Affection of the nervous system in leucoses and malignant lymphomas
Jellinger, K.; Slowik, F.
Zentralblatt für Allgemeine Pathologie U. Pathologische Anatomie 122(5): 439-461
1978
ISSN/ISBN: 0044-4030 PMID: 281819 Document Number: 135345
The involvement of the nervous system in leukemias and malignant lymphomas includes intracranial and spinal-epidural dissemination, isolated affection of the CNS (primary intracranial lymphomas), neuromuscular involvement and complications and secondary lesions (hemorrhages, infections). In a consecutive autopsy series of 180 acute and chronic cases of leukemia (including 45 children) CNS involvement was seen in 53.6% (71% in children), most frequently in acute leukemia (62 and 74%) with almost equal incidence of meningeal and parenchymal invasion (25% each, and 30-35% in children) and rare tumor formation (chloroma). Intracranial hemorrhages were present in 37%, secondary CNS infections in 16%. Among 180 consecutive autopsy cases of systemic malignant lymphomas, including lymphatic leukemias (Kiel classification), there was an overall CNS involvement in 24% or 30% of the non-Hodgkin's lymphomas (including ALL and less frequent in immunoblastomas and centrocytic lymphomas. Bone marrow involvement was present in 90% of the cases with secondary CNS lesions. Leukemic conversion, present in 44% of the total, was demonstrated in 83% of the cases with secondary CNS affection but rarely in epidural spreads. The histologic pattern of meningeal and perivascular infiltrations, with and without invasion of the nervous parenchyma, and rare tumor formation are identical with that seen in CNS leukemia. Neuromuscular involvement was present in 40% of the cases examined; intracranial hemorrhage was seen in 14% and secondary CNS infections in 16%. Pathogenic factors of secondary CNS involvement in leukemia and lymphomas are primarily hematogenous dissemination and rarely transdural invasion, transition of the blood-brain barrier or independent multifocal growth. Primary (isolated) intracranial malignant lymphomas, previously referred to as reticulum cell sarcoma-microglioma, are morphologically identical with extraneural lymphomas. They represent about 1% of intracranial neoplasms, most frequently immunoblastomas (45%) and immunocytomas (443) and less often lymphoblastomas. They may represent primary (lethal) manifestations of a multisystem disease without, and rarely with, secondary generalization, but their pathogenesis is obscure.