Zhang Xiaowei, Yan Chen, Xu Tingting, Ying Jiajia
Department of Pathology, Affiliated Dongyang Hospital of Wenzhou Medical University, Dongyang, Zhejiang, China.
Department of Surgical Center, Affiliated Dongyang Hospital of Wenzhou Medical University, Dongyang, Zhejiang, China.
Front Surg. 2024 Sep 13;11:1417263. doi: 10.3389/fsurg.2024.1417263. eCollection 2024.
Lipofibromatosis-like neural tumors (LPF-NT), which have only recently been established, are intermediate soft tissue tumors with neurotrophic tropomyosin receptor kinase 1 ( gene alterations and are typically misdiagnosed as dermatofibrosarcoma protuberans, low-grade malignant peripheral nerve sheath tumors, or spindle cell lipoma due to their histopathological and immunohistochemical expression of CD34 and S-100.
The patient was admitted to our hospital with a painless back mass that had appeared more than 4 years prior to admission. Physical examination revealed a subcutaneous mass on the back, approximately 1.5 cm in diameter and protruding into the skin, with clear boundaries and no tenderness. The tumor was surgically resected. The postoperative pathological results suggested a spindle cell soft tissue tumor, and dermatofibrosarcoma protuberan was initially considered. After consultation at a provincial hospital, the patient was diagnosed with a cutaneous lipofibromatosis-like neural tumor of the back. A second extended resection was then performed. Intraoperative rapid freezing examination revealed negative incision margins.
Histological and immunohistochemical detections aid in the differential diagnosis of LPF-NTs. Complete surgical resection is the preferred treatment for LPF-NTs.
脂肪纤维瘤样神经肿瘤（LPF-NT）是一种新近才被确认的中间型软组织肿瘤，具有神经营养性原肌球蛋白受体激酶1（基因改变，由于其组织病理学表现以及CD34和S-100的免疫组化表达，通常被误诊为隆突性皮肤纤维肉瘤、低级别恶性外周神经鞘瘤或梭形细胞脂肪瘤。
患者因入院前4年多出现的无痛性背部肿块入住我院。体格检查发现背部有一个皮下肿块，直径约1.5厘米，突出于皮肤，边界清晰，无压痛。肿瘤被手术切除。术后病理结果提示为梭形细胞软组织肿瘤，最初考虑为隆突性皮肤纤维肉瘤。经省级医院会诊，患者被诊断为背部皮肤脂肪纤维瘤样神经肿瘤。随后进行了第二次扩大切除术。术中快速冰冻检查显示切缘阴性。
组织学和免疫组化检测有助于LPF-NT的鉴别诊断。完整的手术切除是LPF-NT的首选治疗方法。
