{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T11:31:05Z","timestamp":1761823865996},"reference-count":26,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["SPI"],"published-print":{"date-parts":[[2017,4]]},"abstract":"<jats:p>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-<jats:sup>18<\/jats:sup>F-fluoro-<jats:sc>l<\/jats:sc>-phenylalanine (<jats:sup>18<\/jats:sup>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. <jats:sup>18<\/jats:sup>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.<\/jats:p>","DOI":"10.3171\/2016.10.spine16948","type":"journal-article","created":{"date-parts":[[2017,1,27]],"date-time":"2017-01-27T15:02:27Z","timestamp":1485529347000},"page":"501-506","source":"Crossref","is-referenced-by-count":9,"title":["Leptomeningeal dissemination of a low-grade lumbar paraganglioma: case report"],"prefix":"10.3171","volume":"26","author":[{"given":"Nick","family":"Thomson","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, University of Arizona College of Medicine Phoenix, Arizona;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karel","family":"Pacak","sequence":"additional","affiliation":[{"name":"Section on Medical Neuroendocrinology, National Institutes of Health, Bethesda, Maryland;\u00a0"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Meic H.","family":"Schmidt","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery;"},{"name":"Huntsman Cancer Institute, Salt Lake City, Utah"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cheryl A.","family":"Palmer","sequence":"additional","affiliation":[{"name":"Pathology, Division of Anatomic Pathology;"},{"name":"Huntsman Cancer Institute, Salt Lake City, Utah"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Karen L.","family":"Salzman","sequence":"additional","affiliation":[{"name":"Radiology, Division of Neuroradiology;"},{"name":"Huntsman Cancer Institute, Salt Lake City, Utah"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marjan","family":"Champine","sequence":"additional","affiliation":[{"name":"Population Health Sciences;"},{"name":"Huntsman Cancer Institute, Salt Lake City, Utah"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joshua D.","family":"Schiffman","sequence":"additional","affiliation":[{"name":"Pediatrics and Oncological Sciences; and"},{"name":"Huntsman Cancer Institute, Salt Lake City, Utah"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adam L.","family":"Cohen","sequence":"additional","affiliation":[{"name":"Medicine, Division of Oncology, University of Utah, Salt Lake City; and"},{"name":"Huntsman Cancer Institute, Salt Lake City, Utah"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"b1-spine16948","series-title":"J Clin Endocrinol Metab","doi-asserted-by":"publisher","first-page":"2110","DOI":"10.1210\/jc.2004-1398","article-title":"Year of diagnosis, features at presentation, and risk of recurrence in patients with pheochromocytoma or secreting paraganglioma","volume":"90","author":"Amar L,","year":"2005","unstructured":"Amar L, Servais A, Gimenez-Roqueplo AP, Zinzindohoue F, Chatellier G, Plouin PF: Year of diagnosis, features at presentation, and risk of recurrence in patients with pheochromocytoma or secreting paraganglioma. J Clin Endocrinol Metab 90:2110\u20132116, 20051564440110.1210\/jc.2004-1398"},{"key":"b2-spine16948","series-title":"J Nucl Med","doi-asserted-by":"publisher","first-page":"1849","DOI":"10.2967\/jnumed.115.159061","article-title":"18F-FLT PET\/CT in the evaluation of pheochromocytomas and paragangliomas: a pilot study","volume":"56","author":"Blanchet EM,","year":"2015","unstructured":"Blanchet EM, Taieb D, Millo C, Martucci V, Chen CC, Merino M, : 18F-FLT PET\/CT in the evaluation of pheochromocytomas and paragangliomas: a pilot study. J Nucl Med 56:1849\u20131854, 20152635926110.2967\/jnumed.115.159061"},{"key":"b3-spine16948","series-title":"Eur J Hum Genet","doi-asserted-by":"publisher","first-page":"457","DOI":"10.1038\/sj.ejhg.5200829","article-title":"Identification of novel SDHD mutations in patients with phaeochromocytoma and\/or paraganglioma","volume":"10","author":"Cascon A,","year":"2002","unstructured":"Cascon A, Ruiz-Llorente S, Cebrian A, Telleria D, Rivero JC, Diez JJ, : Identification of novel SDHD mutations in patients with phaeochromocytoma and\/or paraganglioma. Eur J Hum Genet 10:457\u2013461, 200210.1038\/sj.ejhg.520082912111639"},{"key":"b4-spine16948","series-title":"Endocr Relat Cancer","doi-asserted-by":"publisher","first-page":"T135","DOI":"10.4103\/1450-1147.167575","article-title":"15 years of paraganglioma: imaging and imaging-based treatment of pheochromocytoma and paraganglioma","volume":"22","author":"Castinetti F,","year":"2015","unstructured":"Castinetti F, Kroiss A, Kumar R, Pacak K, Taieb D: 15 years of paraganglioma: imaging and imaging-based treatment of pheochromocytoma and paraganglioma. Endocr Relat Cancer 22:T135\u2013T145, 201510.4103\/1450-1147.167575"},{"key":"b5-spine16948","series-title":"Pancreas","doi-asserted-by":"publisher","first-page":"775","DOI":"10.1097\/MPA.0b013e3181ebb4f0","article-title":"The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer","volume":"39","author":"Chen H,","year":"2010","unstructured":"Chen H, Sippel RS, O'Dorisio MS, Vinik AI, Lloyd RV, Pacak K: The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas 39:775\u2013783, 20102066447510.1097\/MPA.0b013e3181ebb4f0"},{"key":"b6-spine16948","series-title":"West J Med","first-page":"106","article-title":"Treatment of malignant pheochromocytoma","volume":"128","author":"Drasin H:","year":"1978","unstructured":"Drasin H: Treatment of malignant pheochromocytoma. West J Med 128:106\u2013111, 1978629048"},{"key":"b7-spine16948","series-title":"Endocr Pathol","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1007\/s12022-011-9188-1","article-title":"Diagnostic tests and biomarkers for pheochromocytoma and extra-adrenal paraganglioma: from routine laboratory methods to disease stratification","volume":"23","author":"Eisenhofer G,","year":"2012","unstructured":"Eisenhofer G, Tischler AS, de Krijger RR: Diagnostic tests and biomarkers for pheochromocytoma and extra-adrenal paraganglioma: from routine laboratory methods to disease stratification. Endocr Pathol 23:4\u201314, 201210.1007\/s12022-011-9188-122180288"},{"key":"b8-spine16948","series-title":"Horm Metab Res","doi-asserted-by":"publisher","first-page":"405","DOI":"10.1055\/s-0032-1308992","article-title":"External beam radiation therapy (EBRT) for patients with malignant pheochromocytoma and non-head and -neck paraganglioma: combination with 131I-MIBG","volume":"44","author":"Fishbein L,","year":"2012","unstructured":"Fishbein L, Bonner L, Torigian DA, Nathanson KL, Cohen DL, Pryma D, : External beam radiation therapy (EBRT) for patients with malignant pheochromocytoma and non-head and -neck paraganglioma: combination with 131I-MIBG. Horm Metab Res 44:405\u2013410, 201210.1055\/s-0032-130899222566196"},{"key":"b9-spine16948","series-title":"Ann Surg Oncol","doi-asserted-by":"publisher","first-page":"1444","DOI":"10.1245\/s10434-013-2942-5","article-title":"Inherited mutations in pheochromocytoma and paraganglioma: why all patients should be offered genetic testing","volume":"20","author":"Fishbein L,","year":"2013","unstructured":"Fishbein L, Merrill S, Fraker DL, Cohen DL, Nathanson KL: Inherited mutations in pheochromocytoma and paraganglioma: why all patients should be offered genetic testing. Ann Surg Oncol 20:1444\u20131450, 20132351207710.1245\/s10434-013-2942-5"},{"key":"b10-spine16948","series-title":"Am J Cancer Res","first-page":"1558","article-title":"Potential therapeutic target for malignant paragangliomas: ATP synthase on the surface of paraganglioma cells","volume":"5","author":"Fliedner SMJ,","year":"2015","unstructured":"Fliedner SMJ, Yang C, Thompson E, Abu-Asab M, Hsu CM, Lampert G, : Potential therapeutic target for malignant paragangliomas: ATP synthase on the surface of paraganglioma cells. Am J Cancer Res 5:1558\u20131570, 201526101719"},{"key":"b11-spine16948","series-title":"World J Nucl Med","first-page":"71","article-title":"Ga-68 DOTA-NOC PET\/CT for the detection of residual\/recurrence in a rare case of sacral spinal canal paraganglioma","volume":"15","author":"Jain TK,","year":"2016","unstructured":"Jain TK, Basher RK, Shukla J, Bhattacharya A, Prakash M, Mittal BR: Ga-68 DOTA-NOC PET\/CT for the detection of residual\/recurrence in a rare case of sacral spinal canal paraganglioma. World J Nucl Med 15:71\u201372, 2016"},{"key":"b12-spine16948","series-title":"Eur J Nucl Med Mol Imaging","doi-asserted-by":"publisher","first-page":"1784","DOI":"10.1007\/s00259-016-3357-x","article-title":"PET\/CT comparing 68Ga-DOTATATE and other radiopharmaceuticals and in comparison with CT\/MRI for the localization of sporadic metastatic pheochromocytoma and paraganglioma","volume":"43","author":"Janssen I,","year":"2016","unstructured":"Janssen I, Chen CC, Millo CM, Ling A, Taieb D, Lin FI, : PET\/CT comparing 68Ga-DOTATATE and other radiopharmaceuticals and in comparison with CT\/MRI for the localization of sporadic metastatic pheochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging 43:1784\u20131791, 201610.1007\/s00259-016-3357-x26996779"},{"key":"b13-spine16948","series-title":"Am J Surg Pathol","doi-asserted-by":"publisher","first-page":"109","DOI":"10.1097\/00000478-198004000-00002","article-title":"Extra-adrenal paragangliomas of the retroperitoneum: A clinicopathologic study of 12 tumors","volume":"4","author":"Lack EE,","year":"1980","unstructured":"Lack EE, Cubilla AL, Woodruff JM, Lieberman PH: Extra-adrenal paragangliomas of the retroperitoneum: A clinicopathologic study of 12 tumors. Am J Surg Pathol 4:109\u2013120, 198010.1097\/00000478-198004000-000027377461"},{"key":"b14-spine16948","series-title":"World J Surg","doi-asserted-by":"publisher","first-page":"683","DOI":"10.1007\/s00268-007-9360-4","article-title":"Sporadic paraganglioma","volume":"32","author":"Lee JA,","year":"2008","unstructured":"Lee JA, Duh QY: Sporadic paraganglioma. World J Surg 32:683\u2013687, 200810.1007\/s00268-007-9360-418224469"},{"key":"b15-spine16948","series-title":"Mayo Clin Proc","doi-asserted-by":"publisher","first-page":"348","DOI":"10.1016\/S0025-6196(12)64856-3","article-title":"Surgical resection of carotid body tumors: long-term survival, recurrence, and metastasis","volume":"63","author":"Nora JD,","year":"1988","unstructured":"Nora JD, Hallett JW Jr, O'Brien PC, Naessens JM, Cherry KJ Jr, Pairolero PC: Surgical resection of carotid body tumors: long-term survival, recurrence, and metastasis. Mayo Clin Proc 63:348\u2013352, 198810.1016\/S0025-6196(12)64856-33352318"},{"key":"b16-spine16948","series-title":"Cancer","doi-asserted-by":"publisher","first-page":"1358","DOI":"10.1002\/1097-0142(196906)23:6<1358::AID-CNCR2820230618>3.0.CO;2-#","article-title":"Nonfunctional, nonchromaffin paragangliomas of the retroperitoneum","volume":"23","author":"Olson JR,","year":"1969","unstructured":"Olson JR, Abell MR: Nonfunctional, nonchromaffin paragangliomas of the retroperitoneum. Cancer 23:1358\u20131367, 196910.1002\/1097-0142(196906)23:6<1358::AID-CNCR2820230618>3.0.CO;2-#4306104"},{"key":"b17-spine16948","series-title":"Med Oncol","doi-asserted-by":"publisher","first-page":"47","DOI":"10.1007\/s12032-016-0737-9","article-title":"Peptide receptor radionuclide therapy for metastatic paragangliomas","volume":"33","author":"Pinato DJ,","year":"2016","unstructured":"Pinato DJ, Black JRM, Ramaswami R, Tan TM, Adjogatse D, Sharma R: Peptide receptor radionuclide therapy for metastatic paragangliomas. Med Oncol 33:47, 20162705936310.1007\/s12032-016-0737-9"},{"key":"b18-spine16948","series-title":"J Pediatr Endocrinol Metab","doi-asserted-by":"publisher","first-page":"837","DOI":"10.1515\/jpem-2013-0369","article-title":"Counseling patients with succinate dehydrogenase subunit defects: genetics, preventive guidelines, and dealing with uncertainty","volume":"27","author":"Raygada M,","year":"2014","unstructured":"Raygada M, King KS, Adams KT, Stratakis CA, Pacak K: Counseling patients with succinate dehydrogenase subunit defects: genetics, preventive guidelines, and dealing with uncertainty. J Pediatr Endocrinol Metab 27:837\u2013844, 20142485453010.1515\/jpem-2013-0369"},{"key":"b19-spine16948","series-title":"Acta Neurochir (Wien)","doi-asserted-by":"publisher","first-page":"475","DOI":"10.1007\/BF01420312","article-title":"Cauda equina paraganglioma with subsequent intracranial and intraspinal metastases","volume":"138","author":"Roche PH,","year":"1996","unstructured":"Roche PH, Figarella-Branger D, Regis J, Peragut JC: Cauda equina paraganglioma with subsequent intracranial and intraspinal metastases. Acta Neurochir (Wien) 138:475\u2013479, 199610.1007\/BF014203128738400"},{"key":"b20-spine16948","series-title":"Clin Endocrinol (Oxf)","doi-asserted-by":"publisher","first-page":"789","DOI":"10.1111\/cen.12566","article-title":"Role of 18F-FDOPA PET\/CT imaging in endocrinology","volume":"81","author":"Santhanam P,","year":"2014","unstructured":"Santhanam P, Ta\u00efeb D: Role of 18F-FDOPA PET\/CT imaging in endocrinology. Clin Endocrinol (Oxf) 81:789\u2013798, 201410.1111\/cen.1256625056984"},{"key":"b21-spine16948","series-title":"BMC Cancer","doi-asserted-by":"publisher","first-page":"523","DOI":"10.1186\/1471-2407-14-523","article-title":"The size of the primary tumor and age at initial diagnosis are independent predictors of the metastatic behavior and survival of patients with SDHB-related pheochromocytoma and paraganglioma: a retrospective cohort study","volume":"14","author":"Schovanek J,","year":"2014","unstructured":"Schovanek J, Martucci V, Wesley R, Fojo T, Del Rivero J, Huynh T, : The size of the primary tumor and age at initial diagnosis are independent predictors of the metastatic behavior and survival of patients with SDHB-related pheochromocytoma and paraganglioma: a retrospective cohort study. BMC Cancer 14:523, 201410.1186\/1471-2407-14-52325048685"},{"key":"b22-spine16948","series-title":"Am J Surg","doi-asserted-by":"publisher","first-page":"732","DOI":"10.1016\/0002-9610(71)90436-3","article-title":"Carotid body tumor (chemodectoma). Clinicopathologic analysis of ninety cases","volume":"122","author":"Shamblin WR,","year":"1971","unstructured":"Shamblin WR, ReMine WH, Sheps SG, Harrison EG Jr: Carotid body tumor (chemodectoma). Clinicopathologic analysis of ninety cases. Am J Surg 122:732\u2013739, 197110.1016\/0002-9610(71)90436-35127724"},{"key":"b23-spine16948","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"166","DOI":"10.3171\/jns.1995.83.1.0166","article-title":"Symptomatic cerebellar metastasis and late local recurrence of a cauda equina paraganglioma. Case report","volume":"83","author":"Strommer KN,","year":"1995","unstructured":"Strommer KN, Brandner S, Sarioglu AC, Sure U, Yonekawa Y: Symptomatic cerebellar metastasis and late local recurrence of a cauda equina paraganglioma. Case report. J Neurosurg 83:166\u2013169, 199510.3171\/jns.1995.83.1.01667782837"},{"key":"b24-spine16948","series-title":"Surgery","doi-asserted-by":"publisher","first-page":"986","DOI":"10.1016\/j.surg.2009.10.055","article-title":"Local-regional recurrence of sporadic or syndromic abdominal extra-adrenal paraganglioma: incidence, characteristics, and outcome","volume":"146","author":"Van Slycke S,","year":"2009","unstructured":"Van Slycke S, Caiazzo R, Pigny P, Cardot-Bauters C, Arnalsteen L, D'Herbomez M, : Local-regional recurrence of sporadic or syndromic abdominal extra-adrenal paraganglioma: incidence, characteristics, and outcome. Surgery 146:986\u2013992, 200910.1016\/j.surg.2009.10.055"},{"key":"b25-spine16948","series-title":"Front Oncol","doi-asserted-by":"publisher","first-page":"166","DOI":"10.3389\/fonc.2014.00166","article-title":"External beam radiation therapy in treatment of malignant pheochromocytoma and paraganglioma","volume":"4","author":"Vogel J,","year":"2014","unstructured":"Vogel J, Atanacio AS, Prodanov T, Turkbey BI, Adams K, Martucci V, : External beam radiation therapy in treatment of malignant pheochromocytoma and paraganglioma. Front Oncol 4:166, 201410.3389\/fonc.2014.0016625019060"},{"key":"b26-spine16948","series-title":"Brain Res","doi-asserted-by":"publisher","first-page":"439","DOI":"10.1016\/S0006-8993(02)03543-6","article-title":"Resveratrol protects against global cerebral ischemic injury in gerbils","volume":"958","author":"Wang Q,","year":"2002","unstructured":"Wang Q, Xu J, Rottinghaus GE, Simonyi A, Lubahn D, Sun GY, : Resveratrol protects against global cerebral ischemic injury in gerbils. Brain Res 958:439\u2013447, 200210.1016\/S0006-8993(02)03543-612470882"}],"container-title":["Journal of Neurosurgery: Spine"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-spine\/26\/4\/article-p501.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-spine\/26\/4\/article-p501.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-spine\/26\/4\/article-p501.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,15]],"date-time":"2023-04-15T22:24:32Z","timestamp":1681597472000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-spine\/26\/4\/article-p501.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,4]]},"references-count":26,"journal-issue":{"issue":"4"},"URL":"https:\/\/doi.org\/10.3171\/2016.10.spine16948","relation":{},"ISSN":["1547-5654"],"issn-type":[{"value":"1547-5654","type":"print"}],"subject":[],"published":{"date-parts":[[2017,4]]}}}