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

**Authors:** Mei Du, Yanli Li, Xiaorong Fan, Han Gao, Jie Shi, Shiyu Cheng, Tingzhu Meng

PMC · DOI: 10.1186/s13000-025-01620-7 · Diagnostic Pathology · 2025-04-02

## TL;DR

A rare case of Alveolar Soft-Part Sarcoma in the uterine cervix was diagnosed and surgically treated in a 27-year-old woman with no recurrence observed.

## Contribution

This paper presents a rare clinical case of ASPS in the uterine cervix, emphasizing the importance of accurate diagnosis.

## Key findings

- ASPS was confirmed through immunohistochemical and molecular analyses after initial misdiagnosis as PEComa.
- The tumor was confined to the inner third of the cervix with no lymphovascular or perineural invasion.
- No recurrence or metastasis was observed in the patient three months post-surgery without adjuvant therapy.

## 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.

## Linked entities

- **Genes:** TFE3 (transcription factor binding to IGHM enhancer 3) [NCBI Gene 7030]
- **Diseases:** Alveolar Soft-Part Sarcoma (MONDO:0011655), PEComa (MONDO:0006359)

## Full-text entities

- **Genes:** TFE3 (transcription factor binding to IGHM enhancer 3) [NCBI Gene 7030] {aka MRXSPF, RCCP2, RCCX1, TFEA, bHLHe33}
- **Diseases:** vaginal bleeding (MESH:D014592), PEComa (MESH:D054973), malignant neoplasm (MESH:D009369), bleeding (MESH:D006470), ASPS (MESH:D018234), metastasis (MESH:D009362), soft-tissue sarcomas (MESH:D012509)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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## References

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Source: https://tomesphere.com/paper/PMC11963694