title: Long-term outcome of primary endocrine non-Hodgkin lymphomas: does the site make the difference?
abstract: OBJECTIVE
  Primary lymphomas of endocrine glands are extremely rare. Our study adds more data to the few published series regarding the incidence, clinical characteristics, management and overall survival (OS) by comparing the various diffuse large B-cell endocrine lymphomas. Moreover, it contributes to a better understanding of these neoplasms and provides concepts for future research.
  
  
  METHODS
  We retrospectively evaluated the clinical profile and the patterns of outcome among patients who were treated in our center with the diagnosis of aggressive, B-cell, primary endocrine lymphoma.
  
  
  RESULTS
  Between May 1980 and December 2011, 450 patients were diagnosed as primary extranodal non-Hodgkin lymphomas. Among them, 18 cases (4%) were primary testicular lymphoma (PTL), 8 cases (2%) were primary thyroid lymphoma (PTHL) and 4 cases (1%) were primary adrenal lymphoma (PAL). The therapeutic approaches employed were variable, including mainly chemotherapy in combination with radiotherapy and surgery. The median OS for the patients with PTL and PAL was 27 and 6 months, respectively. Better outcome was observed in patients with PTHL for whom the median OS has not been reached yet, whereas the PAL group had the worst prognosis.
  
  
  CONCLUSIONS
  The discrepancies in the outcome among endocrine lymphomas could be partly attributed to their biologic variability, which might be determined by the initial site involved. We conclude that treatment decisions should be made according to a multi-disciplinary approach to avoid unnecessary surgery. Existing treatment strategies for PTL and PAL fail to provide long-term survival, rendering the application of novel therapeutic approaches essential.
authors: Department of Hematology-Oncology, Theagenio Cancer Hospital, 2 Al. Simeonidis Street, Thessaloniki 54007, Greece. ehatjiharissi@gmail.com
authors: E
authors: E
authors: Hatjiharissi
authors: M D
authors: MD
authors: Diamantidis
authors: M
authors: M
authors: Papaioannou
authors: T
authors: T
authors: Dimou
authors: A
authors: A
authors: Chrisoulidou
authors: F
authors: F
authors: Patakiouta
authors: N
authors: N
authors: Constantinou
authors: K
authors: K
authors: Pazaitou-Panayiotou
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000556:secondary; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D004701:Endocrine Gland Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D005260:Female; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D013736:Testicular Neoplasms / Q000556:secondary; D013964:Thyroid Neoplasms / Q000556:secondary; D016896:Treatment Outcome; D055815:Young Adult