Ho, An Thi Nhat

Clinical spectrum of primary adrenal lymphoma: results of a multicenter cohort study. - 2020

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).


English

0804-4643

10.1530/EJE-19-0506 [doi] EJE-19-0506.R4 [pii]


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]


MedStar Union Memorial Hospital


Medicine


Journal Article

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