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Adrenal gland & paraganglia

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Lymphoma

Author: Lan L. Gellert, M.D., Ph.D.

Last author update: 1 June 2016

Last staff update: 12 November 2020

Copyright: 2002-2022, PathologyOutlines.com, Inc.

PubMed Search: Lymphoma [title] adrenal

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Table of Contents

Cite this page: Gellert LL. Lymphoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/adrenallymphoma.html. Accessed August 11th, 2022.

Definition / general

Rare, < 200 cases reported
Usually presents with bilateral adrenal masses, often with adrenal hypofunction
Subtype usually diffuse large B cell lymphoma

Adrenal involvement in up to 25% with disseminated lymphoma at autopsy
Usually no associated adrenal insufficiency

Essential features

Primary adrenal lymphoma is rare
- Most cases have bilateral adrenal involvement and adrenal insufficiency
- Diffuse large B cell lymphoma most common

Epidemiology

Mean age at diagnosis is 62 years (Ann Hematol 2013;92:1583)
Male to female ratio: 1.8:1 (Ann Hematol 2013;92:1583)

Clinical features

Most present with bilateral adrenal involvement, often with adrenal insufficiency
90% of cases are high grade B cell subtypes (Ann Hematol 2013;92:1583)
Most common subtypes: diffuse large B cell lymphoma (78%) and peripheral T cell lymphoma (7%, Ann Hematol 2013;92:1583)
EBV positivity in > 50% (Ann Hematol 2013;92:1583)

Radiology images

Images hosted on other servers:

Bilateral, homogenous adrenal gland enlargement

Fluorodeoxyglucose PET

Prognostic factors

Poor prognosis; most die within one year
Adrenal insufficiency and administration of chemotherapy are significant prognostic factors (Ann Hematol 2013;92:1583)
CNS involvement adversely affects long term prognosis (Ann Hematol 2013;92:1583)

Case reports

36 year old man with primary adrenal nodular lymphocyte predominant Hodgkin lymphoma (Oncol Lett 2014;8:1147)
56 year old man with bilateral adrenal diffuse large B cell lymphoma (Turk J Haematol 2014;31:188)
57 year old man with diffuse B cell lymphoma and pheochromocytoma in same adrenal gland (World J Surg Oncol 2015;13:289)
57 year old man with unilateral adrenal involvement by diffuse large B cell lymphoma (J Clin Diagn Res 2015;9:XD01)
62 year old woman with bilateral large adrenal masses (Case Rep Med 2015;2015:620381)
64 year old woman with primary diffuse large B cell lymphoma (Arch Pathol Lab Med 1990;114:883)
70 year old man with primary adrenal lymphoma associated with Epstein Barr virus reactivation due to immunosuppression (Medicine (Baltimore) 2015;94:e1270)
74 year old man with bilateral T cell lymphoma causing adrenal insufficiency (Hum Pathol 1986;17:634)
76 year old man with Cytokeratin+, CD45-, centroblastic, diffuse large B cell lymphoma (Arch Pathol Lab Med 2001;125:1104)
76 year old man with primary adrenal diffuse large B cell lymphoma with paraneoplastic syndrome (N Am J Med Sci 2013;5:721)
83 year old man with bilateral primary adrenal nonHodgkin lymphoma without adrenal insufficiency (Urol Ann 2015;7:259)
83 year old woman with primary bilateral adrenal diffuse large B cell lymphoma (BMJ Case Rep 2016 Mar 18;2016)