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

by Ho, An Thi Nhat.
Citation: ; European Journal of Endocrinology. 183(4):453-462, 2020 Oct..Journal: European journal of endocrinology.Published: ; 2020; ; ISSN: 0804-4643.Full author list: Antke C; Arlt W; Beuschlein F; Casanovas O; Chortis V; Fassnacht M; Gattermann N; Germing U; Gimm O; Haas R; Haase M; Habra M; Ho ATN; Kanji A; Kondakci M; Laurent C; Limvorapitak W; Majidi F; Martino S; Petit JM; Ronchi CL; Rudelius M; Salvatori R; Schott M; Spyroglou A; Villa D; Wahlin BE.UI/PMID: 32567556.Subject(s): Adrenal Gland Neoplasms/co [Complications] | United States/ep [Epidemiology] | Tomography, X-Ray Computed | Risk Factors | Retrospective Studies | Positron-Emission Tomography | Phenotype | Multimodal Imaging | Middle Aged | Male | *Lymphoma/ep [Epidemiology] | *Lymphoma/di [Diagnosis] | Lymphoma/co [Complications] | Humans | Fluorodeoxyglucose F18/pk [Pharmacokinetics] | Female | Europe/ep [Epidemiology] | Diagnosis, Differential | Cohort Studies | Canada/ep [Epidemiology] | Aged, 80 and over | Aged | Adult | Adrenal Insufficiency/et [Etiology] | Adrenal Insufficiency/ep [Epidemiology] | Adrenal Insufficiency/di [Diagnosis] | *Adrenal Gland Neoplasms/ep [Epidemiology] | *Adrenal Gland Neoplasms/di [Diagnosis]Institution(s): MedStar Union Memorial HospitalDepartment(s): MedicineActivity type: Journal Article.Medline article type(s): Journal ArticleDigital Object Identifier: https://dx.doi.org/10.1530/EJE-19-0506 (Click here) Abbreviated citation: ; EUR. J. ENDOCRINOL.. 183(4):453-462, 2020 Oct.Abstract: 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.Abstract: METHODS: 97 patients from 14 centers in Europe, Canada and the United States were included in this retrospective analysis between 1994 and 2017.Abstract: 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.Abstract: 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).

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