8345/3 Medullary carcinoma with amyloid stroma


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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