Neurosarcomas, or now commonly known as malignant peripheral nerve sheath tumor (MPNST) are also known as malignant peripheral nerve sheath tumors, malignant schwannomas, and neurofibrosarcomas, are rare.4 They develop from Schwann cells or pluripotent cells of the neural crest and occur mainly in adults. They can arise anywhere in the body, including the upper extremity, but are uncommon in the hand. Most patients with a MPNST tumor are between 20 and 50 years of age and preset with a complaint of an enlarging mass. The male/female ratio is approximately 50:50. Pain complaints are variable. Only 3-10% of soft tissue sarcomas are malignant peripheral nerve sheath tumors.2,3 Diagnosis and treatment are often delayed owing to the absence of symptoms or nonspecific symptoms. Most patients with sporadic neurosarcoma have no risk factors for the disease, but tumor growth is aggressive, and the prognosis is poor. On the other hand, patients with neurofibromatosis type 1 (NF-1) have a lifetime risk of developing an MPNST of 8-13%.3 The 2- and 5-year overall survival rates are 57% and 39%, respectively. In patients with rhabdomyosarcomatous differentiation, the 2- and 5-year survival rates are 15% and 11%, respectively.
Pathophysiology
- About 50–75% of neurosarcomas arise from neurofibromas, typically the plexiform type or in the setting of neurofibromatosis type I (NF1; von Recklinghausen disease) and tend to occur in the head and neck.
- Others arise de novo, usually involving the peripheral nerves of the buttocks or thighs.
- In ~15% of neurosarcomas, epithelioid or heterologous differentiation can be observed. Heterologous differentiation includes rhabdomyoblasts, smooth muscle, bone, cartilage, and neuroendocrine components.
Related Anatomy
- Neurofibrosarcomas are not well encapsulated; therefore, surgery cannot preserve the nerve.
- The lesion often extends through multiple compartments.
- Complete surgical excision with negative microscopic surgical margins improves survival and decreases the risk of local recurrence.3
Incidence and Related Conditions
- Peripheral nerve tumors comprise <5% of all tumors of the hand.
- Neurosarcomas are rare, occurring in only 0.001% of the population.
- Neurosarcomas account for 2–3% of malignant tumors of the hand.
- The typical age range for patients with neurosarcoma is 20–50 years.1,2
Differential Diagnosis
- Chondrosarcoma
- Connective tissue diseases
- Fibrosarcoma
- Gall bladder cancer
- Ganglioneuroma
- Hematoma
- Hydatid cyst
- Liposarcoma
- Metastatic carcinoma
- Osteosarcoma
- Rhabdomyosarcoma
- Schwannoma, benign
- Soft-tissue sarcoma