Cancers, Vol. 13, Pages 4482: Papillary Thyroid Carcinoma with Desmoid-Type Fibromatosis: Review of Published Cases

Cancers, Vol. 13, Pages 4482: Papillary Thyroid Carcinoma with Desmoid-Type Fibromatosis: Review of Published Cases Cancers doi: 10.3390/cancers13174482 Authors: Abdallah Roukain Stefano La Rosa Massimo Bongiovanni Marie Nicod Lalonde Valérie Cristina Michael Montemurro Stephane Cochet Alexandra Luquain Peter A. Kopp Gerasimos P. Sykiotis Desmoid-type fibromatosis (DTF) is a very rare variant of papillary thyroid carcinoma (PTC). It is essentially a dual tumor with a component of classical PTC with malignant epithelial proliferation (BRAF-mutated) and another component of mesenchymal proliferation (CTNNB1-mutated). We conducted a literature review on PTC-DTF. In total, 31 articles were identified, that together reported on 54 patients. The mean age was 47 years, with a 2.2:1 female predominance. No ultrasound features were found to be helpful in differentiating PTC-DTF from other PTC variants. Of the 43 cases that reported histological details, 60% had locally infiltrative disease (T3b or T4). Around 48% had cervical lymph node metastases, but none had distant metastases. While PTC-DTF may be locally more aggressive than classic PTC, its overall behavior is similar and can include extrathyroidal extension and lymph node metastases, which may contain a stromal component and show extranodal invasion. The mainstay of treatment for PTC-DTF is surgery, and the DTF component is not expected to be sensitive to radioactive iodine. External radiotherap...
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Review Source Type: research

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