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. Cancer 2008. © 2008 American Cancer Society.

Twenty years of the Italian Fanconi Anemia Registry: where we stand and what remains to be learned

“…21,27 Malignancies play an important role in the natural history of FA, the risk increasing with age for a wide array of cancer types; [10][11][12]18 moreover, some patients can develop multiple cancers, possibly also due to the increased risk associated with anti-cancer treatments (i.e., chemotherapy and radiotherapy). This report confirms the cancer propensity of FA patients and further stresses the need for frequent and careful tumor evaluations, aiming at early therapeutic interventions, 24,28 the only effective strategy for improving long-term survival in FA patients.…”

“…HSCT may cure the hematological disease associated with FA, but it does not reverse the phenotype that results from the involvement of extra-hematological tissues and organs. [29][30][31] This is especially true for the intrinsic risk of cancer due to the genetic instability typical of FA, which might actually be increased by the pre-transplant conditioning regimen and possible detrimental A.M. Risitano et al 324 haematologica | 2016; 101(3) 28,32 Notably, in our study the risk of solid tumors remains high even after HSCT, but apparently it is not increased over that of non-transplanted patients. However, these data should be confirmed with a longer follow-up, possibly within International studies designed to specifically investigate this endpoint.…”

Hematopoietic stem cell transplantation in patients with Fanconi anemia: the experience of the Russian Children's Clinical Hospital and the Dmitry Rogachev Federal Research Center of Pediatric Hematology, Oncology and Immunology

“…Доза корригиру-ется в зависимости от ответа. Однако у данного вида терапии имеются выраженные побочные действия, такие как маскулинизация, низкорослость, аденомы печени и гепатоцеллюлярные карциномы [9]. Кроме того, при проведении ТГСК после терапии андроге-нами может отмечаться выраженная печеночная ток-сичность [10].…”

“…Во всех случаях ответ был частичным и непродолжительным [9]. Кроме того, су-ществует по крайней мере теоретический риск стиму-ляции развития МДС и ОМЛ вследствие применения колониестимулирующих факторов, поэтому препараты данной группы не должны применяться длительно.…”

Induction of TGF-β by Irradiation or Chemotherapy in Fanconi Anemia (FA) Mouse Bone Marrow Ιs Modulated by Small Molecule Radiation Mitigators JP4-039 and MMS350

“…FA patients are also susceptible to late post-transplant induction of leukemia and solid tumors (7)(8)(9)(10)(11)(12)(13)(14)(15). DNA cross-linking agents such as mitomycin-C (14), other chemotherapeutic agents and irradiation induce DNA double strand breaks and must be delivered very cautiously to FA patients.…”