title: Head and neck squamous cell carcinoma in 13 patients with Fanconi anemia after hematopoietic stem cell transplantation.
abstract: BACKGROUND
  Fanconi anemia (FA) is a chromosomal instability disorder with a very high risk of developing head and neck squamous cell carcinoma (HNSCC), most notably after hematopoietic stem cell transplantation (HSCT).
  
  
  METHODS
  In the current study, the authors reported 13 cases of HNSCC in FA patients who underwent HSCT at the Saint Louis Hospital between 1976 and 2007.
  
  
  RESULTS
  The median age of the patients at time of HSCT was 9.7 years. All patients received irradiation-based conditioning before HSCT and all developed extensive chronic graft versus host disease (GVHD). HNSCC was diagnosed at a median interval of 10 years after HSCT, mainly in numerous sites within the oral cavity (11 patients). Lymph node involvement was diagnosed in 4 patients. The TNM classification was: T1 in 6 patients, T2 in 2 patients, T3 in 2 patients, and T4 in 3 patients. Treatment was comprised of surgery in 10 patients, with clear surgical margins reported in 7 (including cervical lymph node dissection in 6 patients). Surgery was performed in addition to other treatments in only 2 patients (radiotherapy or cryotherapy). For the remaining 3 patients, treatment consisted in radiotherapy (2 patients) or chemotherapy (1 patient). Disease progression while receiving therapy was observed in 5 patients and 5 other patients developed disease recurrence between 3.5 and 23.7 months after treatment. Death occurred in 11 patients. At the time of last follow-up, only 2 patients were alive without any disease between 9 and 23 months after diagnosis.
  
  
  CONCLUSIONS
  HNSCC developing in FA patients after HSCT is associated with a very poor prognosis. A systematic surveillance of the oral cavity is essential to permit early surgery, which to the authors' knowledge remains the only curative treatment for a minority of patients. It is very important to attempt to prevent this cancer by reducing chronic GVHD and using conditioning without irradiation.
authors.affiliation: Assistance Publique Hospitaux de Paris, Bone Marrow Transplant Unit, Saint Louis Hospital, Paris, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Masserot
authors.forename: Régis
authors.initials: R
authors.lastname: Peffault de Latour
authors.forename: Vanderson
authors.initials: V
authors.lastname: Rocha
authors.forename: Thierry
authors.initials: T
authors.lastname: Leblanc
authors.forename: Arnaud
authors.initials: A
authors.lastname: Rigolet
authors.forename: Francis
authors.initials: F
authors.lastname: Pascal
authors.forename: Anne
authors.initials: A
authors.lastname: Janin
authors.forename: Jean
authors.initials: J
authors.lastname: Soulier
authors.forename: Eliane
authors.initials: E
authors.lastname: Gluckman
authors.forename: Gérard
authors.initials: G
authors.lastname: Socié
mesh_terms: D000328:Adult; D002294:Carcinoma, Squamous Cell / Q000150:complications / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D005199:Fanconi Anemia / Q000150:complications / Q000628:therapy*; D005260:Female; D006086:Graft vs Host Disease / Q000209:etiology; D006258:Head and Neck Neoplasms / Q000150:complications / Q000209:etiology*; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D008297:Male; D011379:Prognosis; D019172:Transplantation Conditioning