Algorithm for the surgical management of mesenchymal tumors of the perineum in adults

BACKGROUND: Perineal soft tissue tumors are rare, so that little is known about their management and the outcome of treatment. OBJECTIVE: The aim of this study is to describe the presentation, management, and outcome of the surgical treatment of soft tissue tumors and to provide a final decision algorithm. DESIGN: This is a retrospective study. SETTINGS: The study was conducted in a single tertiary care hospital with a dedicated unit on sarcoma. PATIENTS: Fifty-one consecutive patients from 1998 to 2013 were included. MAIN OUTCOME MEASURES: The primary outcomes measured are patient demographics, treatment decisions, and outcome of surgical treatment. RESULTS: Forty-nine patients presented with a primary soft tissue tumor, and 2 underwent simple excisions for isolated metastases. The median tumor size was 75 mm (50-110). Symptoms were nonspecific, and MRI had insufficient specificity for malignancy so that a preoperative biopsy was systematically performed according to European Society for Medical Oncology and National Comprehensive Cancer Network soft tissue tumor guidelines. Six benign soft tissue tumors (3 lipomas, 3 leiomyomas), 16 intermediate soft tissue tumors (12 aggressive angiomyxoma, 4 desmoid tumors), and 27 sarcomas were identified. Treatments and surgery were tailored from the beginning according to histology. All but 1 benign soft tissue tumor were treated by "shelling out." Aggressive angiomyxoma were treated with en bloc resection sparing uninvolved organs. Nonsurgical treatments were our first choice for desmoid tumors. Wide en bloc surgery was planned for all sarcomas (n = 27) after the induction treatment for 16 patients (chemotherapy, n = 12; radiotherapy, n = 4). In the sarcoma group, the 5-year estimated metastasisfree, local recurrence-free, and overall survival rates were 68.1% (95% CI, 50.7-91.5), 84.7% (95% CI, 66.7-100), and 85.7% (95% CI, 71.8-100). In the benign and intermediate tumor groups, there were no deaths, local recurrences, or progression. LIMITATIONS: This study was limited by the small number of patients, given the rarity of this disease in the perineum. CONCLUSION: We provide useful indications for the best strategy necessary to treat these rare tumors located in a complex site.