title: Lymphomatoid Granulomatosis: A Single Institution Experience and Review of the Literature.
abstract: Lymphomatoid granulomatosis (LYG) is a rare B-cell lymphoproliferative disorder with frequent extranodal presentation and involvement of the respiratory system. The purpose of this study is to describe the clinical characteristics, pathologic findings, and treatment outcomes of LYG in a single tertiary institution.
  
  
  
  This is a retrospective review of series of cases of LYG diagnosed at Moffitt Cancer Center (MCC) between 2000 and 2011. We describe clinical presentation, histopathologic findings, and treatment outcomes.
  
  
  
  We identified 11 cases of biopsy-proven LYG at our institution. All patients presented with lung involvement by LYG. Nine patients were treated with rituximab-based chemotherapy. The overall response rate was 63.6% (complete response rate, 36.44%). Extra-pulmonary involvement was common (central nervous system, kidney, adrenal glands, testicles, and liver). The median overall survival and progression-free survival were 23 and 12.2 months, respectively.
  
  
  
  LYG is a rare B-cell lymphoproliferative disorder with involvement if the respiratory system. The presentation is heterogeneous, and response to therapy is variable. Although it is considered a poor prognosis disease, long-term survivors in remission have been described.
authors: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research, Tampa, FL. Electronic address: Julio.c.chavez@moffitt.org.
authors: Julio C
authors: JC
authors: Chavez
authors: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research, Tampa, FL.
authors: Jose
authors: J
authors: Sandoval-Sus
authors: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research, Tampa, FL.
authors: Pedro
authors: P
authors: Horna
authors: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research, Tampa, FL.
authors: Samir
authors: S
authors: Dalia
authors: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research, Tampa, FL.
authors: Celeste
authors: C
authors: Bello
authors: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research, Tampa, FL.
authors: Paul
authors: P
authors: Chevernick
authors: Department of Hematology/Oncology, George Washington University, Washington, DC.
authors: Eduardo M
authors: EM
authors: Sotomayor
authors: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research, Tampa, FL.
authors: Lubomir
authors: L
authors: Sokol
authors: Department of Malignant Hematology, H. Lee Moffitt Cancer Center and Research, Tampa, FL.
authors: Bijal
authors: B
authors: Shah
mesh_terms: D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use; D001706:Biopsy; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D008230:Lymphomatoid Granulomatosis / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D060787:Neoplasm Grading; D009362:Neoplasm Metastasis; D009367:Neoplasm Staging; D012189:Retrospective Studies; D000069283:Rituximab / Q000008:administration & dosage / Q000627:therapeutic use; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D000069283:Rituximab
keywords: B-cell; EBV; Extranodal; Lymphomatoid granulomatosis; Rituximab