Adrenal gland & paraganglia
Other tumors
Lymphoma
Author: Lan L. Gellert, M.D., Ph.D.
Topic Completed: 1 June 2016
Minor changes: 12 November 2020
Copyright: 2002-2021, PathologyOutlines.com, Inc.
PubMed Search: Lymphoma [title] adrenal
Table of Contents
Definition / general | Essential features | Epidemiology | Clinical features | Radiology images | Prognostic factors | Case reports | Treatment | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Molecular / cytogenetics description | Differential diagnosisCite this page: Gellert LL. Lymphoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/adrenallymphoma.html. Accessed May 7th, 2021.
Definition / general
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Primary adrenal lymphoma:
- 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)
Treatment
- Surgery and chemotherapy
- CHOP / CHOP-like chemotherapy regimen plus rituximab
Microscopic (histologic) description
- See Micro Description in diffuse large B cell lymphoma
Microscopic (histologic) images
Images hosted on other servers:
Primary adrenal gland diffuse large B cell lymphoma is CD45+, CD20+
Primary adrenal gland Hodgkin lymphoma
Positive stains
- See Positive Stains in diffuse large B cell lymphoma
Negative stains
- See Negative Stains in diffuse large B cell lymphoma
Molecular / cytogenetics description
- See Molecular / Cytogenetics Description in diffuse large B cell lymphoma
Differential diagnosis
- Metastatic carcinoma: Cytokeratin+, negative for lymphoid markers
- Metastatic melanoma: S100+, MelanA+, HMB45+
- Primary adrenal tumor: negative for lymphoid markers
