Intraspinal atypical teratoid/rhabdoid tumor

Chinese Journal of Contemporary Neurology and Neurosurgery

Hua-liang XIAO¹  Xiao-chu YAN²  Xiao-hong YAO²  Xiu-wu BIAN² 

[1] Department of Pathology, Daping Hospital, the Third Military Medical University of Chinese PLA, Chongqing 400042, China;Institute of Pathology, Southwest Hospital, the Third Military Medical University of Chinese PLA, Chongqing 400038, China;

关键词: Teratoma;    Rhabdomyoma;    Immunohistochemistry;    Pathology;   

DOI  :  

来源: DOAJ

【 摘 要 】

Objective To investigate the clinicopathological featuresof atypical teratoid/rhabdoid tumor (AT/RT) occurred in the spinal cord. Methods The clinical manifestations, imaging,histopathological features and immunophenotype were analyzed in one case ofAT/RT, and relevant literatures were reviewed.  Results A12-year-old male patient presented gradually aggravated pain of both lowerextremities. The patient was gradually not able to stand, and then developed tobe paralyzed and could not turn over by himself. Spinal MRI revealed abnormalsignals in the lumbosacral portion of spinal cord, and exhibited heterogeneousenhancement after contrast. The lesion was totally removed under microscopy.Histologically, the tumor was invasive, with diffusely distributed tumor cellsand old
hemorrhage. Some tumor cells were immature cells with small volume,obvious atypia and hyperchromatic nuclei. Others presented classic rhabdoidfeatures: round to oval cells, eccentrically placed and vacuolar nuclei withprominent nucleoli, and hyperchromatic cytoplasm with eosinophilic roundinclusion bodies. Tumor stroma showed mucoid degeneration. Immunohistochemicalstaining showed partial positivity in the cytoplasm of tumor cells for vimentin(Vim) and smooth muscle actin (SMA). Tumor cells were negative for synaptophysin(Syn), chromogranin A (CgA), S-100 protein (S-100) and oligodendrocytetranscription factor-2 (Olig-2). Besides, tumor cells lacked nuclear expressionof integrase interactor 1 (INI1). Ki-67 labeling index was 15%-20%. Finalpathological diagnosis was AT/RT. After operation the patient was treated byadjuvant chemotherapy and radiotherapy, and symptoms of lower limbs wereimproved markedly. The patient was admitted for 23 d (until the end of firstchemotherapy), and was followed up for 8 months after discharge without tumorrecurrence.  Conclusions AT/RT rarely occurs in spinal cord.The clinical and imaging manifestations are not specific, so a definitediagnosis mainly depends on histological and immunohistochemical features.Especially, lack of nuclear expression of INI1 in tumor cells is thecharacteristic marker of AT/RT.

 

DOI: 10.3969/j.issn.1672-6731.2016.06.006

【 授权许可】

Unknown