Papillary Thyroid Microcarcinoma

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Retrieved

2019-09-22

Source

Omim

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Genes

BRAF, RET, CCDC6, F9, PTCH1, TAS2R38, LOC110806263, NRAS, TG, HT, TSHR, VIM, PAX8, PPP1R13L, FOXP3, ZNF654, SHC3, ANKRD36B, ERAP2, MEDAG, MIR21, MIR221, MIR222, MIR324, MIR146B, VEGFA, ALK, TPO, CD82, CAV1, CD59, CDKN1B, CRYZ, NQO1, GDF1, HIF1A, LAMC2, TP53, LGALS3, NQO2, SERPINF1, PPARG, RNH1, S100A4, CCND1, SLC5A5

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The familial type of medullary thyroid carcinoma (155240) is a well-defined clinical entity. However, much less is known about the familial occurrence of differentiated thyroid carcinoma. Two patterns of presentation had been described for familial differentiated thyroid cancer: a pattern associated with an inherited tumor syndrome such as Gardner syndrome (APC; 175100) and Cowden disease (158350), and a second pattern of familial aggregation without other associated neoplasms. A further classification of thyroid tumors is based on size: papillary microcarcinoma of the thyroid is defined as a papillary carcinoma measuring 1.0 cm or less in diameter. This group of patients has been thought to be a specific low-risk category with a favorable prognosis (Hay et al., 1992). Lupoli et al. (1999) identified a family history of thyroid carcinoma in 7 of 119 patients with papillary thyroid microcarcinoma. The tumor was multifocal in 5 patients, bilateral in 3, and showed vascular invasion in 3 of the 7 patients. Lymph node metastases were found in 4 patients. Three patients had a recurrence and 1 patient with pulmonary metastases died within 11 months. Thus, familial occurrence was observed in 5.9% of cases, together with an unfavorable behavior of the familial form of the disorder.