Primary CNS malignant rhabdoid tumor (MRT): report of two cases and review of literature.
Primary CNS rhabdoid tumor is an enigmatic and extremely rare malignant tumor of early childhood, probably embryonal, that has often been histopathologically classified together with rhabdoid tumor of kidneys or other organs in infants. Only four previously documented cases of primary CNS malignant rhabdoid tumor (MRT) were found in the literature. We report two cases of primary CNS MRT with biopsy and complete autopsy findings that share close clinicopathologic, immunohistochemical and ultrastructural similarities between the two. It is concluded that the primary CNS MRT is an entity which is extremely malignant, easily mistaken as a primitive neuroectodermal tumor, and potentially derives from a meningothelial precursor cell which is embryonally equal to the serosal mesothelial precursor cells which surround the kidneys and other organs. Hence, it tends to anatomically occur at the location of abundant meningeal infoldings such as the cerebellar cortex and may be diffuse or multicentric in its meningeal involvement. Furthermore, it may concurrently or multicentrically occur in association with MRT originating from the serosal membrane of other organs, such as the kidney.
Chou
S M
SM
Department of Pathology (Neuropathology), Cleveland Clinic Foundation, Ohio 44195.
Anderson
J S
JS
D014408:Biomarkers, Tumor / Q000032:analysis*; D001932:Brain Neoplasms / Q000473:pathology*; D002528:Cerebellar Neoplasms / Q000473:pathology*; D002675:Child, Preschool; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008578:Meninges / Q000473:pathology; D008637:Mesenchymoma / Q000473:pathology*; D008854:Microscopy, Electron; D013702:Temporal Lobe / Q000473:pathology*
D014408:Biomarkers, Tumor