Solitary Fibrous Tumor of the Mandible.

IF 3.2 Q2 PATHOLOGY

Head & Neck Pathology Pub Date : 2024-11-26 DOI:10.1007/s12105-024-01731-5

Thamyres Campos Fonsêca, Michelle Agostini, Jefferson Viapiana Paes, Ana Luiza Oliveira Corrêa Roza, Willie F P van Heerden, Mário José Romañach, Aline Corrêa Abrahão

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引用次数: 0

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.

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下颌骨单发纤维瘤

一名 41 岁的妇女因面部精神区不对称，右下颊前庭出现一个疼痛、周界清楚、有触痛的肿块，伴有广泛的界限不清的骨质稀疏，皮质骨吸收不明显。显微镜下观察到平淡的纺锤形细胞增生，其间夹杂着胶原纤维和突出的鹿角状血管。免疫组化分析显示，CD34、Bcl-2、CD99和STAT-6呈强阳性，确诊为孤立性纤维瘤（SFT）。患者接受了保守性手术切除，4 年后发现复发，骨质广泛受累，边缘糜烂，类似恶性肿瘤。SFT 是一种纤维母细胞分化的独特纺锤形细胞肿瘤，其特征是突出的鹿角状分支血管和特异的 NAB2::STAT6 基因融合。我们在此报告了一例下颌骨中央型 SFT，其行为和影像学表现均显示为复发性恶性肿瘤。

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来源期刊

Head & Neck Pathology PATHOLOGY-

CiteScore

5.70

自引率

9.50%

发文量

期刊介绍： Head & Neck Pathology presents scholarly papers, reviews and symposia that cover the spectrum of human surgical pathology within the anatomic zones of the oral cavity, sinonasal tract, larynx, hypopharynx, salivary gland, ear and temporal bone, and neck. The journal publishes rapid developments in new diagnostic criteria, intraoperative consultation, immunohistochemical studies, molecular techniques, genetic analyses, diagnostic aids, experimental pathology, cytology, radiographic imaging, and application of uniform terminology to allow practitioners to continue to maintain and expand their knowledge in the subspecialty of head and neck pathology. Coverage of practical application to daily clinical practice is supported with proceedings and symposia from international societies and academies devoted to this field. Single-blind peer review The journal follows a single-blind review procedure, where the reviewers are aware of the names and affiliations of the authors, but the reviewer reports provided to authors are anonymous. Single-blind peer review is the traditional model of peer review that many reviewers are comfortable with, and it facilitates a dispassionate critique of a manuscript.