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

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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Publication Details

Journal
Diagnostic Pathology
Published
2025-04-02
DOI
https://doi.org/10.1186/s13000-025-01620-7
Primary Topic
Vascular Tumors and Angiosarcomas
Type
article
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article

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

Shiyu Cheng, Mei Du, Jie Shi, Yanli Li et al.
Diagnostic Pathology
Vascular Tumors and Angiosarcomas
article

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

Shiyu Cheng, Mei Du, Jie Shi, Yanli Li, Xiaorong Fan, Han Gao, Tingzhu Meng
article en

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.

Diagnostic PathologyVol. 20(1)
Wuhan University of Science and Technology (CN), Huazhong University of Science and Technology (CN)
Good health and well-being
Openalex Percentile: Top 90%
Vascular Tumors and Angiosarcomas
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A rare case of Alveolar Soft-Part Sarcoma in the Uterine cervix — Shiyu Cheng, Mei Du, et al. · Diagnostic Pathology (2025) | TGRS Research Map | TGRS