Leptomeningeal Dissemination of a Low-grade Lumbar Paraganglioma: Case Report
Leptomeningeal dissemination of paraganglioma is rare, with only 2 prior cases in the literature. The authors present the case of a metastatic low-grade lumbar paraganglioma via leptomeningeal dissemination. This report emphasizes the utility of 3,4-dihydroxy-6-(18)F-fluoro-l-phenylalanine ((18)F-FDOPA) PET scanning for diagnosis, as well as the combination of radiation therapy and alkylating chemotherapeutic agents for the treatment of this rare phenomenon. The patient was a 61-year-old woman who presented with low-back pain and was found to have an isolated L-3 intrathecal tumor on MRI. Sixteen months after gross-total en bloc resection of the paraganglioma, the patient again became symptomatic with new neurological symptoms. MRI findings revealed enhancing leptomeningeal nodules throughout the spine. (18)F-FDOPA PET/CT scanning was used to confirm the diagnosis of disseminated paraganglioma. Intrathecal thiotepa, radiation therapy, and systemic therapy with capecitabine and temozolomide have been used sequentially over a 2-year period, with each able to stabilize tumor growth for several months. The authors also summarize the 2 other reports of leptomeningeal dissemination of paragangliomas in the literature and compare the course and management of the 3 cases.
Thomson, N., Pacak, K., Schmidt, M., Palmer, C., Salzman, K., Champine, M., Schiffman, J., & Cohen, A. (2017). Leptomeningeal Dissemination of a Low-grade Lumbar Paraganglioma: Case Report. Journal of Neurosurgery.Spine, 26 (4), 501-506. https://doi.org/10.3171/2016.10.SPINE16948