Tongue cancer following hematopoietic cell transplantation for Fanconi anemia

Abstract

Objectives The aim of this retrospective study was to determine the incidence and the clinical outcome of tongue cancer (TC) in patients affected by Fanconi anemia (FA) who received an allogeneic hematopoietic cell transplantation (HCT). Materials and methods The patient database from the Bone Marrow Transplant Center of Pescara was reviewed to enroll FA patients. Patients’, donors’, HCT’s, and screening’s data were collected as well to look for the incidence and the treatment of TC. Results Twelve patients affected by FA were identified. Three patients died for transplant-related causes. Five of nine surviving patients were diagnosed with TC at a median of 21.7 years since transplantation and at a median age of 32.10 years. Interestingly, no patient manifested graft-versus-host-disease (GvHD). The 28-year cumulative incidence function of TC was 46.9% (95% CI, 36.9–56.9%). Two patients were treated with chemotherapy alone, two patients were treated with surgery alone, and one with surgery followed by chemotherapy. Overall, 4 patients with TC showed a clinical course characterized by a marked aggressiveness of the tumor disease which led to death due to cancer progression between 2 and 13 months. One patient is surviving 8 months after diagnosis of TC. Conclusions Our study confirms the high incidence of tumors and in particular tongue tumors in allotransplanted FA patients. A careful screening has to be life-long maintained. Clinical relevance Considering the rarity of FA and the frailty of FA patients, this study may add important information for the cancer management of these patients.

Primary Mandibular Condyle Xanthoma: Case Report and Literature Review

“…Therefore, the prognosis seems to be good even with a mild curettage and the saving of noble structures, but careful clinical and imaging follow-up recommendations seems to be indicated for safety [2,14]. However, no guidelines are available as in other ultra-rare diseases [27,28].…”

The onset and progression of oral potentially malignant disorders in Fanconi anemia patients: Highlighting early detection of oral cancer

“…Fanconi anemia (FA) is a rare inherited disease of genomic instability that may lead to bone marrow failure, hematologic and solid malignancies, mainly head and neck cancers. 1 Hematopoietic stem cell transplantation (HSCT) is the only curative therapeutic option for this feature of the disease. 2 Due to better survival rates from successful HSCT, many patients with FA now are surviving into adulthood even though with the risk of chronic graft-versus-host disease (GVHD).…”