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  • Majidi, FatemehHeinrich Heine Univ Dusseldorf, Germany (author)

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

  • Article/chapterEnglish2020

Publisher, publication year, extent ...

  • BIOSCIENTIFICA LTD,2020
  • printrdacarrier

Numbers

  • LIBRIS-ID:oai:DiVA.org:liu-169960
  • https://urn.kb.se/resolve?urn=urn:nbn:se:liu:diva-169960URI
  • https://doi.org/10.1530/EJE-19-0506DOI
  • http://kipublications.ki.se/Default.aspx?queryparsed=id:144543079URI

Supplementary language notes

  • Language:English
  • Summary in:English

Part of subdatabase

Classification

  • Subject category:ref swepub-contenttype
  • Subject category:art swepub-publicationtype

Notes

  • Funding Agencies|National Institute for Health Research (NIHR) Birmingham Biomedical Research Centre at the University Hospitals Birmingham NHS Foundation Trust; University of Birmingham [BRC-1215-20009]; Academy of Medical Sciences UK [SGL020/1018]
  • 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.

Subject headings and genre

Added entries (persons, corporate bodies, meetings, titles ...)

  • Martino, SamuelaHeinrich Heine Univ Dusseldorf, Germany (author)
  • Kondakci, MustafaHeinrich Heine Univ Dusseldorf, Germany (author)
  • Antke, ChristinaHeinrich Heine Univ Dusseldorf, Germany (author)
  • Haase, MatthiasHeinrich Heine Univ Dusseldorf, Germany (author)
  • Chortis, VasileiosBirmingham Hlth Partners, England; Univ Birmingham, England (author)
  • Arlt, WiebkeBirmingham Hlth Partners, England; Univ Birmingham, England (author)
  • Ronchi, Cristina L.Univ Birmingham, England; Univ Wurzburg, Germany (author)
  • Fassnacht, MartinUniv Wurzburg, Germany (author)
  • Laurent, ClaireUniv Hosp Dijon, France (author)
  • Petit, Jean-MichelUniv Hosp Dijon, France (author)
  • Casasnovas, OlivierUniv Hosp Djion, France (author)
  • Habra, Amir MouhammedUniv Texas MD Anderson Canc Ctr, TX 77030 USA (author)
  • Kanji, AleemBaylor Coll Med, TX 77030 USA (author)
  • Salvatori, RobertoJohns Hopkins Univ, MD USA (author)
  • Nhat Ho, An ThiMedstar Harbor Hosp, MD USA (author)
  • Spyroglou, AriadniUniv Spital Zurich, Switzerland (author)
  • Beuschlein, FelixUniv Spital Zurich, Switzerland; Ludweig Maximilians Univ Munchen, Germany (author)
  • Villa, DiegoBC Canc Ctr Lymphoid Canc, Canada; Univ British Columbia, Canada (author)
  • Limvorapitak, WasithepBC Canc Ctr Lymphoid Canc, Canada; Univ British Columbia, Canada; Thammasat Univ, Thailand (author)
  • Wahlin, Bjoern EngelbrektKarolinska Institutet (author)
  • Gimm, OliverLinköpings universitet,Avdelningen för kirurgi, ortopedi och onkologi,Medicinska fakulteten,Region Östergötland, Kirurgiska kliniken US(Swepub:liu)oligi51 (author)
  • Rudelius, MartinaLudweig Maximilians Univ Munchen, Germany (author)
  • Schott, MatthiasHeinrich Heine Univ Dusseldorf, Germany (author)
  • Germing, UlrichHeinrich Heine Univ Dusseldorf, Germany (author)
  • Haas, RainerHeinrich Heine Univ Dusseldorf, Germany (author)
  • Gattermann, NorbertHeinrich Heine Univ Dusseldorf, Germany (author)
  • Heinrich Heine Univ Dusseldorf, GermanyBirmingham Hlth Partners, England; Univ Birmingham, England (creator_code:org_t)

Related titles

  • In:European Journal of Endocrinology: BIOSCIENTIFICA LTD183:4, s. 453-4620804-46431479-683X

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