Nerventumoren

Nerventumoren, also referred to as peripheral nerve sheath tumors, arise from the cells that comprise the protective covering of peripheral nerves. The most common types include schwannomas, which are benign encapsulated tumors of Schwann cells; neurofibromas, which typically involve multiple nerve fibers and have a higher incidence in patients with neurofibromatosis type 1; and malignant peripheral nerve sheath tumors, which are rare but aggressive cancers that can develop from pre‑existing benign lesions or arise de novo.

Clinical presentation varies with location but often involves a slow‑growing mass, pain, paresthesia, or motor weakness. The majority of sporadic Schwannomas and neurofibromas are benign and asymptomatic until compression of adjacent structures occurs, whereas malignant forms frequently present with rapid enlargement, invasive growth, and systemic symptoms such as weight loss or fatigue. Diagnosis relies on imaging—MRI provides detailed soft‑tissue contrast and delineates tumor boundaries—followed by core or excisional biopsy to confirm histology. Immunohistochemical staining for S‑100 protein is characteristic of Schwann cells and neurofibromas, while high mitotic activity and p53 expression help identify malignancy.

Treatment strategies are tailored to tumour type, size, and symptomatology. Complete surgical excision remains the mainstay for benign lesions and is often curative, with adjuvant radiation considered for marginal resections or unresectable lesions. Malignant peripheral nerve sheath tumours are managed with wide surgical margins when feasible, complemented by radiotherapy and cytotoxic chemotherapy (e.g., doxorubicin or ifosfamide) for metastatic or unresectable disease. Targeted therapies such as MEK inhibitors are emerging for neurofibromatosis type 1‑associated tumours. Long‑term follow‑up is essential due to the risk of recurrence, especially for malignant variants.