Acute hydrocephalus due to a primary malignant peripheral nerve sheath tumor of the cervicothoracic junction: A case report and review of the literature.

BACKGROUND

The estimated incidence of malignant nerve sheath tumors is 0.001% per year, and only 2-3% of those tumors involve the spinal nerves. We present a rare case of acute hydrocephalus caused by primary malignant peripheral nerve sheath tumor of the cervicothoracic junction.

METHODS

A 29-year-old previously healthy male patient, except for a history of two previous surgeries for ulnar nerve entrapment and progressive left upper extremity weakness, presented with acute onset somnolence. The CT and MRI revealed hydrocephalus and periventricular edema. The patient underwent ventriculoperitoneal shunt surgery. Postoperative MRI of the spine revealed a 6×3×3cm intra-extradural lesion at C7-T1 level and multiple metastases in other spinal segments. The patient underwent combined surgical excision and the tumor was diagnosed as a malignant peripheral nerve sheath tumor based on pathological and immunohistological findings. Radiation therapy and chemotherapy were initiated.

CONCLUSIONS

Primary malignant peripheral nerve sheath tumor of the spine is a very aggressive tumor with a very high recurrence rate, significant potential for metastasis and very poor overall prognosis. They may present with features of more frequent diseases, such as peripheral neuropathies and may be overlooked as in our case. Thus, suspected cases should undergo a more detailed examination.