Abstract
IntroductionParagangliomas are rare tumors that originate from the autonomic nervous system-associated paraganglia. They metastasize infrequently. Malignancy can only be demonstrated by the presence of chromaffin tissue at sites where it usually is not present, such as bone, lung or liver, or local recurrence after total resection of a primary mass. Paragangliomas within the central nervous system are usually intradural near the conus medullaris. The metastatic spread of a retroperitoneal paraganglioma to a vertebral body is extremely rare, and there are only a few cases reported in the literature.Case presentationWe report the case of a 16-year-old Caucasian girl who had undergone resection of a retroperitoneal paraganglioma that measured 15 × 11.5 × 9.5 cm. After further staging, a solitary metastatic paraganglioma was detected in the first lumbar vertebral body. After initial chemotherapy, marginal en bloc resection and reconstruction were performed followed by radiotherapy. Histologic examination of the specimen revealed that the tumor cells did not show any response to preoperative chemotherapy, which is in line with a few other reports in the literature. Ten years after operative treatment, the patient is free of complaints, very satisfied with the result and without signs of local recurrence or distant metastases.ConclusionWe recommend en bloc spondylectomy and local radiotherapy in the treatment of solitary spinal metastatic paragangliomas.
Highlights
Paragangliomas are rare tumors that originate from the autonomic nervous system-associated paraganglia
Case presentation: We report the case of a 16-year-old Caucasian girl who had undergone resection of a retroperitoneal paraganglioma that measured 15 × 11.5 × 9.5 cm
We recommend en bloc spondylectomy and local radiotherapy in the treatment of solitary spinal metastatic paragangliomas
Summary
Paraganglioma is a rare tumor that originates from the autonomic nervous system-associated paraganglia. 90% of paragangliomas arise from the adrenal medulla, carotid body and glomus jugulare [1,2,3]. Preoperative chemotherapy, en bloc spondylectomy and postoperative radiotherapy were performed. The postoperative course was uneventful, but because of the potential malignant behavior of extra-adrenal paragangliomas, Tc-99-MDP (Tc-99m-methylene diphosphonate) and I-123-MIBG (123 I-metaiodobenzylguanidine) scintigraphy was performed 10 and 21 days postoperatively. Because of the marginal resection and the poor response to preoperative chemotherapy, postoperative radiation therapy was added with a dose of 50 Gy. Ten years postoperatively, the 26-year-old female patient is in excellent general condition without signs of local recurrence or further distant metastasis. CT has revealed that the autologous bone within the cage is mineralized and has most probably fused with the adjacent endplates of the T12 and L2 vertebral bodies, as far as this can be evaluated with this or any other imaging technique
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