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.
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: Mark E Bernard; Hyun Kim; Hebist Berhane; Michael W Epperly; Darcy Franicola; Xichen Zhang; Frank Houghton; Donna Shields; Hong Wang; Christopher J Bakkenist; Marie-Celine Frantz; Erin M Forbeck; Julie P Goff; Peter Wipf; Joel S Greenberger Journal: Radiat Res Date: 2011-09-22 Impact factor: 2.841
Authors: David I Kutler; Krupa R Patel; Arleen D Auerbach; Jennifer Kennedy; Francis P Lach; Erica Sanborn; Marc A Cohen; William I Kuhel; Agata Smogorzewska Journal: Laryngoscope Date: 2015-10-20 Impact factor: 3.325
Authors: P N Floriano; T Abram; L Taylor; C Le; H Talavera; M Nguyen; R Raja; A Gillenwater; J McDevitt; N Vigneswaran Journal: Oral Dis Date: 2015-04-06 Impact factor: 3.511
Authors: Thomas H Beckham; Jonathan Leeman; Chiaojung Jillian Tsai; Nadeem Riaz; Eric Sherman; Bhuvanesh Singh; Nancy Lee; Sean McBride; Daniel S Higginson Journal: Head Neck Date: 2019-01-11 Impact factor: 3.147
Authors: Igor Sirák; Zuzana Šinkorová; Mária Šenkeříková; Jiří Špaček; Jan Laco; Hana Vošmiková; Stanislav John; Jiří Petera Journal: Rep Pract Oncol Radiother Date: 2014-12-05
Authors: M S Thakar; P Kurre; R Storb; M Kletzel; H Frangoul; M A Pulsipher; W Leisenring; M E D Flowers; B M Sandmaier; A Woolfrey; H-P Kiem Journal: Bone Marrow Transplant Date: 2010-06-28 Impact factor: 5.483