Metastasizing malignant peripheral nerve sheath tumor initially presenting as intracerebral hemorrhage. Case report and review of the literature.

BACKGROUND: Malignant peripheral nerve sheath tumors, infrequent sarcomas arising within a peripheral nerve, mostly metastasize to the lung at terminal stage of disease. However, metastasis to the brain without pulmonary involvement is quite unlikely to occur. CASE DESCRIPTION: A 21-year-old man was brought in the emergency department due to sudden unconsciousness, and imaging studies showed huge intracerebral hemorrhage. Surgical removal and adjuvant therapy was performed for pathologically proven MPNST. Concurrent painful chest masses were also confirmed as MPNST through surgical resection. Nine months after craniotomy, multiple masses in the lung field and axilla region were uncovered, and surgical resection followed. Despite postoperative irradiation, the patient died of the respiratory complications at 16 months after craniotomy.
CONCLUSION: To our knowledge, this kind of metastatic cerebral bleeding is the first case that was ever reported. The MPNST actually can exhibit an apoplectic manifestation even without pulmonary involvement in a young adult, albeit this is quite rare. Thus, high index of suspicion should be paid to minute complaints regarding MPNST in peripheral locations so as not to overlook an advanced or metastasized disease.