RESUMO
BACKGROUND: Paragangliomas are tumors that arise from the paraganglion system, which is a component of the neuroendocrine system. Approximately 10% are located in the extra-adrenal paraganglion system. Paragangliomas of the spine, however, are rare. They usually present as an intradural tumor in the cauda equina. There are only three reports of primary intraosseous paragangliomas of the sacrum. CASE DESCRIPTION: A 69-year-old man presented with low back pain and urinary incontinence. Imaging revealed a large intraosseous mass at S2, S3 and S4. Surgical resection was accomplished through a posterior midline incision exposing the spine from L5 to the coccyx. The tumor was located in the extradural space. It was friable, grayish and bleeding. Total tumor removal was performed, with normal bone margins. Follow-up at 2 years showed complete resolution of the preoperative symptoms and no evidence of local recurrence. CONCLUSION: Although rare, the possibility of paraganglioma should be included in the differential diagnosis of sacral tumors. The majority of the spinal paragangliomas are benign, slowly growing tumors with low proliferative activity. Despite these characteristics, local recurrence has been reported in cases of both macroscopically total and subtotal resection. Postoperative radiation therapy for patients with incomplete excision may not prevent recurrence, so gross tumor removal should be the goal of surgery.