A rare case of Alveolar Soft-Part Sarcoma in the Uterine cervix.

IF 2.3 3区 医学 Q2 PATHOLOGY Diagnostic Pathology Pub Date : 2025-04-02 DOI:10.1186/s13000-025-01620-7
Mei Du, Yanli Li, Xiaorong Fan, Han Gao, Jie Shi, Shiyu Cheng, Tingzhu Meng
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Abstract

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.

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宫颈肺泡性软组织肉瘤1例。
肺泡软组织肉瘤(Alveolar soft-part sarcoma, ASPS)是一种罕见的软组织恶性肿瘤,占所有软组织肉瘤的不到1%,具有独特的组织病理学和分子标记。27岁女性,前一个月有性交后阴道出血和间歇性出血史。影像学检查发现异常回声和血管模式在后颈唇。初步组织病理学检查提示血管周围上皮样细胞瘤(PEComa)伴TFE3基因重排;然而,随后的免疫组织化学和分子分析证实了ASPS的诊断。患者行腹腔镜全子宫切除术及双侧输卵管卵巢切除术。术后病理显示残余肿瘤局限于宫颈内三分之一,未见淋巴血管或神经周围浸润。患者未接受辅助治疗,术后随访3个月,无复发转移。鉴于ASPS极为罕见,其诊断需要病理学家仔细检查,以告知和指导后续的治疗方法。
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来源期刊
Diagnostic Pathology
Diagnostic Pathology 医学-病理学
CiteScore
4.60
自引率
0.00%
发文量
93
审稿时长
1 months
期刊介绍: Diagnostic Pathology is an open access, peer-reviewed, online journal that considers research in surgical and clinical pathology, immunology, and biology, with a special focus on cutting-edge approaches in diagnostic pathology and tissue-based therapy. The journal covers all aspects of surgical pathology, including classic diagnostic pathology, prognosis-related diagnosis (tumor stages, prognosis markers, such as MIB-percentage, hormone receptors, etc.), and therapy-related findings. The journal also focuses on the technological aspects of pathology, including molecular biology techniques, morphometry aspects (stereology, DNA analysis, syntactic structure analysis), communication aspects (telecommunication, virtual microscopy, virtual pathology institutions, etc.), and electronic education and quality assurance (for example interactive publication, on-line references with automated updating, etc.).
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