Clinical spectrum of primary adrenal lymphoma: results of a multicenter cohort study

in European Journal of Endocrinology
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  • 1 Department of Hematology, Oncology and Clinical Immunology, Heinrich Heine University Düsseldorf, Düsseldorf, Germany
  • 2 Department of Endocrinology, Heinrich Heine University Düsseldorf, Düsseldorf, Germany
  • 3 Center for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
  • 4 Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
  • 5 Divison of Endocrinology and Diabetes, Department of Internal Medicine, University Hospital, University of Würzburg, Würzburg, Germany
  • 6 Comprehensive Cancer Center Mainfranken, University of Würzburg, Würzburg, Germany
  • 7 University Hospital of Dijon, Dijon, France
  • 8 Department of Hematology, University Hospital of Djion, Dijon, France
  • 9 Department of Endocrine Neoplasia and Hormonal Disorders, MD Anderson Cancer Center, Houston, Texas, USA
  • 10 Baylor College of Medicine, Houston, Texas, USA
  • 11 Division of Endocrinology, Diabetes and Metabolism, Johns Hopkins University, Baltimore, Maryland, USA
  • 12 Klinik für Endokrinologie und Klinische Ernährung, Universitätsspital Zürich, Zürich, Switzerland
  • 13 Klinik und Poliklinik IV, Klinikum der Universität München, Ludweig-Maximilians-Universität München, Munich, Germany
  • 14 BC Cancer Centre for Lymphoid Cancer and University of British Columbia, Vancouver, Canada
  • 15 Division of Hematology, Department of Internal Medicine, Thammasat University, Pathumthani, Thailand
  • 16 Department of Medicine, Unit of Hematology, Karolinska Institute, Stockholm, Sweden
  • 17 Departments of Surgery, and Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden
  • 18 Institute of Pathology, Ludweig-Maximilians-Universität München, München, Germany
  • 19 Klinik für Nuklearmedizin, Heinrich Heine Universität Düsseldorf, Düsseldorf, Germany
  • 20 Department of Medicine, Medstar Harbor Hospital, Baltimore, Maryland, USA

Correspondence should be addressed to F Majidi; Email:
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We sought to refine the clinical picture of primary adrenal lymphoma (PAL), a rare lymphoid malignancy with predominant adrenal manifestation and risk of adrenal insufficiency.


Ninety-seven patients from 14 centers in Europe, Canada and the United States were included in this retrospective analysis between 1994 and 2017.


Of the 81 patients with imaging data, 19 (23%) had isolated adrenal involvement (iPAL), while 62 (77%) had additional extra-adrenal involvement (PAL+). Among patients who had both CT and PET scans, 18FDG-PET revealed extra-adrenal involvement not detected by CT scan in 9/18 cases (50%). The most common clinical manifestations were B symptoms (55%), fatigue (45%), and abdominal pain (35%). Endocrinological assessment was often inadequate. With a median follow-up of 41.6 months, 3-year progression-free (PFS) and overall (OS) survival rates in the entire cohort were 35.5% and 39.4%, respectively. The hazard ratios of iPAL for PFS and OS were 40.1 (95% CI: 2.63–613.7, P = 0.008) and 2.69 (95% CI: 0.61–11.89, P = 0.191), respectively. PFS was much shorter in iPAL vs PAL+ (median 4 months vs not reached, P = 0.006), and OS also appeared to be shorter (median 16 months vs not reached), but the difference did not reach statistical significance (P = 0.16). Isolated PAL was more frequent in females (OR = 3.81; P = 0.01) and less frequently associated with B symptoms (OR = 0.159; P = 0.004).


We found unexpected heterogeneity in the clinical spectrum of PAL. Further studies are needed to clarify whether clinical distinction between iPAL and PAL+ is corroborated by differences in molecular biology.


     European Society of Endocrinology

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