Surgery at the Crossroads: Craniocervical Neoplasms

Tumors of the craniovertebral junction present with a wide constellation of neurological signs and symptoms, and false localizing signs are numerous. Precipitous decline in function can occur with malignant growth, and the potential for rapid progression to irreversible myelopathy demands early recognition and treatment (29, 38, 47, 56, 65). There is no single symptom or neurological finding pathognomonic for a lesion in this location. Symptoms may arise only after significant size has been achieved because of the generous subarachnoid spaces of the cervicomedullary junction. Anatomic complexities of the region and the decussation of the sensory and motor tracts can lead to erroneous diagnosis and fluctuating neurological symptomatology. Elsberg and Strauss (23) were the first to report on a systematic evaluation of foramen magnum tumors. Since then, several authors have reported on extra-axial lesions affecting the region, such as meningioma and neurinomas, and osseous neoplasms, such as chordoma, chondrosarcoma, plasmacytoma, osteoblastoma, fibrous dysplasia, giant cell tumors, and metastatic tumors. Our experience is summarized in this brief presentation. A retrospective analysis of the medical records and radiographs of patients from the University of Iowa Hospitals and Clinics Neurosurgery database and tumor registry was performed. Eight hundred eight patients were evaluated by the senior author (AHM) at the University of Iowa Hospitals and Clinics between the years 1977 and 2003. Three hundred eighty-two patients presented with osseous tumors, and 426 had neural tumors. Of these, 179 patients underwent operative management by the Neurosurgery Service at the University of Iowa Hospitals and Clinics, and inclusion criterion was histopathology consistent with extramedullary origin in the region of the craniovertebral junction. One hundred seventy-nine patients ultimately underwent operative management with the final pathology as follows: chordoma = 49; meningioma = 41; schwannoma = 5; plasmacytoma = 4; osteoblastoma = 3; neurenteric cyst = 5; calcium pyrophosphate masses = 36; and miscellaneous = 36. The miscellaneous category included fibrous dysplasia, multiple myeloma, dermoid masses, metastatic tumor, Ewing’s sarcoma, and aneursymal bone cyst.