Saad Hajeri1, Abdulsalam Al Jabab2, Manal Al Sheddi3, Hanadi Fatani2. 1. Oral and Maxillofacial Surgery, King Faisal Medical City, Abha, Saudi Arabia. hajeri@me.com. 2. Department of Head and Neck Oncology Surgery, King Fahad Medical City, Riyadh, Saudi Arabia. 3. College of Dentistry, Department of Oral Medicine and Diagnostic Sciences, King Saud University, Riyadh, Saudi Arabia. dr_aljabab@hotmail.com.
Abstract
UNLABELLED: Myofibroblastoma is a rare benign tumor of the head and neck region, which is characterized by a large, rapidly growing, and destructive mass. A 3-year-old boy presented with an 8-week history of a rapidly growing swelling of the right mandible. Examination revealed a firm 13-cm mass occupying the entire right body and ramus of the mandible. The clinical and radiological features were suggestive of a sarcoma. An initial biopsy taken in the referring hospital was inconclusive, and the second biopsy showed a myofibroblastic neoplasm consistent with a desmoplastic fibroma. Progressive tumor growth necessitated a tracheostomy. Right hemimandibulectomy was performed, and the defect was reconstructed with free microvascular fibula flap. Histopathology and immunocytochemistry revealed a myofibroblastoma. This entity differs from other myofibroblasts and fibroblast tumors such as inflammatory myofibroblastic tumor (IMT), myofibroma, and desmoplastic fibroma. The child has been followed up for 2 years. CONCLUSION: Differentiation between myofibroblasts and fibroblastic tumors as well as some malignancies can be challenging. Myofibroblastoma can behave as a malignant neoplasm, and the clinical distinction of this entity lies primarily in its recognition as a benign neoplasm.
UNLABELLED: Myofibroblastoma is a rare benign tumor of the head and neck region, which is characterized by a large, rapidly growing, and destructive mass. A 3-year-old boy presented with an 8-week history of a rapidly growing swelling of the right mandible. Examination revealed a firm 13-cm mass occupying the entire right body and ramus of the mandible. The clinical and radiological features were suggestive of a sarcoma. An initial biopsy taken in the referring hospital was inconclusive, and the second biopsy showed a myofibroblastic neoplasm consistent with a desmoplastic fibroma. Progressive tumor growth necessitated a tracheostomy. Right hemimandibulectomy was performed, and the defect was reconstructed with free microvascular fibula flap. Histopathology and immunocytochemistry revealed a myofibroblastoma. This entity differs from other myofibroblasts and fibroblast tumors such as inflammatory myofibroblastic tumor (IMT), myofibroma, and desmoplastic fibroma. The child has been followed up for 2 years. CONCLUSION: Differentiation between myofibroblasts and fibroblastic tumors as well as some malignancies can be challenging. Myofibroblastoma can behave as a malignant neoplasm, and the clinical distinction of this entity lies primarily in its recognition as a benign neoplasm.