PDF(Pubmed)
Abstract：
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 radiotherapy, non-steroidal anti-inflammatory drugs, tyrosine kinase inhibitors and chemotherapy have also been used in selected cases. Due to the rarity of these tumors and the lack of specific treatment guidelines, management should be discussed in a multidisciplinary team.
摘要：
纤维样型纤维瘤病（DTF）是甲状腺乳头状癌（PTC）的一种非常罕见的变种。它本质上是一种双重肿瘤，具有恶性上皮增殖的经典PTC成分（BRAF突变）和间充质增殖的另一个成分（CTNNB1突变）。我们对PTC-DTF进行了文献综述。总的来说,确定了31篇文章，总共报告了54名患者。平均年龄是47岁,女性占比2.2:1。没有发现超声特征有助于区分PTC-DTF与其他PTC变体。在43例报告组织学细节的病例中，60%患有局部浸润性疾病(T3b或T4)。大约48%有颈淋巴结转移,但没有远处转移.虽然PTC-DTF在局部可能比经典PTC更具侵略性，它的整体行为是相似的，可以包括甲状腺外延伸和淋巴结转移，可能含有基质成分并显示结外侵入。PTC-DTF的主要治疗方法是手术，预计DTF成分对放射性碘不敏感。外部放射治疗,非甾体抗炎药，酪氨酸激酶抑制剂和化疗也已用于选定的病例。由于这些肿瘤的罕见性和缺乏具体的治疗指南，管理应该在多学科团队中讨论。
