title: Clinical characteristics and outcomes of patients with Hodgkin lymphoma with central nervous system involvement: An international multicenter collaboration.
abstract: Central nervous system (CNS) involvement is rare in patients with Hodgkin lymphoma (HL). Thus, the clinical features and outcomes are not well described. Cases of histologically confirmed CNS HL diagnosed between 1995 and 2015 were retrospectively identified in institutional (n = 7), national (n = 2), and cooperative group (n = 1) databases. We screened 30,781 patients with HL in our combined databases and identified 21 patients meeting eligibility criteria, an estimated frequency of 0.07%. CNS involvement was present at initial diagnosis in 10 patients (48%) and a feature of relapsed/refractory disease in 11 (52%). Among these 11 patients, the median time from initial diagnosis of HL to development of CNS involvement was 1.9 years (range 0.4-6.6) and the median number of prior lines of therapy was 2 (range 1-7). Altogether, treatments included radiation, multiagent systemic chemotherapy, combined modality therapy, and subtotal resection. The overall response rate was 65%. After a median follow-up of 3.6 years (range 0.8-13.2) from diagnosis of CNS HL, the median PFS and OS were 7.6 and 29 months, respectively. CNS involvement as a feature of relapsed/refractory disease was adversely prognostic for both PFS and OS; however, four patients remain alive and free of relapse at 7-78 months follow-up. CNS involvement in HL is exceedingly rare and has a distinct clinical presentation with predilection for parenchymal lesions with dural extension. Around one-quarter of patients, mostly with CNS involvement at initial HL diagnosis, experience prolonged disease-free survival. Am. J. Hematol. 91:894-899, 2016. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Department of Lymphoma/Myeloma, University of Texas MD Anderson Cancer Center, Houston, Texas.|Department of Haematology, Sir Charles Gairdner Hospital and Pathwest Laboratory Medicine WA, Perth, Western Australia, Australia.|University of Western Australia, Crawley, Western Australia, Australia.
authors.forename: Chan Y
authors.initials: CY
authors.lastname: Cheah
authors.affiliation: Department of Internal Medicine and German Hodgkin Study Group, University Hospital of Cologne, Cologne, Germany.|Lymphoma Service, Memorial Sloan Kettering Cancer Center, New York, New York.
authors.forename: Paul J
authors.initials: PJ
authors.lastname: Bröckelmann
authors.affiliation: Department of Lymphoma/Myeloma, University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Dai
authors.initials: D
authors.lastname: Chihara
authors.affiliation: Lymphoma Service, Memorial Sloan Kettering Cancer Center, New York, New York.
authors.forename: Alison J
authors.initials: AJ
authors.lastname: Moskowitz
authors.affiliation: Department of Internal Medicine and German Hodgkin Study Group, University Hospital of Cologne, Cologne, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Engert
authors.affiliation: Department of Oncology, Lund University, Lund, Sweden.
authors.forename: Mats
authors.initials: M
authors.lastname: Jerkeman
authors.affiliation: Department of Hematology, Aalborg University Hospital, Aalborg, Denmark.
authors.forename: Tarec C
authors.initials: TC
authors.lastname: El-Galaly
authors.affiliation: Department of Haematology, Sir Charles Gairdner Hospital and Pathwest Laboratory Medicine WA, Perth, Western Australia, Australia.|University of Western Australia, Crawley, Western Australia, Australia.
authors.forename: Bradley
authors.initials: B
authors.lastname: Augustson
authors.affiliation: Department of Hematology/Oncology, University of Nebraska Medical Center, Omaha, Nebraska.
authors.forename: Julie
authors.initials: J
authors.lastname: Vose
authors.affiliation: Division of Oncology, Washington University, St Louis, Missouri.
authors.forename: Nancy L
authors.initials: NL
authors.lastname: Bartlett
authors.affiliation: British Columbia Cancer Agency Centre for Lymphoid Cancer, Vancouver, British Columbia, Canada.
authors.forename: Diego
authors.initials: D
authors.lastname: Villa
authors.affiliation: British Columbia Cancer Agency Centre for Lymphoid Cancer, Vancouver, British Columbia, Canada.
authors.forename: Joseph M
authors.initials: JM
authors.lastname: Connors
authors.affiliation: Department of Hematology, Hackensack University Cancer Center, Hackensack, New Jersey.
authors.forename: Tatyana
authors.initials: T
authors.lastname: Feldman
authors.affiliation: Department of Radiation Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Chelsea C
authors.initials: CC
authors.lastname: Pinnix
authors.affiliation: Department of Radiation Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Sarah A
authors.initials: SA
authors.lastname: Milgrom
authors.affiliation: Department of Radiation Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Bouthaina
authors.initials: B
authors.lastname: Dabaja
authors.affiliation: Department of Lymphoma/Myeloma, University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Oki
authors.affiliation: Department of Lymphoma/Myeloma, University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: Michelle A
authors.initials: MA
authors.lastname: Fanale
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016543:Central Nervous System Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D006689:Hodgkin Disease / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult