Modak Baishakhi, Godkhindi Vishwapriya Mahadev, Venkadasalapathy Narayana, Radhakrishnan Raghu
Department of Oral and Maxillofacial Pathology, Manipal College of Dental Sciences, Manipal Academy of Higher Education, Manipal, Karnataka, 576104, India.
Mangalore Institute of Oncology, Mangaluru, Karnataka, 575007, India.
Head Neck Pathol. 2025 Jul 29;19(1):93. doi: 10.1007/s12105-025-01816-9.
Solitary fibrous tumors encompass a heterogeneous group of spindle cell neoplasms, ranging from biologically low-risk lesions to, in rare instances, highly aggressive tumors with malignant potential. Dedifferentiation in solitary fibrous tumors is uncommon and typically occurs in the retroperitoneum, with extrapleural involvement being among the least frequently reported.
A 13-year-old male presented with a rapidly enlarging mass in the lower jaw of 20 days duration, involving the submandibular triangle and floor of the mouth.
Histopathological examination of the excisional biopsy revealed spindle-shaped cells arranged in compact fascicles with a haphazard distribution, and areas of hyalinization. Immunohistochemical analysis demonstrated positivity for CD34, STAT6, MyoD1, α-SMA, Bcl-2, and CD99, confirming the diagnosis of extrapleural dedifferentiated solitary fibrous tumor (DSFT).
The lesion was surgically excised but recurred, likely due to disease progression. Re-excision was planned, but the child died 10 days before surgery.
孤立性纤维性肿瘤是一组异质性的梭形细胞瘤，范围从生物学低风险病变到罕见的具有恶性潜能的高度侵袭性肿瘤。孤立性纤维性肿瘤中的去分化并不常见，通常发生于腹膜后，胸膜外受累的报道极为罕见。
一名13岁男性患者，下颌出现迅速增大的肿块，持续20天，累及下颌下三角和口腔底部。
切除活检的组织病理学检查显示梭形细胞排列成紧密束状，分布杂乱，并有玻璃样变区域。免疫组织化学分析显示CD34、信号转导和转录激活因子6（STAT6）、肌分化抗原1（MyoD1）、α-平滑肌肌动蛋白（α-SMA）、Bcl-2和CD99呈阳性，确诊为胸膜外去分化孤立性纤维性肿瘤（DSFT）。
病变已手术切除，但复发，可能是由于疾病进展。计划再次切除，但患儿在手术前10天死亡。
