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甲状腺原发性孤立性纤维瘤:基于一例病例报告的综述

Primary solitary fibrous tumor of the thyroid gland: A review starting from a case report.

作者信息

Negură Ion, Ianole Victor, Dănilă Radu, Ungureanu Maria-Christina, Apostol Delia Gabriela Ciobanu

机构信息

Pathology Department, "Sf. Spiridon" Emergency County Clinical Hospital, Iasi, Romania.

Pathology Department, "Grigore T. Popa" University of Medicine and Pharmacy, Iasi, Romania.

出版信息

Arch Clin Cases. 2021 Dec 29;8(4):97-105. doi: 10.22551/2021.33.0804.10193. eCollection 2021.

Abstract

Primary solitary fibrous tumor (SFT) of the thyroid gland is a rare mesenchymal tumor with fibroblastic differentiation, ramified, thin-walled, enlarged (staghorn) vessels and specific NAB2-STAT6 gene fusion, which is more commonly found in pleura and peritoneum. This neoplasm can be located in a variety of anatomical sites outside pleura and peritoneum including bone, visceral organs and soft tissues, head and neck examples representing only 10-15% of the extra-pleural and extra-peritoneal tumors. Diagnosing this entity can be difficult, especially in thyroid gland, mainly because of the rarity of this neoplasm, but presence of characteristic microscopic features together with positivity for STAT6 and CD34 can confirm the diagnosis and exclude other differential diagnosis. Information about the diagnosis and treatment options of thyroid SFTs is limited but almost all primary thyroid SFTs have a good prognosis and indolent clinical course. Clinical surveillance is still necessary because some SFTs can be aggressive. Raising awareness regarding extra-pleural and extra-peritoneal location of this tumor in endocrine organs can help to better manage these patients. We report the case of a 34-year-old female with primary SFT of the thyroid gland. Additionally, we review the literature for the main clinical, paraclinical and pathological features of this neoplasm.

摘要

甲状腺原发性孤立性纤维瘤(SFT)是一种罕见的具有成纤维细胞分化的间叶性肿瘤,有分支状、薄壁、扩张(鹿角状)血管以及特异性的NAB2 - STAT6基因融合,该肿瘤更常见于胸膜和腹膜。这种肿瘤可位于胸膜和腹膜外的多种解剖部位,包括骨骼、内脏器官和软组织,头颈部的病例仅占胸膜外和腹膜外肿瘤的10% - 15%。诊断这种疾病可能很困难,尤其是在甲状腺,主要原因是这种肿瘤罕见,但特征性的微观特征以及STAT6和CD34阳性可确诊并排除其他鉴别诊断。关于甲状腺SFT诊断和治疗选择的信息有限,但几乎所有原发性甲状腺SFT预后良好且临床过程惰性。仍需要临床监测,因为一些SFT可能具有侵袭性。提高对该肿瘤在内分泌器官中胸膜外和腹膜外位置的认识有助于更好地管理这些患者。我们报告一例34岁女性原发性甲状腺SFT病例。此外,我们回顾了该肿瘤主要临床、副临床和病理特征的文献。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/491b/8717007/87a55acafdeb/acc-08-04-97-g001.jpg

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