Synonyms

Parafollicular cell carcinoma
C cell carcinoma

Definitions

Thyroid gland
ICD-O-3 topography code:	C73
ICD10:	C73

A malignant tumour of the thyroid gland showing C-cell differentiation. The majority of medullary carcinomas of the thyroid gland are associated with amyloid deposits and express calcitonin.
Medullary thyroid carcinoma (MTC)comprises 5 to 10% of all thyroid malignancies. Up to 25% of these tumours occur in the setting of multiple endocrine neoplasia type IIA (MEN2A)^OMIM ,caused by gain of function germline mutations in the RET proto-oncogene, with an autosomal dominant mode of inheritance¹

Ronald A. DeLellis, Ricardo V. Lloyd, Philipp U. Heitz, Charis Eng
World Health Organization Classification of Tumours. Pathology and Genetics of Tumors of Endocrine Organs
IARC Press: Lyon 2004

. The etiology of sporadic MTC is unknown. Limited data suggest that chronic hypercalcemia may be a risk factor ²

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Livolsi VA, Feind CR (1979)
Incidental medullary thyroid carcinoma in sporadic hyperparathyroidism. An expansion of the concept of C-cell hyperplasia.
Am J Clin Pathol 71: 595-9

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Medullary carcinoma tends to metastasize early, particularly to cervical lymph nodes. Distant metastases are found at operation in approximately 20% of patients who present with palpable lesions ³

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Bergholm U, Adami HO, Bergström R, Johansson H, Lundell G, Telenius-Berg M, Akerström G (1989)
Clinical characteristics in sporadic and familial medullary thyroid carcinoma. A nationwide study of 249 patients in Sweden from 1959 through 1981.
Cancer 63: 1196-204

. United States SEER data showed 5- and 10-year survival rates of 83% and 74%, respectively ⁴

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Bhattacharyya N (2003)
A population-based analysis of survival factors in differentiated and medullary thyroid carcinoma.
Otolaryngol Head Neck Surg 128: 115-23

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