title: Algorithm for the surgical management of mesenchymal tumors of the perineum in adults.
abstract: 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.
  
  
  METHODS
  This is a retrospective study.
  
  
  METHODS
  The study was conducted in a single tertiary care hospital with a dedicated unit on sarcoma.
  
  
  METHODS
  Fifty-one consecutive patients from 1998 to 2013 were included.
  
  
  METHODS
  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 metastasis-free, 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.
  
  
  CONCLUSIONS
  This study was limited by the small number of patients, given the rarity of this disease in the perineum.
  
  
  CONCLUSIONS
  We provide useful indications for the best strategy necessary to treat these rare tumors located in a complex site.
authors: 1 Department of Surgical Oncology, Institut Gustave Roussy, Villejuif, France 2 Department of Radiology, Institut Gustave Roussy, Villejuif, France 3 Department of Pathology, Institut Gustave Roussy, Villejuif, France 4 Department of Medical Oncology, Institut Gustave Roussy, Villejuif, France 5 Department of Radiotherapy, Institut Gustave Roussy, Villejuif, France.
authors: Isabelle
authors: I
authors: Sourrouille
authors: Laurence
authors: L
authors: Vilcot
authors: Charles
authors: C
authors: Honoré
authors: Sara
authors: S
authors: Coppola
authors: Philippe
authors: P
authors: Terrier
authors: Axel
authors: A
authors: le Cesne
authors: Cécile
authors: C
authors: Le Péchoux
authors: Sylvie
authors: S
authors: Bonvalot
mesh_terms: D000328:Adult; D000465:Algorithms; D001706:Biopsy; D019468:Disease Management; D004210:Dissection / Q000379:methods / Q000401:mortality; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009367:Neoplasm Staging; D017063:Outcome Assessment, Health Care; D010502:Perineum / Q000473:pathology*; D011300:Preoperative Care / Q000379:methods; D011379:Prognosis; D012189:Retrospective Studies; D012509:Sarcoma / Q000453:epidemiology / Q000473:pathology / Q000601:surgery; D012983:Soft Tissue Neoplasms / Q000145:classification / Q000453:epidemiology / Q000473:pathology / Q000601:surgery; D015996:Survival Rate