Adrenal relapse of primary central nervous system diffuse large B-cell lymphoma

Abstract

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.

Relapsed Primary Central Nervous System Lymphoma: Current Advances

“…Sites of isolated systemic relapses include lymph nodes (lung, ventral, paratracheal, retroperitoneal, and cervical), the musculoskeletal system, testis, bone marrow, kidney and adrenal structures, extranodal lymphoid tissue, liver, small intestine, and abdominal wall, but lymph nodes, the musculoskeletal system, testis and bone marrow are the most common ( 19 , 35 , 45 ). Isolated systemic relapses can also occur in rare sites.…”

“…Patients with isolated systemic relapses usually receive chemotherapy alone (e.g., CHOP, R-CHOP, ACVBP, DHAP, and R-hyper CVAD), surgery alone, radiation therapy alone, hematopoietic stem cell transplantation or combination. The CR rate is 50-83%, and the mOS after relapse is 15.5 months ( 19 , 35 , 45 , 47 ). Most relapsed patients with NL receive chemotherapies based on high-dose MTX or cytarabine and radiation therapy, while patients with brain/spinal root involvement or higher CSF cell counts receive intrathecal chemotherapy, and the survival rates at 12 months and 36 months are approximately 46% and 24%, respectively ( 50 , 51 ).…”

Systemic Relapse in a Young Adult Patient with Primary CNS Diffuse Large B-Cell Lymphoma

“…PCNS-DLBCL is characterized by an aggressive disease course and overall long-term survival rates that range from 20% to 40% [ 5 ]. Age, performance status, lactate dehydrogenase levels, and elevated cerebrospinal fluid protein are variables used in prognostic models [ 7 ].…”

“…Interestingly, there are few published cases of adrenal relapse. Ma et al described a patient found to have bilateral adrenal involvement of their PCNS-DLBCL following MTX-based chemotherapy and WBRT, who was started on rituximab-hyper CVAD and then transitioned to R–CHOP [ 5 ]. In patients found to have bilateral adrenal involvement, primary adrenal lymphoma (PAL) with secondary CNS involvement should also be considered in the differential [ 5 ].…”

“…While CNS relapse is common, systemic relapse is rare, occurring in 7–10% of patients and also represents a major therapeutic challenge [ 5 ]. Novel insights into the pathophysiology of PCNSL have identified the B-cell receptor (BCR) pathway as a key mechanism in the pathogenesis of PCNS-DLBCL.…”