Acute hydrocephalus due to a primary malignant peripheral nerve sheath tumor of the cervicothoracic junction: A case report and review of the literature
Neurochirurgie, cilt.63, sa.2, ss.91-95, 2017 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Tam Makale
- Cilt numarası: 63 Sayı: 2
- Basım Tarihi: 2017
- Doi Numarası: 10.1016/j.neuchi.2016.10.006
- Dergi Adı: Neurochirurgie
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
- Sayfa Sayıları: ss.91-95
- Anahtar Kelimeler: Hydrocephalus, Primary malignant peripheral nerve sheath tumor, Spinal tumor
- İstanbul Medipol Üniversitesi Adresli: Evet
Özet
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. Case description 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 × 3 cm 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. Conclusion 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.