title: The association between home parenteral nutrition and patients with FAP-associated intra-abdominal desmoids: a retrospective study.
abstract: BACKGROUND
  Intra-abdominal desmoid tumors (IADTs) are a common complication of familial adenomatous polyposis (FAP). Treatment is not standardized for advanced disease. Medical and surgical treatments may be ineffective in preventing complications, which can cause intestinal failure. Home parenteral nutrition (HPN) can be a life-saving treatment in these patients. The aim of this study was to investigate the association with HPN in FAP-IADTs.
  
  
  METHODS
  A retrospective review of FAP patients with IADTs at the Cleveland Clinic (CC) between 1980 and 2009 was performed. Patients and tumor characteristics were retrieved from the CC Jagelman Registry for Inherited Neoplasms and CC HPN database. Inclusion criteria were FAP-IADTs and 6-month follow up at CC. Exclusion criteria were <6-month follow-up, lack of 3-dimensional lesion or sheet desmoid, and/or incomplete medical records. Kaplan-Meier curves were analyzed for HPN and non-HPN groups.
  
  
  RESULTS
  One hundred fifty-four patients were included and divided into 2 groups: HPN (n = 41, 26.6%) and non-HPN (n = 113, 73.4%). The HPN group was more likely to have advanced-stage disease and significantly higher incidence of chronic abdominal pain, narcotic dependency, bowel obstruction, ureteral obstruction, deep vein thrombosis, pulmonary embolism, fistulae, and sepsis (P < .05). The need for HPN represented a strong predictor of mortality (5-year survival HPN = 72% vs non-HPN = 95%), but duration of HPN did not affect mortality.
  
  
  CONCLUSIONS
  HPN, although a life-saving treatment, is an independent poor prognostic factor associated with high morbidity and mortality.
authors: Department of Gastroenterology and Hepatology, Digestive Disease Institute, The Cleveland Clinic, Cleveland, Ohio 44195, USA. Shatnaa@ccf.org
authors: Abdullah
authors: A
authors: Shatnawei
authors: Monica
authors: M
authors: Habib
authors: Cindy
authors: C
authors: Hamilton
authors: Ezra
authors: E
authors: Steiger
authors: Donald F
authors: DF
authors: Kirby
authors: Rex
authors: R
authors: Speerhas
authors: Cristiano
authors: C
authors: Quintini
mesh_terms: D011125:Adenomatous Polyposis Coli / Q000150:complications / Q000188:drug therapy* / Q000453:epidemiology*; D000293:Adolescent; D000328:Adult; D005260:Female; D018222:Desmoid Tumors / Q000150:complications / Q000188:drug therapy* / Q000453:epidemiology*; D006801:Humans; D015994:Incidence; D007415:Intestinal Obstruction; D008297:Male; D009017:Morbidity; D016331:Parenteral Nutrition, Home / Q000009:adverse effects*; D016016:Proportional Hazards Models; D012042:Registries; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
keywords: adult; desmoid tumors; home nutrition support; parenteral nutrition