A case report of giant malignant schwannoma of the sciatic nerve associated with neurofibromatosis-1: A CARE-compliant article.

Autor: Gokkus K; Baskent University, Alanya Research and Practice Hospital, Department of Orthopedics and Traumatology, Antalya, Turkey., Saylik M; Department of Orthopaedic Surgery, Istinye University Medical Faculty, Topkapi Kampüsü, Maltepe Mah, Edirne Çirpici Yolu, İstanbul, Turkey., Birtay T; Baskent University, Alanya Research and Practice Hospital, Department of Anesthesiology, Alanya/Antalya, Turkey., Sahin MS; Baskent University, Alanya Research and Practice Hospital, Department of Orthopedics and Traumatology, Antalya, Turkey.
Jazyk: angličtina
Zdroj: Medicine [Medicine (Baltimore)] 2023 Nov 24; Vol. 102 (47), pp. e36358.
DOI: 10.1097/MD.0000000000036358
Abstrakt: Rationale: Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous syndrome that causes multiple central and peripheral nerve sheath tumors. People with NF1 have a 10% chance of developing malignant peripheral nerve sheath tumors (MPNSTs). Here we report a unique instance of a malignant schwannoma that has remained free of metastasis since its initial removal a decade ago. The malign schwannoma has been infrequently documented in the literature, and remarkably, no instances of such an extensive postoperative time without metastases have ever been described.
Patient Concerns: A 46-year-old male patient with NF had multiple neurofibromas in different parts of his body, underwent surgery about 10 years ago (2013), and was diagnosed histopathologically as MPNST.
Diagnoses: He was admitted to our institution with a recurrent mass in the posterior third of the proximal thigh and severe pain radiating to the left lower extremity, which presented as sciatic pain (2021). A magnetic resonance imaging and fluorodeoxyglucose-positron emission tomography examination revealed that the tumor was likely malignant.
Interventions: Surgical excision was performed.
Outcome: A 10-year follow-up revealed no metastases or neurologic impairment.
Lessons: When articles about benign schwannomas are placed in a separate category, little is written about NF-1-related malignant schwannomas of the sciatic nerve. MPNSTs are high-grade, aggressive sarcomas with a high risk of local recurrence (40%-65%) and metastasis to other body parts. Therefore, among the various benign peripheral nerve sheath tumors in NF-1 patients, the diagnosis of MPNST is crucial.Orthopedic surgeons should be aware that neurofibromas in NF-1 have a significant risk of developing MPNSTs. This study reports the successful treatment of a giant malignant sciatic nerve schwannoma with a long follow-up period without metastasis.
Competing Interests: The authors have no funding and conflicts of interest to disclose.
(Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)
Databáze: MEDLINE