Clinical Features and Treatment Outcomes of Vulvar Sarcoma: A Single-Institution Review of 21 Cases

Abstract

BackgroundValvar sarcoma is an extremely rare disease in which the data for treatment and prognosis are scarce. The aim of this study was to analysis the clinical features and treatment outcomes of initial and recurrent valvar sarcoma.MethodThe medical records of patients with initial or recurrent vulvar sarcoma treated at Sun Yat-sen University Cancer Center between January 2005 and April 2019 were retrospectively reviewed.Results A total of 21 patients with vulvar sarcoma were identified. The median age of them was 43 years (range: 16 to 71). The histological subtypes were epithelioid sarcoma, leiomyosarcoma, dermatofibrosarcoma protuberans, rhabdomyosarcoma, and angiosarcoma in 9, 4, 4, 3 and 1 patients, respectively. None of the patients received wide resection when they were initially treated at communities. Thirteen patients were referred immediately after initial managements (Group A), whereas 8 patients were referred with recurrent disease (Group B). Wide resections were performed with pathologically negative margins in 20 patients, of whom 9 also received lymphadenectomy and 3 showed metastases. One patient in Group B only received chemoradiotherapy for unresectable pelvic mass. Adjuvant chemoradiotherapy were performed for 12 patients evaluated to have high risk of recurrence. The median follow-up time was 39 months (range: 11 to 174) for Group A and 107 (range: 2 to 139) for Group B. During the follow-up, relapse occurred in 3 patients in Group A and 5 in Group B. The 3- and 5-year survival rates were 92.3% and 76.9% for Group A, 50.0% and 37.5% for Group B, respectively.ConclusionsVS is usually resected as benign disease at initial management. Immediate extended resection with negative margins provided favorable outcomes for most of the patients. The prognosis for recurrent cases were poor, however there remained survival opportunities if aggressive treatments were carried out.