Medullary Thyroid Carcinoma – Update and Present Management Controversies

Al-Rawi, Mahir A.; Wheeler, Malcolm H.

doi:10.1308/003588406x117043

Cited by 41 publications

(21 citation statements)

References 27 publications

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“…Medullary thyroid carcinoma (MTC) arises from the C cells (calcitonin cells) and accounts for between 5% and 10% of thyroid carcinomas [117][118][119]. As this tumor is derived from C cells, those seeking identification and treatment for MTC are provided at least one invaluable immunohistochemical and biochemical marker, calcitonin.…”

Section: C-cell Neoplasmsmentioning

confidence: 99%

Review: The Role of Neural Crest Cells in the Endocrine System

Adams

Bronner‐Fraser

2009

Endocr Pathol

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The neural crest is a pluripotent population of cells that arises at the junction of the neural tube and the dorsal ectoderm. These highly migratory cells form diverse derivatives including neurons and glia of the sensory, sympathetic, and enteric nervous systems, melanocytes, and the bones, cartilage, and connective tissues of the face. The neural crest has long been associated with the endocrine system, although not always correctly. According to current understanding, neural crest cells give rise to the chromaffin cells of the adrenal medulla, chief cells of the extra-adrenal paraganglia, and thyroid C cells. The endocrine tumors that correspond to these cell types are pheochromocytomas, extra-adrenal paragangliomas, and medullary thyroid carcinomas. Although controversies concerning embryological origin appear to have mostly been resolved, questions persist concerning the pathobiology of each tumor type and its basis in neural crest embryology. Here we present a brief history of the work on neural crest development, both in general and in application to the endocrine system. In particular, we present findings related to the plasticity and pluripotency of neural crest cells as well as a discussion of several different neural crest tumors in the endocrine system.

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Section: C-cell Neoplasmsmentioning

confidence: 99%

Review: The Role of Neural Crest Cells in the Endocrine System

Adams

Bronner‐Fraser

2009

Endocr Pathol

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“…It is extremely uncommon for metastatic spread to occur to the lateral nodes without first involving the ipsilateral central nodes. Surgical excision is the only effective therapy for these metastases (26).…”

Section: Discussionmentioning

confidence: 99%

Medullary thyroid carcinoma: The third most common thyroid cancer reviewed

et al. 2010

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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...

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“…Before the genetic test was available, the decision for thyroidectomy was based on clinical evidence of carcinoma; positive laboratory screening tests, determined by the presence of hyperplasia of C-cells and/or cancerous cells; and the presence of the characteristic phenotype of MEN2B in patients with inheritance of this syndrome [9,12].…”

Section: Discussionmentioning

confidence: 99%