[Clinicopathologic and immunohistochemical study of atypical teratoid/rhabdoid tumor of central nervous system].
OBJECTIVE
To study the clinicopathologic features and differential diagnosis of atypical teratoid/rhabdoid tumor (AT/RT) occurring in the central nervous system.


METHODS
Two cases of AT/RT were studied by hematoxylin-eosin, reticulin and immunohistochemical staining. The clinical and pathologic features were analyzed and the literatures reviewed.


RESULTS
Histologically, 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.


CONCLUSIONS
AT/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.
Wang
Zhen
Z
Department of Pathology, First Affiliated Hospital, Nanjing Medical University, Nanjing 210029, China. mtshp@21cn.com
Fan
Qin-he
QH
Yu
Mei-ning
MN
Zhang
Wei-ming
WM
D000071443:12E7 Antigen; D000199:Actins / Q000032:analysis; D000328:Adult; D015703:Antigens, CD / Q000032:analysis; D001932:Brain Neoplasms / Q000378:metabolism / Q000473:pathology*; D015815:Cell Adhesion Molecules / Q000032:analysis; D002675:Child, Preschool; D003893:Desmin / Q000032:analysis; D005904:Glial Fibrillary Acidic Protein / Q000032:analysis; D006801:Humans; D007150:Immunohistochemistry; D007633:Keratins / Q000032:analysis; D008297:Male; D018396:Mucin-1 / Q000032:analysis; D009130:Muscle, Smooth / Q000737:chemistry; D016900:Neurofilament Proteins / Q000032:analysis; D018335:Rhabdoid Tumor / Q000378:metabolism / Q000473:pathology*; D009418:S100 Proteins / Q000032:analysis; D013724:Teratoma / Q000378:metabolism / Q000473:pathology*; D014746:Vimentin / Q000032:analysis
D000071443:12E7 Antigen; D000199:Actins; D015703:Antigens, CD; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; D003893:Desmin; D005904:Glial Fibrillary Acidic Protein; D018396:Mucin-1; D016900:Neurofilament Proteins; D009418:S100 Proteins; D014746:Vimentin; D007633:Keratins