, and the lack of primary hyperparathyroidism.…”