Alveolar Soft Part Sarcoma Presenting as a Uterine Polyp

Abstract Background Alveolar soft part sarcoma (ASPS) is a rare malignant soft tissue tumor, mainly localized in the extremities, occurring principally in adolescents and young adults. ASPS is uncommon in the female genital tract. Case report We here report a case of ASPS in a 20-year-old nullipara, presenting with vaginal bleeding and profound anemia requiring blood transfusions. Ultrasonographic examination revealed a polyp in the lower uterine segment. Surgical resection of the polyp was performed, and pathological evaluation showed the typical histological, immunohistochemical, and molecular features consistent with ASPS. At the time of this report, the patient was scheduled for total hysterectomy with promising results. Conclusion Given the infrequency of ASPS, pathological evaluation is or utmost importance in order to establish an accurate diagnosis. When presenting as a primary tumor, complete excision is fundamental in the treatment.