title: Hemangioblastomas with leptomeningeal dissemination: case series and review of the literature.
abstract: Leptomeningeal dissemination of hemangioblastomas (HB) of the central nervous system (CNS) is extremely rare. Few studies have reported leptomeningeal involvement in sporadic HB or in HB associated with von Hippel-Lindau syndrome. The clinical and radiological features of leptomeningeal involvement in HB after surgery have not been described in detail.
  
  
  
  This retrospective case review involved patients from three different tertiary referral centers with leptomeningeal dissemination of HB after surgery for the primary mass. A literature review was also performed to describe the clinical and radiological characteristics and long-term outcomes of patients who developed leptomeningeal dissemination after initial surgical resection.
  
  
  
  This study included seven patients, five males and two females, ranging in age from 36 to 54 years. Incidence of leptomeningeal dissemination in patients with HB was about 4.3 % (3/69). It appeared at a mean 94.9 months (range, 39-204 months) after gross total resection of CNS HBs. Three of the seven patients died 5, 38, and 79 months, respectively, after diagnosis of leptomeningeal dissemination. Review of the literature identified 21 patients with characteristics of leptomeningeal dissemination similar to those in our series.
  
  
  
  Leptomeningeal dissemination of HB is a rare pattern of long-term recurrence. Long-term outcomes may be fatal. The long developmental period suggests that early detection and aggressive management may improve prognosis in patients with CNS leptomeningeal dissemination of HB.
authors.affiliation: Department of Radiology, Asan Medical Center, Ulsan University School of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 138-736, South Korea.|Department of Neurosurgery, Ilsan Paik Hospital, College of Medicine, Inje University, Goyang, South Korea.
authors.forename: Hae-Won
authors.initials: HW
authors.lastname: Koo
authors.affiliation: Department of Radiology, Asan Medical Center, Ulsan University School of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 138-736, South Korea.
authors.forename: Ji Eun
authors.initials: JE
authors.lastname: Park
authors.affiliation: Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.
authors.forename: Jihoon
authors.initials: J
authors.lastname: Cha
authors.affiliation: Department of Radiology, Severance Hospital, Yonsei University School of Medicine, Seoul, South Korea.
authors.forename: Dong Joon
authors.initials: DJ
authors.lastname: Kim
authors.affiliation: Department of Pathology, Asan Medical Center, Ulsan University School of Medicine, Seoul, South Korea.
authors.forename: S G
authors.initials: SG
authors.lastname: Kang
authors.affiliation: Department of Neurosurgery, Asan Medical Center, Ulsan University School of Medicine, Seoul, South Korea.
authors.forename: S C
authors.initials: SC
authors.lastname: Lim
authors.affiliation: Department of Radiology, Asan Medical Center, Ulsan University School of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul, 138-736, South Korea. dcsuh@amc.seoul.kr.
authors.forename: Dae Chul
authors.initials: DC
authors.lastname: Suh
mesh_terms: D000328:Adult; D005260:Female; D018325:Hemangioblastoma / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008297:Male; D008577:Meningeal Neoplasms / Q000556:secondary*; D008875:Middle Aged; D006623:von Hippel-Lindau Disease / Q000473:pathology* / Q000601:surgery
keywords: Hemangioblastoma; Hemangioblastomatosis; Leptomeningeal seeding; Spine metastasis; von Hippel–Lindau