ArticleCancer diagnosis & prognosis
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.
Article in Cancer diagnosis & prognosis. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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Who cites it
2 citing papers in PubMed.
- Long-term Stable Unilateral Mandibular Deformity Associated With Ipsilateral Skull-base Soft-tissue Lesion and Degenerated Pterygoid Muscles in Neurofibromatosis Type 1.Cancer diagnosis & prognosisArticle
- Combined Soft and Hard Tissue Facial Lesions In Early Childhood Pioneering the Diagnosis of the Tumor Predisposition Syndrome Neurofibromatosis Type 1.Cancer diagnosis & prognosisArticle
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3 authors.
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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.
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