Karaman Serap, Celkan Tiraje, Dervışoğlu Sergülen, Ergen Miraç, Özkan Alp, Apak Hilmi, Yildiz Inci, Pazarli Halit
İstanbul Üniversitesi, Cerrahpaşa Tıp Fakültesi, Pediatrik Hematoloji-Onkoloji, Anabilim Dalı, İstanbul, Türkiye.
Turk Patoloji Derg. 2013;29(1):69-72. doi: 10.5146/tjpath.2013.01152.
Malignant rhabdoid tumor is a rare and malignant tumor of childhood. Generally it originates from the kidney and central nervous system, but occasionally it may arise from the orbit. Differential diagnosis from other childhood soft tissue sarcomas should be done. We report here an 8-week-old female infant with malignant rhabdoid tumor of the orbit who was treated with chemotherapy and surgery. The 8 week-old girl was referred to our hospital with a history of right proptosis first noted at birth. Physical and laboratory evaluation of the patient was normal except for right proptosis. The mass was removed surgically. Histopathologic examination and immunohistochemical findings of the specimen were evaluated as malignant rhabdoid tumor. Chemotherapy was administered. While in clinical remission, she succumbed during a febrile episode. Malignant rhabdoid tumor can rarely originate from the orbit. Malignant rhabdoid tumor should be kept in mind in the differential diagnosis of orbital masses, and surgery, chemotherapy and local radiotherapy should be used as combined therapy due to the poor prognosis.
恶性横纹肌样瘤是一种罕见的儿童恶性肿瘤。一般起源于肾脏和中枢神经系统，但偶尔也可能起源于眼眶。应与其他儿童软组织肉瘤进行鉴别诊断。我们在此报告一名8周大的眼眶恶性横纹肌样瘤女婴，接受了化疗和手术治疗。这名8周大的女孩因出生时首次发现右眼球突出的病史被转诊至我院。除右眼球突出外，患者的体格检查和实验室检查均正常。肿块通过手术切除。对标本进行组织病理学检查和免疫组化检查，结果评估为恶性横纹肌样瘤。给予化疗。在临床缓解期间，她在一次发热发作中死亡。恶性横纹肌样瘤很少起源于眼眶。在眼眶肿块的鉴别诊断中应考虑恶性横纹肌样瘤，由于预后较差，应采用手术、化疗和局部放疗联合治疗。
