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Clinical spectrum o...
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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 ...
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BIOSCIENTIFICA LTD,2020
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printrdacarrier
Numbers
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LIBRIS-ID:oai:DiVA.org:liu-169960
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https://urn.kb.se/resolve?urn=urn:nbn:se:liu:diva-169960URI
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https://doi.org/10.1530/EJE-19-0506DOI
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http://kipublications.ki.se/Default.aspx?queryparsed=id:144543079URI
Supplementary language notes
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Language:English
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Summary in:English
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Classification
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Subject category:ref swepub-contenttype
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Subject category:art swepub-publicationtype
Notes
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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]
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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.
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Martino, SamuelaHeinrich Heine Univ Dusseldorf, Germany
(author)
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Kondakci, MustafaHeinrich Heine Univ Dusseldorf, Germany
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Antke, ChristinaHeinrich Heine Univ Dusseldorf, Germany
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Haase, MatthiasHeinrich Heine Univ Dusseldorf, Germany
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Chortis, VasileiosBirmingham Hlth Partners, England; Univ Birmingham, England
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Arlt, WiebkeBirmingham Hlth Partners, England; Univ Birmingham, England
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Ronchi, Cristina L.Univ Birmingham, England; Univ Wurzburg, Germany
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Fassnacht, MartinUniv Wurzburg, Germany
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Laurent, ClaireUniv Hosp Dijon, France
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Petit, Jean-MichelUniv Hosp Dijon, France
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Casasnovas, OlivierUniv Hosp Djion, France
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Habra, Amir MouhammedUniv Texas MD Anderson Canc Ctr, TX 77030 USA
(author)
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Kanji, AleemBaylor Coll Med, TX 77030 USA
(author)
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Salvatori, RobertoJohns Hopkins Univ, MD USA
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Nhat Ho, An ThiMedstar Harbor Hosp, MD USA
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Spyroglou, AriadniUniv Spital Zurich, Switzerland
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Beuschlein, FelixUniv Spital Zurich, Switzerland; Ludweig Maximilians Univ Munchen, Germany
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Villa, DiegoBC Canc Ctr Lymphoid Canc, Canada; Univ British Columbia, Canada
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Limvorapitak, WasithepBC Canc Ctr Lymphoid Canc, Canada; Univ British Columbia, Canada; Thammasat Univ, Thailand
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Wahlin, Bjoern EngelbrektKarolinska Institutet
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Gimm, OliverLinköpings universitet,Avdelningen för kirurgi, ortopedi och onkologi,Medicinska fakulteten,Region Östergötland, Kirurgiska kliniken US(Swepub:liu)oligi51
(author)
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Rudelius, MartinaLudweig Maximilians Univ Munchen, Germany
(author)
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Schott, MatthiasHeinrich Heine Univ Dusseldorf, Germany
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Germing, UlrichHeinrich Heine Univ Dusseldorf, Germany
(author)
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Haas, RainerHeinrich Heine Univ Dusseldorf, Germany
(author)
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Gattermann, NorbertHeinrich Heine Univ Dusseldorf, Germany
(author)
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Heinrich Heine Univ Dusseldorf, GermanyBirmingham Hlth Partners, England; Univ Birmingham, England
(creator_code:org_t)
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In:European Journal of Endocrinology: BIOSCIENTIFICA LTD183:4, s. 453-4620804-46431479-683X
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Majidi, Fatemeh
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Martino, Samuela
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Kondakci, Mustaf ...
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Antke, Christina
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Haase, Matthias
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Chortis, Vasilei ...
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Arlt, Wiebke
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Ronchi, Cristina ...
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Fassnacht, Marti ...
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Laurent, Claire
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Petit, Jean-Mich ...
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Casasnovas, Oliv ...
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Habra, Amir Mouh ...
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Kanji, Aleem
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Salvatori, Rober ...
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Nhat Ho, An Thi
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Spyroglou, Ariad ...
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Beuschlein, Feli ...
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Villa, Diego
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Limvorapitak, Wa ...
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Wahlin, Bjoern E ...
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Gimm, Oliver
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Rudelius, Martin ...
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Schott, Matthias
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Germing, Ulrich
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Haas, Rainer
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Gattermann, Norb ...
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