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

MedStar author(s):
Citation: European Journal of Endocrinology. 183(4):453-462, 2020 Oct.PMID: 32567556Institution: MedStar Union Memorial HospitalDepartment: MedicineForm of publication: Journal ArticleMedline article type(s): Journal ArticleSubject headings: *Adrenal Gland Neoplasms/di [Diagnosis] | *Adrenal Gland Neoplasms/ep [Epidemiology] | *Lymphoma/di [Diagnosis] | *Lymphoma/ep [Epidemiology] | Adrenal Gland Neoplasms/co [Complications] | Adrenal Insufficiency/di [Diagnosis] | Adrenal Insufficiency/ep [Epidemiology] | Adrenal Insufficiency/et [Etiology] | Adult | Aged | Aged, 80 and over | Canada/ep [Epidemiology] | Cohort Studies | Diagnosis, Differential | Europe/ep [Epidemiology] | Female | Fluorodeoxyglucose F18/pk [Pharmacokinetics] | Humans | Lymphoma/co [Complications] | Male | Middle Aged | Multimodal Imaging | Phenotype | Positron-Emission Tomography | Retrospective Studies | Risk Factors | Tomography, X-Ray Computed | United States/ep [Epidemiology]Year: 2020ISSN:
  • 0804-4643
Name of journal: European journal of endocrinologyAbstract: 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.METHODS: 97 patients from 14 centers in Europe, Canada and the United States were included in this retrospective analysis between 1994 and 2017.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.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).All authors: 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 BEOriginally published: European Journal of Endocrinology. 2020 Jun 01Fiscal year: FY2021Fiscal year of original publication: FY2020Digital Object Identifier: Date added to catalog: 2020-08-26
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Journal Article MedStar Authors Catalog Article 32567556 Available 32567556

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.

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

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.

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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