Surgical Measures Improving Functional Limitations of the Masticatory System, Aesthetic Deficits, and Skeletal Malformations in Neurofibromatosis Type 1-associated Hemifacial Diffuse Plexiform Neurofibroma Complicated by Rapidly Growing Midfacial Peripheral Nerve Sheath Tumor.

BACKGROUND/AIM: Neurofibromatosis type 1 (NF1) is a genetic disorder characterized by the development of multiple tumors, including plexiform neurofibromas (PNFs), which often affect the craniofacial region and cause significant functional and aesthetic impairments. This report presents long-term surgical management of a patient with hemifacial diffuse PNF, complicated by the emergence of a rapidly enlarging midfacial tumor.

CASE REPORT: The patient was treated for hemifacial invasive diffuse plexiform neurofibroma. During follow-up, the patient developed a rapidly growing tumor suggesting malignant transformation. Histological analysis revealed plexiform and diffuse neurofibroma with necrosis. Defect coverage after resection was complex and required tissue replacement with a microvascular graft. In the further course, new neurofibromas developed in the oral transplant.

CONCLUSION: Surgical measures enable functional and aesthetic improvements despite extensive facial destruction caused by the tumor. Rapid tumor growth is a finding that requires immediate diagnosis in patients with NF1. Even in tumor-free transplants at the time of tissue transfer, (nodular) neurofibromas can develop at the recipient site.