• Shuang Ma, Siddhartha Sen Sen, Rachel Jug, Xuefeng Zhang, Wan-Lin Zhang, Shuai Shen, Cheng-Qian Yu, Hong-Tao Xu, Lian-He Yang, and Endi Wang.
• Department of Neurology, Sheng Jing Hospital of China Medical University, Shenyang, Liaoning, China Department of Pathology, Duke University Medical Center, Durham, NC Department of Pathology, College of Basic Medical Sciences and First Affiliated Hospital, China Medical University, Shenyang, Liaoning, China.
• Medicine (Baltimore). 2018 Sep 1; 97 (38): e12482.

RationalePrimary 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 ConcernsA 72-year-old woman, first presented 7 years ago with complaints of headache and dizziness.DiagnosesEnhanced 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.InterventionsThe patient underwent methotrexate-based chemotherapy and whole-brain radiotherapy after the initial diagnosis of primary central nervous system-large B-cell lymphoma (CNS-DLBCL).OutcomesAfter 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.LessonsPCNSL 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.

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