Chou S M, Anderson J S
Department of Pathology (Neuropathology), Cleveland Clinic Foundation, Ohio 44195.
Clin Neuropathol. 1991 Jan-Feb;10(1):1-10.
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.
原发性中枢神经系统横纹肌样瘤是一种神秘且极为罕见的儿童早期恶性肿瘤，可能起源于胚胎，在组织病理学上常与婴儿肾脏或其他器官的横纹肌样瘤归为一类。文献中仅发现4例先前记录的原发性中枢神经系统恶性横纹肌样瘤（MRT）病例。我们报告2例原发性中枢神经系统MRT病例，伴有活检及完整尸检结果，二者在临床病理、免疫组化及超微结构方面具有密切相似性。结论是，原发性中枢神经系统MRT是一种高度恶性的实体肿瘤，易被误诊为原始神经外胚层肿瘤，可能起源于与围绕肾脏及其他器官的浆膜间皮前体细胞在胚胎学上等同的脑膜间皮前体细胞。因此，它在解剖学上倾向于发生在脑膜褶皱丰富的部位，如小脑皮质，并且在脑膜受累时可能呈弥漫性或多中心性。此外，它可能与起源于其他器官（如肾脏）浆膜的MRT同时或多中心发生。
