Primary intraosseous paraganglioma of the sacrum: a case report.
BACKGROUND
Paragangliomas are neuroendocrine tumors that most frequently arise in the adrenal medulla, carotid body, and glomus jugulare. However, they have been reported in many other sites throughout the body. Within the central nervous system, the overwhelming majority of paragangliomas arise intradurally in the area of the cauda equina. We report a case of a woman who presented with back pain and radiculopathy and was found to have an intraosseous sacral mass on magnetic resonance imaging (MRI). Operative biopsy revealed that this lesion was a paraganglioma. Chest, abdomen, and pelvis computed tomography scans revealed no other tumors. This lesion was treated with limited resection, decompression, stabilization, and radiation therapy.


OBJECTIVE
To report a rare case of an intrasacral paraganglioma that presented with back pain and radiculopathy and was treated with intralesional decompression, stabilization, and radiation.


METHODS
Observational case report.


METHODS
A 69-year-old woman presented with back and leg pain. An MRI scan revealed a grade II spondylolisthesis at L5/S1 and a left-sided intraosseus mass at S1 with significant obstruction of the left S1 nerve root foramen. The patient underwent initially an open biopsy of this lesion that revealed a paraganglioma. Subsequently, she underwent transarterial embolization of this lesion, partial resection with decompression of the nerve root, and stabilization and arthrodesis from L4 to the ilium.


RESULTS
Intraoperatively, a highly vascular lesion was encountered. The patient's back and leg pain improved significantly after surgery. She was referred for intensity-modulated radiation therapy. One year after surgery, she was neurologically intact without evidence of growth of the lesion.


CONCLUSIONS
Only two cases of primary intraosseous sacral paragangliomas were reported in the past. We believe that paragangliomas should be included in the differential diagnosis of intraosseous sacral tumors. Optimal treatment of the lesions can be difficult because of their hypervascular nature and the high morbidity associated with complete surgical resection. Treatment should combine preoperative embolisation, surgical resection if possible, and radiation therapy.
Laufer
Ilya
I
Department of Neurological Surgery, Weill Medical College of Cornell University, New York Presbyterian Hospital, New York, NY 10021, USA. ilyalaufer@yahoo.com
Edgar
Mark A
MA
Härtl
Roger
R
D000368:Aged; D001416:Back Pain / Q000473:pathology / Q000601:surgery; D001706:Biopsy; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D010235:Paraganglioma / Q000473:pathology* / Q000601:surgery; D011843:Radiculopathy / Q000473:pathology / Q000601:surgery; D012447:Sacrum / Q000473:pathology* / Q000601:surgery; D013123:Spinal Fusion; D013125:Spinal Neoplasms / Q000473:pathology* / Q000601:surgery