The most frequently encountered hematopoietic neoplasms of the brain encompass primary CNS lymphoma, intravascular lymphoma, post-transplant T-cell lymphoproliferation, intraocular lymphoma, neurolymphomatosis, and plasmacytoma.
Primary CNS lymphoma (PCNSL) is a diffuse large B-cell lymphoma confined to the CNS at presentation. It occurs in inherited or acquired immunodeficiency. There is an association with Epstein-Barr virus (EBV) infection in immunocompromised patients. Molecular changes include alterations and translocations in copy number of 9p.24/PD-L1/PD-L2, neurotropic factors, JAK/STAT signaling pathway, dysregulation in B cell receptor signaling pathway, in NF-κB pathway, in Toll-like receptor signaling pathway, mutations in PIM1, TBL1XR1, TRDM1, BTG2, PRDM1, protein-changing mutations, and chromosomal translocations involving oncogenes. Treatment in immunocompetent PCNSL consists in the administration of glucocorticoids, radiation therapy, and hemotherapy (CHOP: cyclophosphamide, doxorubicin, vincristine, prednisone). In AIDS-related PCNSL, high-dose intravenous methotrexate is given. 5-year survival is 29.3%. Molecular predictive markers for adverse prognosis or favorable outcome have been published.
Intravascular lymphoma is characterized by exclusively intravascular growth. It is sought to be derived from immunoglobulin gene rearrangement accompanied by altered expression of adhesion molecules, i.e., CD44, ß-1 integrin. Treatment consists in a combination of chemotherapy (combination of MTX-based intrathecal chemotherapy and R-CHOP), intrathecal therapy with cytarabine, prednisolone, intravascular use of unfractionated heparin, and bone marrow transplantation. Because of its aggressive behavior, survival ranges between 14 months and 2 years.
Post-transplant lymphoproliferative disorders (PTLDs) are lymphoid or plasmacytic proliferations that develop as a consequence of immunosuppression in a recipient of a solid organ or stem cell allograft. Surgical resection, reduction in immunosuppression, steroids, radiotherapy, high-dose methotrexate, and immunotherapy make up the treatment armamentarium. Prognosis is poor with a median survival of 13 weeks and a mortality rate of 60–90%.
Plasmacytoma is composed of mature-appearing neoplastic plasma cells. The neoplastic cells originate from postgerminal center. Clonal rearrangement of the IgL- and IgH-chain genes was described. Treatment includes surgical resection followed by radiation therapy. Clinical outcome is variable. 66% of cases progress within 3 years to generalized myeloma.
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