PubMed · 11079144
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E Zucca, F Cavalli. 2000. Extranodal lymphomas.. https://doi.org/10.1093/annonc%2F11.suppl_3.219
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Vacuolar myelopathy (VM) in leukemia is rare. We report a boy with leukemia who developed isolated central nervous system (CNS) relapse during reinduction therapy. 5 months after cranial radiotherapy, he gradually developed quadriparesis. Magnetic resonance imaging revealed an intramedullary lesion which extended through the cervical spine. Serum vitamin B12, folic acid, cerebrospinal fluid methyl malonic acid were normal. Viral screening by ELISA was negative. He had lymphopenia, and reduced immunoglobulins, from a cardiac arrest. Biopsy revealed VM. He responded to weekly vitamin B12 treatment but on the 6th week of the therapy he died after developing periventricular, gliotic, hyperintense lesions in the brain.
Roughly 30-40% of diffuse large B-cell lymphomas (DLBCL) arise primarily in extranodal sites. Most frequently, they occur in the gastrointestinal tract, especially in the gastric mucosa. They also occur in the central nervous system, as testicular lymphomas, in the lungs, or in the skin. Morphologically, they show the whole spectrum of peripheral B-blasts: centroblasts, immunoblasts, or plasmoblasts. Thus, there is no actual difference in their cytomorphological presentation compared to their nodal-and frequently systemic-counterparts. However, recent data point to profound differences in primary extranodal DLBCL compared to primary nodal tumors, as well as to each other, frequently relating to their molecular characteristics and especially implying organotypic features. These characteristics may relate to a particular organotypic site of origin, or the particular clinico-pathogenetic setting in which the tumors arise. This is exemplified in the description of the DLBCL subtypes as defined by the World Health Organization classification (mediastinal or intravascular B-cell lymphoma; primary effusion lymphoma). On the other hand, primary extranodal DLBCL are frequently characterized by a particular (cyto-)genetic constitution, often related to their site of origin. Finally, some preliminary data on gene expression profiling strongly argue in favor of particular gene signatures for primary extranodal DLBCL, and hence in favor of particular organotypic transformation pathways.
Non-Hodgkin's lymphoma invades the brain, the vitreous body and nerves of the eye, the meninges, and the nerve roots of brain and spine, leading to the development of a primary CNS lymphoma. The mechanism of involvement of these locations by malignant B lymphocytes is unknown, but it might involve molecular targeting of lymphoma cells generated at cryptic systemic sites. The diagnosis of primary CNS lymphoma has been facilitated by advances in imaging techniques and the discovery of molecular markers. Methotrexate-based regimens, even when radiation is deferred, prolong overall survival to over 5 years, but relapses eventually occur in most cases. Better tools for earlier diagnosis and monitoring of treatment response will emerge from molecular studies of therapeutic targets.