title: Surgery for Li Fraumeni syndrome: pushing the limits of surgical oncology.
abstract: OBJECTIVE
  Li Fraumeni syndrome is an autosomal dominant cancer syndrome due to a germline mutation in the p53 tumor suppressor gene. It results in multiple primary neoplasms in children and adults. A common question when faced with a Li Fraumeni patient who develops multiple primary cancers and/or recurrences is what is the proper treatment? Data suggests that ionizing radiation exposure increases the incidence of second malignancies in the Li Fraumeni population. Therefore, how much surgery can a cancer patient tolerate and still derive benefit from it?
  
  
  METHODS
  We describe a representative case of a 54-year-old female with Li Fraumeni syndrome with an enlarging adrenocortical hepatic metastasis, a new primary ampullary cancer, and an extensive surgical history.
  
  
  RESULTS
  We performed a simultaneous pancreaticoduodenectomy and repeat partial hepatectomy.
  
  
  CONCLUSIONS
  We propose that surgery is underutilized in metastatic solid organ familial cancers in general, and argue that an aggressive surgical approach should be considered in a multidisciplinary manner for patients with Li Fraumeni syndrome and recurrent tumors. However, because of the rarity of this familial cancer there is a paucity of evidence to support this approach, therefore a review of the literature is presented.
authors.affiliation: †Department of Surgery, Georgetown University Hospital, Washington, DC ‡Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA *Surgery Branch, National Cancer Institute, National Institutes of Health §Department of Surgery, Division of Surgical Oncology, Walter Reed National Military, Medical Center ∥Department of Surgery, Uniformed Services University of the Health Sciences, Bethesda, MD ¶Bon Secours Cancer Institute, Richmond, VA.
authors.forename: Russell C
authors.initials: RC
authors.lastname: Langan
authors.forename: Kiran H
authors.initials: KH
authors.lastname: Lagisetty
authors.forename: Scott
authors.initials: S
authors.lastname: Atay
authors.forename: Prakash
authors.initials: P
authors.lastname: Pandalai
authors.forename: Alexander
authors.initials: A
authors.lastname: Stojadinovic
authors.forename: Udo
authors.initials: U
authors.lastname: Rudloff
authors.forename: Itzhak
authors.initials: I
authors.lastname: Avital
mesh_terms: D000230:Adenocarcinoma / Q000601:surgery*; D000306:Adrenal Cortex Neoplasms / Q000473:pathology*; D018268:Adrenocortical Carcinoma / Q000556:secondary / Q000601:surgery*; D014670:Ampulla of Vater; D001943:Breast Neoplasms / Q000601:surgery; D002280:Basal Cell Carcinoma / Q000601:surgery; D018270:Carcinoma, Ductal, Breast / Q000601:surgery; D003138:Common Bile Duct Neoplasms / Q000601:surgery*; D005260:Female; D006498:Hepatectomy; D051677:Histiocytoma, Malignant Fibrous / Q000601:surgery; D006801:Humans; D016864:Li-Fraumeni Syndrome / Q000601:surgery*; D008113:Liver Neoplasms / Q000556:secondary / Q000601:surgery*; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000601:surgery; D016577:Pancreaticoduodenectomy; D012878:Skin Neoplasms / Q000601:surgery
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