Hemangiopericytoma invading the craniovertebral junction: First reported case and review of the literature
This study reports a case of a 56-year-old fairly healthy male patient presented complaining of neck pain that radiated into his head and both shoulders. His pain was present for several months and no longer relieved by anti-inflammatory medications.\nOn neurological exam, he had intact motor and sensory responses bilaterally, with normal reflexes. Romberg, cerebellar and gait examinations were normal as well. The gadolinium-enhanced cervical spine magnetic resonance imaging (MRI) revealed an intradural extra-medullary lesion extending from the occiput to C4 in the ventrolateral position with central stenosis and cord compression []. In addition, there was a small enhancing lesion seen at the upper thoracic spine []. Both neural foramina were normal. MRIs of the brain and lumbar spine were both within normal limits.\nA suboccipital craniectomy and C1-4 complete laminectomy were performed. With the aid of the microscope, a midline dural incision was made to expose a solid, red tumor in the dorsolateral right gutter, displacing the spinal cord to the left and bordered by clear arachnoid.\nThe tumor was removed in several large fragments and debulked away from a number of nerves in its proximity with a Cavitron. It was found to be densely adherent to the spinal cord at the C1 level around dentate ligaments, extending ventrolaterally. Multiple lobules of tumor were removed in a clear arachnoid plane except where there was adherence to the spinal cord at the C1 level. There was pial invasion on one side, which with cauterization of the pia, the residual tumor was removed and a gross total excision was attained. Careful hemostasis and closure were achieved without issues.\nGrossly, the tumor was a large hemorrhagic mass. The pathologic specimens were designated as intradural extra-medullary and partially invasive intradural intramedullary at the C1 level. On microscopy, the tumor cells had widened nuclei with spotted chromatin and mitotic figures (up to 30 per 10 high-power fields). There were foci of hemorrhage, but no areas of necrosis. There was minimal reticulin deposition. Immunostaining revealed the tumor was positive for CD34 and B-cell lymphoma 2, but negative for smooth muscle actin, epithelial membrane antigen, synaptophysin and glial fibrillary acidic protein and a Ki-67 proliferative index of 12% []. On electron microscopy, the tumor was highly vascular and contained numerous extravasated erythrocytes and other cellular blood elements. Final histology was a tumor borderline grade between HPC WHO grade II based on morphology and anaplastic HPC WHO grade III based on elevated mitotic count and intratumoral hemorrhage.\nThe post-operative course was uneventful and there was complete resection of the tumor as evidenced on MRI. Initially, the patient experienced mild right deltoid weakness without tingling, numbness, headaches or other neurologic symptoms. A post-operative MRI at 6-months revealed the remaining enhancing drop metastasis at the T2-3 level. At an outside facility, the patient underwent successful radiosurgery of the area. His reported right periscapular shoulder girdle atrophy has been responding to physical therapy and other treatments. Five years post-operatively, he is neurologically at baseline and follow-up MRI [] reveals no evidence of recurrence in either the cervical or thoracic regions.