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下颌骨孤立性纤维瘤。

Solitary Fibrous Tumor of the Mandible.

机构信息

Department of Oral Diagnosis and Pathology, School of Dentistry, Federal University of Rio de Janeiro (UFRJ), Av. Professor Rodolpho Paulo Rocco, 325, 1º andar, Rio de Janeiro, 21941- 913, Brazil.

School of Dentistry, University of Planalto Catarinense, Lages, Santa Catarina, Brazil.

出版信息

Head Neck Pathol. 2024 Nov 26;18(1):124. doi: 10.1007/s12105-024-01731-5.

Abstract

A 41-year-old woman presented with a facial asymmetry in the mental region and a painful, well-circumscribed, tender mass in the right lower buccal vestibule, associated with extensive ill-defined bone rarefaction with subtle cortical bone resorption. Microscopically, a proliferation of bland spindle cells interspersed with collagen fibers and prominent staghorn-like blood vessels was observed. Immunohistochemical analysis revealed strong positivity for CD34, Bcl-2, CD99, and STAT-6, confirming the diagnosis of Solitary Fibrous Tumor (SFT). Conservative surgical enucleation was performed, and 4 years later, recurrence was observed with extensive bone involvement and moth-eaten margins resembling a malignant tumor. SFT is a distinctive spindle cell tumor of fibroblastic differentiation, characterized by prominent branching staghorn-like vessels and a specific NAB2::STAT6 gene fusion. We herein contribute with a central SFT of the mandible with recurrent behavior and radiographic appearance suggesting malignancy.

摘要

一位 41 岁女性因面部中线不对称和右下颊部有触痛、界限清楚、质软的肿块就诊,肿块处伴有广泛边界不清的骨稀疏,伴有轻微的皮质骨吸收。显微镜下观察到,大量的梭形细胞增生,其间穿插着胶原纤维和明显的鹿角状血管。免疫组织化学分析显示 CD34、Bcl-2、CD99 和 STAT-6 强阳性,明确诊断为孤立性纤维瘤(SFT)。行保守的手术剜除术,4 年后,复发并广泛累及骨骼,边缘呈虫蚀样,类似于恶性肿瘤。SFT 是一种具有独特梭形细胞分化的成纤维细胞肿瘤,特征为明显的分支鹿角状血管和特定的 NAB2::STAT6 基因融合。我们在此报道一例具有复发行为和放射学表现提示恶性肿瘤的下颌中央 SFT。

相似文献

1
Solitary Fibrous Tumor of the Mandible.下颌骨孤立性纤维瘤。
Head Neck Pathol. 2024 Nov 26;18(1):124. doi: 10.1007/s12105-024-01731-5.
3
Solitary Fibrous Tumor of the Tongue: a Case Report.舌部孤立性纤维瘤:一例报告
Head Neck Pathol. 2025 Jan 30;19(1):12. doi: 10.1007/s12105-024-01738-y.

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