Intraosseous Sacral Paraganglioma with Extradural Extension

Abstract

Introduction 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. Patient and Methods: 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. Results Extra-adrenal paragangliomas have been found in locations where paraganglia are not normally located, such as the stomach, duodenum, uterus, and lungs. In the central nervous system, a few cases in the cerebellopontine angle and cerebellum have been described. A spinal location is also rare; tumors have been found in the intradural, extramedullary compartment, predominantly in the lumbar region and, rarely, in the cervical or thoracic regions. Only three cases of paraganglioma originating primarily within sacral bone have been reported. In 1998, Coles reported the first case of a primary intraosseous tumor, located at S3. This was followed by a report from Sundgern in 1999 of another case located at the S1 vertebral body. Recently, in 2007, Laufer reported the third case of primary sacral paraganglioma, located at the S1 vertebral body. 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.