title: Leptomeningeal dissemination of a low-grade lumbar paraganglioma: case report.
abstract: 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-6-18F-fluoro-l-phenylalanine (18F-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.18F-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.
authors.affiliation: Department of Internal Medicine, University of Arizona College of Medicine Phoenix, Arizona
authors.forename: Nick
authors.initials: N
authors.lastname: Thomson
authors.affiliation: Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland
authors.forename: Karel
authors.initials: K
authors.lastname: Pacak
authors.affiliation: Department of Neurosurgery, University of Utah, Salt Lake City, Utah|Huntsman Cancer Institute, Salt Lake City, Utah
authors.forename: Meic
authors.initials: MH
authors.lastname: Schmidt
authors.affiliation: Department of Pathology, Division of Anatomic Pathology, University of Utah, Salt Lake City, Utah|Huntsman Cancer Institute, Salt Lake City, Utah
authors.forename: Cheryl
authors.initials: CA
authors.lastname: Palmer
authors.affiliation: Department of Radiology, Division of Neuroradiology, University of Utah, Salt Lake City, Utah|Huntsman Cancer Institute, Salt Lake City, Utah
authors.forename: Karen
authors.initials: KL
authors.lastname: Salzman
authors.affiliation: Huntsman Cancer Institute, Salt Lake City, Utah|Department of Population Health Sciences, University of Utah, Salt Lake City, Utah
authors.forename: Marjan
authors.initials: M
authors.lastname: Champine
authors.affiliation: Department of Pediatrics and Oncological Sciences, University of Utah, Salt Lake City, Utah|Huntsman Cancer Institute, Salt Lake City, Utah
authors.forename: Joshua
authors.initials: JD
authors.lastname: Schiffman
authors.affiliation: Department of Medicine, Division of Oncology, University of Utah, Salt Lake City, Utah|Huntsman Cancer Institute, Salt Lake City, Utah
authors.forename: Adam
authors.initials: AL
authors.lastname: Cohen
mesh_terms: D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D017116:Low Back Pain / Q000209:etiology / Q000628:therapy; D008577:Meningeal Neoplasms / Q000000981:diagnostic imaging / Q000556:secondary* / Q000628:therapy; D008875:Middle Aged; D010235:Paraganglioma / Q000000981:diagnostic imaging / Q000473:pathology* / Q000556:secondary* / Q000628:therapy; D013120:Spinal Cord Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy
keywords: 18F-FDOPA = 3,4-dihydroxy-6-18F-fluoro-l-phenylalanine; 18F-FLT = 3′-deoxy-3′-18F-fluorothymidine; lumbar; metastasis; neuroendocrine tumor; oncology; paraganglioma; spinal neoplasm