title: Vulvar fibromatosis: a clinical enigma.
abstract: OBJECTIVE
  To present a case of recurrent vulvar fibromatosis in an adolescent, discuss the specific difficulties of treating adolescents, and review the literature on available treatment.
  
  
  METHODS
  We present a case of recurrent vulvar fibromatosis in a 14-year-old girl, requiring several treatment modalities, including multiple surgeries, radiation therapy, and multiagent chemotherapy. We then discuss management strategies for these tumor types, and specifically examine how tumor location may impact their treatment.
  
  
  RESULTS
  Vulvar desmoids are extremely uncommon and they can be disfiguring and cause significant discomfort for women. Initial management of these tumors is surgical excision, yet failed surgery is often followed by other treatment modalities, including radiation, tyrosine kinase inhibitors, nonsteroidal anti-inflammatory drugs, hormonal therapy, and chemotherapy. This case clearly highlights the difficulties in managing these rare tumors, particularly in the adolescent population.
  
  
  CONCLUSIONS
  Desmoid tumors are nonmalignant, locally aggressive neoplasms most common in the 15 to 60 years age group. They are associated with high estrogen states, prior surgical trauma, and Gardner syndrome. Most commonly, desmoid tumors present in the abdominal wall, shoulder, neck, and chest, but can occur anywhere in the body. Given their rarity and lack of definitive therapy, vulvar desmoid tumors can be exceedingly difficult to treat, and are best managed with an interdisciplinary approach.
authors.affiliation: *Obstetrics & Gynecology Department at Montefiore Departments of †Gynecologic and Oncology ‡Pathology §Pediatric Hematology-Oncology, Albert Einstein College of Medicine and Montefiore Medical Center, New York, NY.
authors.forename: Erin M
authors.initials: EM
authors.lastname: Conroy
authors.forename: Marina
authors.initials: M
authors.lastname: Frimer
authors.forename: Rouzan G
authors.initials: RG
authors.lastname: Karabakhtsian
authors.forename: Richard
authors.initials: R
authors.lastname: Gorlick
authors.forename: Gary L
authors.initials: GL
authors.lastname: Goldberg
mesh_terms: D000293:Adolescent; D005260:Female; D005350:Fibroma / Q000628:therapy*; D006801:Humans; D014846:Vulvar Neoplasms / Q000628:therapy*