Case report: alveolar soft tissue sarcoma of the vagina
DOI:
https://doi.org/10.5327/2237-4574-125%20Keywords:
alveolar soft tissue sarcoma, ASPSCR1, TFE3Abstract
Alveolar soft tissue sarcoma is a rare malignancy, accounting for 0.5–1% of all soft tissue sarcomas. The participation of the ASPSCR1 and TFE3 genes, as well as the translocation (X; 17) (p11; q25), were demonstrated in this type of sarcoma. Complete resection of the sarcoma with adequate margins is often the first line of treatment, and may be associated with chemotherapy and radiotherapy. Here, we report a case of vaginal alveolar soft tissue sarcoma in a 42-year-old patient who, on pelvic nuclear magnetic resonance (NMR), had a retention cyst in the vagina. It was decided to perform excision of the vaginal nodule to clarify the diagnosis. The anatomopathological and immunohistochemical reports were conclusive for alveolar soft tissue sarcoma with characteristics in the major axis, with high histological grade and marked nuclear atypia. The patient was then referred to the gynecological oncology service for continued treatment, staging, and follow-up. Due to the rarity of alveolar soft tissue sarcoma and the importance of knowledge on the subject, it is necessary to disclose cases of this condition to increase knowledge, diagnosis, and appropriate treatment.
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