Work

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

Year: 2025

Type: article

Abstract: 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.

Source: Cancer Diagnosis & Prognosis

DOI: 10.21873/cdp.10471

Authors: REINHARD E. FRIEDRICH, FELIX K. KOHLRUSCH, CHRISTIAN HAGEL

Institutions: Universität Hamburg, University Medical Center Hamburg-Eppendorf

Language: English

Topic: Neurofibromatosis and Schwannoma Cases

Subfield: Neurology

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: bronze

OpenAlex ID: W4413959936