Diagnostic and Surgical Dilemmas in Hereditary Medullary Thyroid Carcinoma

被引：4

作者：

Allen, Shawn M. ^{[1
]}

Bodenner, Donald ^{[2
,3
]}

Suen, James Y. ^{[1
]}

Richter, Gresham T. ^{[1
]}

机构：

[1] Univ Arkansas Med Sci, Dept Otolaryngol Head & Neck Surg, Little Rock, AR 72205 USA

[2] Univ Arkansas Med Sci, Dept Geriatr, Little Rock, AR 72205 USA

[3] Cent Arkansas VA Med Ctr, Little Rock, AR USA

来源：

LARYNGOSCOPE | 2009年 / 119卷 / 07期

关键词：

Medullary thyroid carcinoma; familial medullar thyroid carcinoma; hereditary medullary thyroid carcinoma; diagnosis; therapy; dilemmas; MULTIPLE ENDOCRINE NEOPLASIA; NEEDLE-ASPIRATION-CYTOLOGY; SERUM CALCITONIN; ROUTINE MEASUREMENT; PREOPERATIVE DIAGNOSIS; RET PROTOONCOGENE; FOLLOW-UP; CANCER; MANAGEMENT; MUTATIONS;

D O I：

10.1002/lary.20299

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

Medullary thyroid carcinoma (MTC) is a rare malignancy arising from the parafollicular C cells within the thyroid gland. The majority of cases are sporadic, but at least 30% are hereditary in nature. Inherited forms of MTC occur as familial MTC or as a manifestation of multiple endocrine neoplasia type 2. Early diagnosis and aggressive surgical management, including prophylactic thyroidectomy, improve the prognosis of patients with hereditary MTC. Several issues regarding the diagnosis and treatment of MTC remain controversial. Genetic penetrance and virulence are variable. We present an index case of familial MTC to illustrate common difficulties in the initial diagnosis and dilemmas in the surgical approach, followed by a review of current literature relevant to the management of hereditary MTC.

引用

页码：1303 / 1311

页数：9

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