title: A case series of primary central nervous system posttransplantation lymphoproliferative disorder: imaging and clinical characteristics.
abstract: BACKGROUND
  Primary central nervous system posttransplantation lymphoproliferative disorder (PCNS-PTLD) is a rare complication after solid organ transplantation (SOT). With increasing rates of SOT, PCNS-PTLD incidence is increasing.
  
  
  OBJECTIVE
  To describe the characteristics of PCNS-PTLD patients requiring neurosurgical intervention.
  
  
  METHODS
  From 2000 to 2011, 10 patients with prior SOT underwent biopsy for evaluation of brain lesions and were diagnosed with PCNS-PTLD. Data collected included imaging characteristics, pathology, treatments administered, and survival outcomes.
  
  
  RESULTS
  All patients had kidney transplantation, and 3 had concurrent pancreas transplantation. Median age at diagnosis was 49 years, with a median of 4.5 years from SOT to diagnosis (range, 1.8-11.4 years). Presenting symptoms most often included focal neurological deficits (n = 6), although several patients had nonspecific symptoms of headache and altered mental status. Brain lesions were generally multiple (n = 7), supratentorial (n = 8), and lobar or periventricular in distribution with ring enhancement. Diagnosis was established by stereotactic (n = 4) and open surgical (n = 6) biopsy. Treatments most frequently administered included reduction of immunosuppression (n = 10), dexamethasone (n = 10), rituximab (n = 8), high-dose methotrexate (n = 3), and whole-brain radiotherapy (n = 6). Six patients remain alive without PCNS-PTLD relapse, including 4 patients who have sustained remissions beyond 2 years from diagnosis of PCNS-PTLD. Of 4 observed deaths, 1 was related to progressive PCNS-PTLD.
  
  
  CONCLUSIONS
  PCNS-PTLD must be considered in the differential diagnosis of any patient with prior SOT presenting with an intracranial lesion. Histological diagnosis with brain biopsy is imperative, given the risk for opportunistic infections that may have similar imaging findings and presentation. Prognosis is variable, although long-term survival has been reported.
authors.affiliation: Department of Neurological Surgery, School of Medicine and Public Health, University of Wisconsin, Madison, Wisconsin 53792, USA.
authors.forename: Wendell
authors.initials: W
authors.lastname: Lake
authors.forename: Julie E
authors.initials: JE
authors.lastname: Chang
authors.forename: Tabassum
authors.initials: T
authors.lastname: Kennedy
authors.forename: Adam
authors.initials: A
authors.lastname: Morgan
authors.forename: Shahriar
authors.initials: S
authors.lastname: Salamat
authors.forename: Mustafa K
authors.initials: MK
authors.lastname: Başkaya
mesh_terms: D000328:Adult; D000368:Aged; D002493:Central Nervous System Diseases / Q000175:diagnosis / Q000209:etiology / Q000276:immunology*; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000276:immunology; D005260:Female; D006801:Humans; D016867:Immunocompromised Host; D016030:Kidney Transplantation / Q000009:adverse effects*; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000209:etiology / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D016035:Pancreas Transplantation / Q000009:adverse effects; D011183:Postoperative Complications / Q000453:epidemiology* / Q000209:etiology