title: Prognosis of orbital lymphoid hyperplasia.
abstract: BACKGROUND
  Orbital lymphoid hyperplasia can be associated with systemic non-Hodgkin lymphoma (NHL), even when polyclonal proliferation is found in the orbit. Although irradiation is recommended, some orbital lymphoid hyperplasias are treated by steroids (when inflammation is clinically presumed) or left untreated.
  
  
  METHODS
  The incidence of concurrent NHL and the incidence of future NHL after oral prednisone, radiotherapy, or no treatment were retrospectively evaluated in 33 cases of lymphoid hyperplasia (22 benign lymphoid hyperplasias, BLH, and 11 atypical lymphoid hyperplasias, ALH), after follow-up of 2-13 years.
  
  
  RESULTS
  NHL occurred in 12 of 33 cases (36.4%). In seven patients it was concurrent; in five patients it occurred 2-6 years later. In the actuarial curve, at 5 years 55% of patients were free of lymphoma, at 10 years, 46%. NHL was more commonly observed when the lacrimal gland was involved (57% vs 21%; P = 0.03). Of the 13 patients treated with oral steroids, 46% had complete response, 39% partial response, and 15% future NHL. Of the seven irradiated patients, five (71%) had complete response, two (29%) partial response, and none future NHL. Of the eight untreated patients, five (63%) had partial response and three (37%) future NHL. Irradiated lacrimal gland BLHs only achieved partial response, one having radiation-induced orbital inflammation.
  
  
  CONCLUSIONS
  Because of a high risk of NHL, in all orbital lymphoid tumors systemic staging and follow-up are mandatory. The advised management is irradiation, except for Sjögren syndrome, an initially inflammatory lacrimal gland BLH, where a course of steroid is suggested before considering radiotherapy.
authors.affiliation: Department of Ophthalmology and Neurosurgery, University of Siena, Italy.
authors.forename: E
authors.initials: E
authors.lastname: Polito
authors.forename: A
authors.initials: A
authors.lastname: Leccisotti
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D005500:Follow-Up Studies; D006801:Humans; D006965:Hyperplasia; D015994:Incidence; D007766:Lacrimal Apparatus Diseases / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D008221:Lymphoid Tissue / Q000473:pathology*; D008228:Lymphoma, Non-Hodgkin / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D009367:Neoplasm Staging; D009916:Orbital Diseases / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D009918:Orbital Neoplasms / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D011241:Prednisone / Q000627:therapeutic use; D011379:Prognosis; D012189:Retrospective Studies
chemical_list: D000893:Anti-Inflammatory Agents; D011241:Prednisone