Exploring Head and Neck Fibromatosis: A Case Series and Literature Review

Indian J Surg Oncol. 2024 Dec;15(4):849-856. doi: 10.1007/s13193-024-02010-z. Epub 2024 Jul 1.

Abstract

This study aimed to retrospectively review the clinical data, management protocols, clinical outcomes, and literature review of patients diagnosed with head and neck fibromatoses. This case series aims to present and discuss seven cases of rare head and neck fibromatosis. The study design involves retrospective analysis of medical records, radiological imaging, and histopathological reports of the included cases. Ethical approval was obtained from the relevant Institutional Review Board. The results of the study reveal that individuals diagnosed with fibromatosis had a wide age range, starting from 6 to 58 years, with the median age being 44.3 years. Out of the 8 patients, 5 (62.5%) were females and 3 (37.5%) were males. The maxilla was found to be the most common subsite of origin in the head and neck area, followed by the masseter and temporal fossa. At the time of diagnosis, 2 (25%) patients were found to have unresectable disease. Multidisciplinary management therapies, including surgery, chemotherapy, hormonal, targeted therapy, and radiotherapy, were used. Out of the 6 patients who underwent surgery, 5 (83.3%) experienced tumor recurrence. In conclusion, the retrospective analysis underscores the complexity of managing fibromatoses of the head and neck region; complete tumor resection is not achievable always due to the inherent challenges, complex anatomy, and proximity of vital structures in the head and neck. Adjuvant strategies including radiotherapy, chemotherapy, and hormonal therapy have demonstrated efficacy in disease management. The rarity of these tumors necessitates the development of standardized treatment protocols to guide clinical practice effectively. Moving forward, collaborative efforts are crucial to enhance our understanding of fibromatoses and refine therapeutic strategies for improved patient outcomes.

Keywords: Desmoid tumor; Fibromatosis; Head and neck sarcoma; Multidisciplinary management.