Spinal Paraganglioma- Case Report and Review of the Literature

Abstract

Spinal paraganglioma represents an unusual tumor from neuroendocrine origin without the secretory properties typical for some of the tumors arising outside of the nervous system. In none of the few cases reported in the literature preoperative diagnosis was possible and surgical findings raised questions in the differential diagnosis. We report 37-year-old male who presented with lumbar back pain and slowly progressive weakness for the lower extremities. MRI demonstrated huge intradural extramedullar spinal tumor in the lumbar region from L1 to S1, compressing the medulla and displacing the radices. Preoperative differential diagnosis was neurinoma or ependymoma. Intraoperatively the tumor resembled myxopapillary ependymoma or neurinoma. The tumor underwent total excision and histological study. Histology allowed precise identification as the patient was diagnosed with spinal paraganglioma (WHO grade I). Morphological description of the histological findings was a tumor composed of cell groups (Zellballen) - monomorphic tumor cells with abundant eosinophilic cytoplasm and round nuclei surrounded by a layer of sustentacular cells and delicate fibrovascular stroma; tumor cells expressed diffuse and intensive - Chromogranin and Vimentin; tumor cells did not express GFAP, CK AE1/AE3, EMA and Ki - 67; S -100 protein was expressed by sustentacular cells. Postoperatively, neurological deterioration was not observed. At 6 months follow-up, the patient demonstrated full recovery and no data for distant metastases. In patients suspected for spinal paraganlioma MRI is the study of choice for preoperative evaluation, but there are overlapping findings, mainly ependymoma and neurinoma. The complete removal of spinal paraganglioma is possible and the prognosis after that is excellent.