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: fatemeh.majidi@med.uni-duesseldorf.de
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Purpose:

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.

Methods:

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

Results:

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).

Conclusion:

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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