Bone Marrow Failure Disease and Head-and-Neck Squamous Cell Carcinoma in King Faisal Specialist Hospital and Research Center in Riyadh

Abstract

Listen

Translate article

Objective: This study aims to look at bone marrow failure disease patients who presented to our center, as to analyze the presentation and possible ways to improve the survival outcome in these cases. Methods: This was a retrospective chart review study. Results: In our series, most of the head-and-neck squamous cell carcinomas (HNSCCs) were found in Fanconi anemia (FA) patients, and most are located within the oral cavity, most frequently at the tongue margins and the gingival areas. These tumors arise in both male and female bone marrow failure disease patients without a prior history of excessive tobacco and alcohol use. Even if a patient presents at an early stage, there are frequent relapses. The highly aggressive nature of the tumors is reflected by poor survival, amounting to <2 years. FA is a rare but well-studied inherited disorder that is clinically characterized by progressive bone marrow failure, congenital malformations, and increased incidence of malignancies, especially acute myeloid leukemia and squamous cell carcinomas (SSCs) of the head and neck. Greatly improved protocols for stem cell transplantation increasingly save the lives of these young patients. However, in both transplanted and not transplanted patients, the emergence of aggressive SSC represents a major medical challenge. Noninvasive screening options, including frequent self-examination and inspection by a medical team, should lead to early detection and treatment. Standard platinum-based chemotherapy in combination with radiotherapy cannot be used in FA patients due to therapy-associated toxicities and mortalities even with reduced dosing. Therefore, surgery is the most important treatment option for HNSCC in bone marrow failure disease patients and requires an early and efficient detection of malignant lesions. Conclusion: So far, no uniform treatment protocol for the management of HNSCCs in FA patients exists. Therefore, we propose that the information on affected FA patients should be collected worldwide, practical therapeutic guidelines developed and national treatment centers established.