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

Research output: Contribution to journalArticlepeer-review


  • Fatemeh Majidi
  • Samuela Martino
  • Mustafa Kondakci
  • Christina Antke
  • Matthias Haase
  • Martin Fassnacht
  • Claire Laurent
  • Jean-Michel Petit
  • Olivier Casasnovas
  • Mouhammed Habra
  • Aleem Kanji
  • Roberto Salvatori
  • An Thi Nhat Ho
  • Adriadni Spyroglou
  • Felix Beuschlein
  • Diego Villa
  • Wasithep Limvorapitak
  • Bjorn Engelbrekt Wahlin
  • Oliver Gimm
  • Martina Rudelius
  • Matthias Schott
  • Ulrich Germing
  • Rainer Haas
  • Norbert Gattermann

Colleges, School and Institutes

External organisations

  • Ludwig Maximillian University of Munich (LMU)


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

Results: Of 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 versus 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.


Original languageEnglish
Pages (from-to)453–462
JournalEuropean Journal of Endocrinology
Issue number4
Early online date30 Jun 2020
Publication statusPublished - Oct 2020