International Journal of Women's Health (Jan 2024)
Alveolar Soft Part Sarcoma in the Female Genital Tract: Case Series with Literature Review and SEER Database Analysis
Abstract
Xingtao Long,1 Qingming Jiang,2 Rengui Li,1 Dong Wang,1 Dongling Zou1 1Department of Gynaecological Oncology, Chongqing University Cancer Hospital, Chongqing, People’s Republic of China; 2Department of Pathology, Chongqing University Cancer Hospital, Chongqing, People’s Republic of ChinaCorrespondence: Dongling Zou, Department of Gynaecological Oncology, Chongqing University Cancer Hospital, 181 Hanyu Road, Shapingba District, Chongqing, 400000, People’s Republic of China, Tel +8613657690699, Fax +8602365075612, Email [email protected]: Alveolar soft part sarcoma (ASPS) is a rare and distinct subtype of soft tissue sarcoma. This study aims to describe the unique presentation of ASPS in the female genital tract.Methods: Prognostic factors for cancer-specific overall survival (CSS) were evaluated using multivariate analyses.Results: In our case series, we identified a novel TFE3-PRCC gene fusion in a 24-year-old unmarried patient with cervical ASPS who underwent fertility-sparing surgery and remained recurrence-free for 41 months. The other two patients underwent radical hysterectomy and bilateral salpingo-oophorectomy. At the time of writing, the two patients had been disease-free for 49 and 71 months, fluorescence in situ hybridization showed break-apart signals for the ASPL-TFE3 gene. Among the 55 cases with available information from the PubMed/Medline database, most presented with localized disease, and at the last follow-up, all patients were alive and 45 patients showed no evidence of disease. The 5-year CSS rate in the female genital tract cohort from SEER database was 86.2%. Multivariate analysis revealed that older age was associated with a 1.042-fold increased risk of cancer-specific mortality (HR=1.042, 95% CI 1.022– 1.063, P < 0.001), involvement of soft tissue including the heart was associated with a 4.786-fold higher risk (HR=4.7868, 95% CI 1.681– 13.623, P= 0.003), and regional infiltration and distant metastasis were associated with approximately 8.6-fold and 18-fold higher risk of cancer-specific mortality compared to local disease, respectively (HR=8.652, 95% CI 2.529– 29.63, P = 0.001; HR=18.366, 95% CI 6.153– 54.817, P< 0.001). Patients who underwent radical excision did not show reduced cancer-specific mortality compared to those who underwent local excision (HR=0.492, 95% CI 0.224– 1.081, P = 0.078).Discussion: Previously unrecognized genetic diversity exists in ASPS. Patients with ASPS in the female genital tract have the lowest likelihood of presenting with a distant disease and are associated with a more favorable survival outcome.Keywords: ASPSCR1, TFE3, alveolar soft part sarcoma, fusion
