Clinicopathologic and immunohistochemical study of atypical teratoid/rhabdoid tumor of central nervous system.
- Author:
Zhen WANG
1
;
Qin-he FAN
;
Mei-ning YU
;
Wei-ming ZHANG
Author Information
- Publication Type:Case Reports
- MeSH: 12E7 Antigen; Actins; analysis; Adult; Antigens, CD; analysis; Brain Neoplasms; metabolism; pathology; Cell Adhesion Molecules; analysis; Child, Preschool; Desmin; analysis; Glial Fibrillary Acidic Protein; analysis; Humans; Immunohistochemistry; Keratins; analysis; Male; Mucin-1; analysis; Muscle, Smooth; chemistry; Neurofilament Proteins; analysis; Rhabdoid Tumor; metabolism; pathology; S100 Proteins; analysis; Teratoma; metabolism; pathology; Vimentin; analysis
- From: Chinese Journal of Pathology 2006;35(8):458-461
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVETo study the clinicopathologic features and differential diagnosis of atypical teratoid/rhabdoid tumor (AT/RT) occurring in the central nervous system.
METHODSTwo cases of AT/RT were studied by hematoxylin-eosin, reticulin and immunohistochemical staining. The clinical and pathologic features were analyzed and the literatures reviewed.
RESULTSHistologically, AT/RT was characterized by the presence of rhabdoid cells associated with various degrees of primitive neuroectodermal, epithelial or mesenchymal differentiation. Abundant reticulin fibers and a complex immunophenotype were observed. The tumor cells were positive for vimentin, CD99, epithelial membrane antigen, cytokeratin, glial fibrillary acidic protein, S-100 protein, neurofilament, desmin and smooth muscle actin. They were negative for synaptophysin, MyoD1, placental alkaline phosphatase and HMB45.
CONCLUSIONSAT/RT is a highly malignant tumor occurring in the central nervous system. It manifests mainly in children and occasionally in adults. The tumor is characterized by a heterogeneous histologic and immunohistochemical phenotype. It needs to be distinguished from a number of central nervous system tumors, including medulloblastoma, primitive neuroectodermal tumor, germ cell neoplasm and rhabdoid meningioma.
