Du Mei, Li Yanli, Fan Xiaorong, Gao Han, Shi Jie, Cheng Shiyu, Meng Tingzhu
Medical College, Wuhan University of Science and Technology, Wuhan, China.
Department of Gynecology Hubei Province Maternal and Infant Health Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Diagn Pathol. 2025 Apr 2;20(1):33. doi: 10.1186/s13000-025-01620-7.
Alveolar soft-part sarcoma (ASPS), a rare and malignant neoplasm of soft tissues, comprises less than 1% of all soft-tissue sarcomas and is characterized by distinct histopathological and molecular markers. A 27-year-old female presented with a history of postcoital vaginal bleeding and intermittent bleeding over the preceding month. Imaging studies identified abnormal echogenicity and vascular patterns in the posterior cervical lip. Initial histopathological assessment indicated a perivascular epithelioid cell tumor (PEComa) with TFE3 gene rearrangement; however, subsequent immunohistochemical and molecular analyses corroborated the diagnosis of ASPS. The patient underwent a total laparoscopic hysterectomy with bilateral salpingo-oophorectomy. Postoperative pathology revealed that the residual tumor was confined to the inner third of the cervix, with no evidence of lymphovascular or perineural invasion. The patient did not receive adjuvant therapy and was followed for three months postoperatively, during which no recurrence or metastasis was observed. Given the extreme rarity of ASPS, its diagnosis necessitates meticulous scrutiny by pathologists to inform and guide subsequent therapeutic approaches.
肺泡软组织肉瘤(ASPS)是一种罕见的软组织恶性肿瘤,占所有软组织肉瘤的比例不到1%,具有独特的组织病理学和分子标志物。一名27岁女性,有性交后阴道出血病史,且在前一个月出现间歇性出血。影像学检查发现宫颈后唇有异常回声和血管形态。初步组织病理学评估显示为伴有TFE3基因重排的血管周上皮样细胞瘤(PEComa);然而,随后的免疫组织化学和分子分析证实了ASPS的诊断。患者接受了全腹腔镜子宫切除术及双侧输卵管卵巢切除术。术后病理显示残留肿瘤局限于宫颈内三分之一,无淋巴管或神经周围侵犯迹象。患者未接受辅助治疗,术后随访三个月,期间未观察到复发或转移。鉴于ASPS极为罕见,其诊断需要病理学家进行细致检查,以便为后续治疗方法提供依据和指导。