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Medullary thyroid carcinoma
Author(s) -
Leboulleux Sophie,
Baudin Eric,
Travagli JeanPaul,
Schlumberger Martin
Publication year - 2004
Publication title -
clinical endocrinology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.055
H-Index - 147
eISSN - 1365-2265
pISSN - 0300-0664
DOI - 10.1111/j.1365-2265.2004.02037.x
Subject(s) - medicine , calcitonin , thyroid carcinoma , medullary cavity , pheochromocytoma , medullary thyroid cancer , disease , ret proto oncogene , thyroid , medullary carcinoma , pathological , pathology , oncology , germline mutation , mutation , biology , biochemistry , gene
Summary Medullary thyroid carcinoma (MTC) arises from parafollicular or C cells that produce calcitonin (CT), and accounts for 5–10% of all thyroid cancers. MTC is hereditary in about 25% of cases. The discovery of a MTC in a patient has several implications: disease extent should be evaluated, phaeochromocytoma and hyperparathyroidism should be screened for and whether the MTC is sporadic or hereditary should be determined by a direct analysis of the RET proto‐oncogene. In this review, pathological characteristics, tumour markers and genetic abnormalities in MTC are discussed. The diagnostic and therapeutic modalities applied to patients with clinical MTC and those identified with preclinical disease through familial screening are also described. Progresses concerning genetics, initial treatment, follow‐up, screening and treatment of pheochromocytoma have permitted an improvement in the long‐term outcome. However, there is no effective treatment for distant metastases, and new therapeutic modalities are urgently needed.