Adrenal relapse of primary central nervous system diffuse large B-cell lymphoma: A case report.

Journal Article (Journal Article)

RATIONALE: Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma with a dismal outcome. Most patients relapse in intracranial sites and <5% of patients relapse in extracranial sites. Here, we present the first case of PCNSL with an adrenal relapse. PATIENT CONCERNS: A 72-year-old woman, first presented 7 years ago with complaints of headache and dizziness. DIAGNOSES: Enhanced magnetic resonance imaging revealed the mass within the splenium of the corpus callosum. On histological examination, there was a diffuse growth pattern of neoplastic cells in the brain biopsy. Immunohistochemistry and flow cytometric analysis demonstrated that the neoplastic cells were of B-cell lineage. INTERVENTIONS: The patient underwent methotrexate-based chemotherapy and whole-brain radiotherapy after the initial diagnosis of primary central nervous system-large B-cell lymphoma (CNS-DLBCL). OUTCOMES: After 4 years of clinical remission, the patient was diagnosed with endometrial cancer. Interestingly, a radiological study following the treatment of endometrial cancer demonstrated a right adrenal mass, which was suspicious for malignancy. Morphologic examination and immunohistochemistry studies confirmed the diagnosis of diffuse large B-cell lymphoma. A fluorescent in situ hybridization panel for lymphoma showed rearrangement of Immunoglobulin heavy chain (IGH) and B-cell lymphoma 6 (BCL6), respectively, suggesting fusion of BCL6/IGH. Immunoglobulin kappa analysis demonstrated a common origin for the brain and adrenal lesions, which led to the final diagnosis of an adrenal relapse of CNS-DLBCL. LESSONS: PCNSL is a highly infiltrative neoplasm, particularly at relapse. To the best of our knowledge, this is the first case of CNS-DLBCL with adrenal relapse. Considering the poor outcome of CNS-DLBCL, molecular genetic studies should be done to identify a common origin for the primary and secondary lesion.

Full Text

Duke Authors

Cited Authors

  • Ma, S; Sen, SS; Jug, R; Zhang, X; Zhang, W-L; Shen, S; Yu, C-Q; Xu, H-T; Yang, L-H; Wang, E

Published Date

  • September 2018

Published In

Volume / Issue

  • 97 / 38

Start / End Page

  • e12482 -

PubMed ID

  • 30235749

Pubmed Central ID

  • PMC6160128

Electronic International Standard Serial Number (EISSN)

  • 1536-5964

Digital Object Identifier (DOI)

  • 10.1097/MD.0000000000012482


  • eng

Conference Location

  • United States