title: Clinicopathological analysis of primary adrenal diffuse large B-cell lymphoma: effectiveness of rituximab-containing chemotherapy including central nervous system prophylaxis.
abstract: BACKGROUND
  Primary adrenal lymphoma (PAL) is an extremely rare subtype of extranodal non-Hodgkin's lymphoma. Some researchers have reported some of the characteristics of PAL and its association with poor prognosis; however, the clinicopathological features of PAL remain to be elucidated.
  
  
  METHODS
  From 2008 to 2011 we experienced seven cases of PAL in our institutions. We retrospectively analyzed the clinical and pathological features of these patients.
  
  
  RESULTS
  The patients ranged in age from 50 to 85 years, with a median of 71 years. The overall male:female ratio was 6:1. All seven patients were diagnosed with diffuse large B-cell lymphoma (DLBCL) pathologically. Bilateral adrenal involvement was confirmed in five patients. The median largest tumor diameter at diagnosis was 58 mm. The Ki-67 index was generally high (>70%). All patients were treated with rituximab-containing chemotherapy, and central nervous system (CNS) prophylaxis was conducted for three patients. One patient with CNS involvement at the time of the diagnosis also received whole-brain radiation. The overall survival rate at two years was 57% (median follow-up; 24.8 months). It is noteworthy that the three patients who received a full course of the rituximab-containing regimen and CNS prophylaxis are currently alive without disease relapse, and that none of the seven patients died due to progression of lymphoma.
  
  
  CONCLUSIONS
  Primary adrenal DLBCL can be a clinically aggressive disease entity. Rituximab-containing chemotherapy combined with CNS prophylaxis could be a reasonable option for the treatment of PAL; however, analyses of more PAL cases are needed for the establishment of this strategy.
authors.affiliation: Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-cho, Sendai 980-8574, Japan. nfukuhara@med.tohoku.ac.jp.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Ichikawa
authors.forename: Noriko
authors.initials: N
authors.lastname: Fukuhara
authors.forename: Ai
authors.initials: A
authors.lastname: Inoue
authors.forename: Hiroki
authors.initials: H
authors.lastname: Katsushima
authors.forename: Rie
authors.initials: R
authors.lastname: Ohba
authors.forename: Yuna
authors.initials: Y
authors.lastname: Katsuoka
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Onishi
authors.forename: Joji
authors.initials: J
authors.lastname: Yamamoto
authors.forename: Osamu
authors.initials: O
authors.lastname: Sasaki
authors.forename: Jun
authors.initials: J
authors.lastname: Nomura
authors.forename: Osamu
authors.initials: O
authors.lastname: Fukuhara
authors.forename: Kenichi
authors.initials: K
authors.lastname: Ishizawa
authors.forename: Ryo
authors.initials: R
authors.lastname: Ichinohasama
authors.forename: Hideo
authors.initials: H
authors.lastname: Harigae
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