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.

Keywords: Bone marrow transplantation; Fanconi; Oral cancer; Oxidative stress; Squamous cell carcinoma; Tongue.

Fig. 1

Cumulative incidence function of tongue cancers in Fanconi anemia patients.*HCT, hemopoietic cell transplantation

Fig. 2

UPN 230 presented to the outpatient clinic with a painful swelling of the right side of the tongue (A). A concomitant ulceration of the adherent gingiva in the fourth quadrant was observed as well during the clinical examination (B). Bioptic examination of the lesions documented a squamous cell carcinoma in both cases

Fig. 3

UPN 333 showed a painful swelling of the left lingual margin (A). The COVID-19 pandemic determined a 5-month diagnostic delay, with a consistent growth in size of the tumor (B)