Abstract. Medullary thyroid cancer is a type of thyroid cancer of neuroendocrine origin. It occurs in hereditary and sporadic forms, and its aggressive behavior is associated with the clinical presentation and type of RET mutation. Total thyroidectomy remains the ideal choice of treatment. Early diagnosis and treatment are the fundamental for a 100% cure rate. In this study, we present our experience of 3 cases, along with a complete review of the literature derived from a Pubmed Database search. IntroductionThyroid cancer represents approximately 2% of the malignancies occurring in the US, accounting for an estimated 37,200 cancer diagnoses and 1,630 cancer-related deaths per year (1). Of these cancers, 2-3% are medullary thyroid cancer (MTC) (2,3). In the literature, sporadic carcinomas are described in 48-86% of patients and hereditary carcinomas in 14-52% of patients with MTC (4). In the familial forms of MTC, multicentric carcinomas were reported in 56-85% of patients. Both sporadic and hereditary types of MTC metastasize to the cervical lymph nodes in 68-80% of patients. The 5-and 10-year survival for medullary carcinomas is 65-89% and 71-87%, respectively (5). Average survival for MTC is lower than that for more common thyroid cancers, e.g., 83% 5-year survival for MTC compared to 90-94% 5-year survival for papillary and follicular thyroid cancer (6). Survival is correlated with stage at diagnosis, and decreased survival in MTC can be partly accounted for by a high proportion of late-stage diagnoses (7). A Surveillance, Epidemiology and End Results (SEER) population-based study of 1,252 MTC patients found that survival varied by the extent of local disease. For example, the 10-year survival rates ranged from 95.6% for disease confined to the thyroid gland to 40% for patients with distant metastases (8).MTC arises from parafollicular calcitonin-secreting cells of the thyroid gland. MTC occurs in sporadic and familial forms and may be preceded by C-cell hyperplasia (CCH), although CCH is a relatively common abnormality in middle-aged adults. In a population-based study in Sweden, 26% of patients with MTC presented with the familial form (9). A French national registry and a US clinical series both reported a higher proportion of familial cases (43 and 44%, respectively) (10). Familial cases often indicate the presence of multiple endocrine neoplasia type 2, a group of autosomal dominant genetic disorders caused by inherited mutations in the RET proto-oncogene.MTC is a malignancy arising from the parafollicular or C-cells of neuroendocrine origin. MTC was first described by Jaquet in the German literature as ʻmalignant goiter with amyloidʼ (11). C-cells are named due to their calcitonin hormone secretion and account for up to 1% of thyroid cells. These cells are found throughout the thyroid gland but are mostly located in the posterior upper third of the lateral lobes, where the majority of MTCs are found. C-cells also produce carcinoembryonic antigen (CEA) (12).MTCs usually have a slow growth rate and appea...

This analysis shows that TBV has several advantages over AVG and provides a valuable access for HD but raises the need for a comparative trial between TBV and the newer generation AVGs. There is no clear superiority of the one-stage over the two-stage procedure.

Objective: Medullary thyroid cancer (MTC) is a rare and particularly aggressive type of thyroid cancer of neuroendocrine origin. It occurs in hereditary and sporadic forms and its aggressiveness is related to the clinical presentation and the type of RET mutation. Methods: In this article, we present the criteria, as reviewed in contemporary literature, regarding lymph node dissection and radical neck dissection in patients with either sporadic or hereditary MTC. Results: Early diagnosis and treatment remains the key to a 100% cure rate. Conclusions: Routine central lymph node dissection is the minimum procedure recommended for all sporadic and hereditary MTCs. Routine lateral lymph node dissection on either side is necessary when lymph node metastases are found in the central neck compartment.

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