Tongue cancer following hematopoietic cell transplantation for Fanconi anemia

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

Adenomatoid odontogenic tumor. A case report

The adenomatoid odontogenic tumor is a benign bony lesion of the maxilla and mandible, it represents 3% of all odontogenic tumors, and is more frequent in females in their second decade of life. It affects mainly the maxilla associated with embedded teeth, often canines, but it also occurs in the mandible, especially in the anterior region. It originates from epithelial cells of the dental lamina complex system, but the histologic feature with gland-like structures explains its name and classification. Clinically silent, it is often discovered during radiological examinations as a well defined unilocular radiolucent image. The definitive diagnosis is obtained by histological examinations, which allow the differential diagnosis from other bony lesions. Treatment must include close examinations, such as axial and coronal CT scanning and eventually 3D reconstruction. Histological examination with a preoperative biopsy suggests a conservative surgical treatment consisting of a simple enucleation for the low incidence of recurrence. A clinical case is presented with peculiar features different from that reported in letterature