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

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TitreClinical spectrum of primary adrenal lymphoma: results of a multicenter cohort study
Type de publicationJournal Article
Year of Publication2020
AuteursMajidi F, Martino S, Kondakci M, Antke C, Haase M, Chortis V, Arlt W, Ronchi CL, Fassnacht M, Laurent C, Petit J-M, Casasnovas O, Habra AMouhammed, Kanji A, Salvatori R, Ho AThi Nhat, Spyroglou A, Beuschlein F, Villa D, Limvorapitak W, Wahlin BEngelbrekt, Gimm O, Rudelius M, Schott M, Germing U, Haas R, Gattermann N
JournalEUROPEAN JOURNAL OF ENDOCRINOLOGY
Volume183
Pagination453-462
Date PublishedOCT
Type of ArticleArticle
ISSN0804-4643
Résumé

{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

DOI10.1530/EJE-19-0506