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. Cancer 2008. © 2008 American Cancer Society.

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RÉSUMÉLes schwannomes ou neurilemmomes sont des tumeurs bénignes encapsulées, le plus souvent solitaires, issues des cellules de Schwann des gaines nerveuses. Dans la région cervico-faciale, 1 % des schwannomes sont retrouvés dans la cavité buccale et surtout sur la langue. Les auteurs rapportent le cas d'un schwannome localisé sur le bord latéral gauche de la langue. Une exérèse chirurgicale complète a été réalisée, et l'examen histopathologique de la pièce opératoire était en faveur d'un schwannome. Les suites ont été simples, et après 4 ans de recul, le patient ne présente pas de récidive. Le diagnostic positif repose sur l'examen histopathologique. Le

knockdown may have altered the immune surveillance milieu in the skin with its complex cytokine patterns and reduced numbers and function of Tregs, thereby leading to the increase of NER mechanisms and subsequent reduction of DNA damage as measured by CPDs (Figure 1c, d, and f). In addition, the CD28 ligands BB-1 and B7 that are expressed in keratinocytes provide an important co-stimulatory signal for CD3mediated proliferation of T lymphocytes, including alloreacting CD4 þ T cells (Koulova et al., 1991; Simon et al., 1994). This raises the possibility that T-cell activation by keratinocytes after UVB exposure may have been inhibited by in vivo CD28 siRNA knockdown. In this regard, it is interesting to note that functional blockade of CD80/CD86 significantly decreased UV-induced tumor development (Loser et al., 2005). In any case, further studies are warranted to determine the precise mechanisms by which CD28 knockdown leads to increased NER and decreased DNA damage and skin alterations.