title: The challenge of extraabdominal desmoid tumour management in patients with Gardner's syndrome: radiofrequency ablation, a promising option.
abstract: Desmoid tumours are benign, myofibroblastic stromal neoplasms common in Gardner's syndrome, which is a subtype of familial adenomatous polyposis characterized by colonic polyps, osteomas, thyroid cancer, epidermoid cysts, fibromas and sebaceous cysts. The primary treatment is surgery, followed by adjuvant radiotherapy, but the local recurrence rate is high, and wide resection can result in debilitating loss of function. We report the case of a 39-year-old man with Gardner's syndrome who had already undergone a total prophylactic colectomy. He developed desmoid tumours localized in the mesenteric root, abdominal wall and dorsal region, which were treated from 2003 through 2013 with several surgical procedures and percutaneous radiofrequency ablation. In 2008 and 2013, RFA was applied under ultrasonographic guidance to two desmoid tumours localized in the dorsal thoracic wall. The outcomes were low-grade pain and one case of superficial skin necrosis, but so far there has been no recurrence of desmoid tumours in these locations. Surgical resection remains the first-line therapy for patients with desmoid tumours, but wide resection may lead to a poor quality of life. Radiofrequency ablation is less invasive and expensive and is a possible therapeutic option for desmoid tumours in patients with Gardner's syndrome.
authors.affiliation: General Surgery 1, Fondazione IRCCS Policlinico San Matteo, Viale Camillo Golgi 19, 27100 Pavia, Italy. l.cobianchi@smatteo.pv.it.
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Cobianchi
authors.forename: Valentina
authors.initials: V
authors.lastname: Ravetta
authors.forename: Francesca Torello
authors.initials: FT
authors.lastname: Viera
authors.forename: Claudia
authors.initials: C
authors.lastname: Filisetti
authors.forename: Barbara
authors.initials: B
authors.lastname: Siri
authors.forename: Edoardo
authors.initials: E
authors.lastname: Segalini
authors.forename: Marcello
authors.initials: M
authors.lastname: Maestri
authors.forename: Tommaso
authors.initials: T
authors.lastname: Dominioni
authors.forename: Mario
authors.initials: M
authors.lastname: Alessiani
authors.forename: Sandro
authors.initials: S
authors.lastname: Rossi
authors.forename: Paolo
authors.initials: P
authors.lastname: Dionigi
mesh_terms: D000328:Adult; D017115:Catheter Ablation / Q000379:methods*; D019468:Disease Management; D018222:Desmoid Tumors / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D005736:Gardner Syndrome / Q000150:complications / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008297:Male; D011379:Prognosis