title: Adrenal lymphoma: presentation, management and prognosis.
abstract: OBJECTIVE
  This study aimed to identify the clinical, radiological and prognostic features of primary adrenal lymphoma (PAL) in order to diagnose the disease more accurately.
  
  
  METHODS
  A retrospective multi-centre study was conducted on the clinical, biological and radiological features as well as the treatment and overall survival outcomes in PAL.
  
  
  RESULTS
  Between 1994 and 2014, 28 patients from five regions of eastern France were diagnosed with primary adrenal lymphoma. The revealing symptoms were a worsening general state (77%), weight loss (77%) and abdominal pain (42%). Biological features of PAL were almost omnipresent: increased LDH, β2 microglobulin, CRP or ferritinaemia levels. The PAL was bilateral in 20 cases (71%), adrenal insufficiency was searched for in 11 patients and found in eight (73%). CT scans showed masses of various sizes measuring up to 180 mm. On MRI, the lesions were hypointense in T1 and hyperintense in T2. When done, positron emission tomography with fluorodeoxyglucose (FDG-PET) showed locations not seen on the CT and revealed extra-adrenal locations in 70% of examinations. Adrenalectomy brought no benefit. The overall survival rate was poor (61.9% at 2 years) despite polychemotherapy.
  
  
  CONCLUSIONS
  The clinical presentation of PAL comprised major general symptoms. Adrenal insufficiency was very common in patients with bilateral involvement but was not systematically tested. PET was an efficient examination to visualize extra-adrenal locations. The preliminary results of MRI to distinguish between PAL and adrenocortical carcinoma should be confirmed. Further studies are needed to establish an optimal strategy for the management of these primary adrenal lymphomas.
authors: From the Department of Endocrinology, University Hospital of Dijon, Dijon, France.
authors: C
authors: C
authors: Laurent
authors: Department of Hematology, University Hospital of Dijon, Dijon, France.
authors: O
authors: O
authors: Casasnovas
authors: Department of Pathology, University Hospital of Dijon, Dijon, France.
authors: L
authors: L
authors: Martin
authors: Department of Hematology, University Hospital of Besancon, Besancon, France.
authors: A
authors: A
authors: Chauchet
authors: Department of Hematology, Centre Leon Berard, University Hospital of Lyon, Lyon, France.
authors: H
authors: H
authors: Ghesquieres
authors: Department of Hematology, Centre Leon Berard, University Hospital of Lyon, Lyon, France.
authors: G
authors: G
authors: Aussedat
authors: Department of Hematology, University Hospital of Strasbourg, Strasbourg, France.
authors: L M
authors: LM
authors: Fornecker
authors: Department of Hematology, University Hospital of Nancy, Nancy, France.
authors: S
authors: S
authors: Bologna
authors: Department of Endocrinology, University Hospital of Besancon, Besancon, France.
authors: S
authors: S
authors: Borot
authors: University of Science and Technology of Nancy, Nancy, France.
authors: K
authors: K
authors: Laurent
authors: From the Department of Endocrinology, University Hospital of Dijon, Dijon, France.
authors: B
authors: B
authors: Bouillet
authors: From the Department of Endocrinology, University Hospital of Dijon, Dijon, France.
authors: B
authors: B
authors: Verges
authors: From the Department of Endocrinology, University Hospital of Dijon, Dijon, France.
authors: J-M
authors: JM
authors: Petit
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D000309:Adrenal Insufficiency / Q000453:epidemiology / Q000209:etiology; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000453:epidemiology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D049268:Positron-Emission Tomography; D011379:Prognosis; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed