Turkish Journal of Pathology

Türk Patoloji Dergisi

Turkish Journal of Pathology

Turkish Journal of Pathology

2013, Vol 29, Num, 1     (Pages: 069-072)

Malignant Rhabdoid Tumor of the Orbit

Serap KARAMAN 1, Tiraje CELKAN 1, Sergülen DERVİŞOĞLU 2, Miraç ERGEN 1, Alp ÖZKAN 1, Hilmi APAK 1, İnci YILDIZ 1, Halit PAZARLI 3

1 Department of Pediatric Hematology-Oncology, İstanbul University, Cerrahpaşa Faculty of Medicine, İSTANBUL, TURKEY
2 Department of Pathology, İstanbul University, Cerrahpaşa Faculty of Medicine, İSTANBUL, TURKEY
3 Department of Eye, İstanbul University, Cerrahpaşa Faculty of Medicine, İSTANBUL, TURKEY

DOI: 10.5146/tjpath.2013.01152
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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.

Keywords : Rhabdoid tumor, Orbita, Child