Abstract
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.
Highlights
Malignant rhabdoid tumor is a rare and malignant tumor of childhood
We report here an 8-week-old female infant with malignant rhabdoid tumor of the orbit who was treated with chemotherapy and surgery
Sonuç olarak yenidoğan döneminde saptanan ve hızla büyüyen göz kitlelerinin ayırıcı tanısında Malign rabdoid tümör (MRT) yer almalı, vakit kaybetmeden cerrahi, kemoterapi ve lokal radyoterapi gibi tedavi seçenekleri kombine olarak kullanılmalıdır
Summary
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. Malign rabdoid tümör (MRT), çocukluk çağının nadir gözlenen ve kötü gidişli bir tümörüdür [1]. Mikroskopik olarak başta yumuşak doku sarkomları olmak üzere diğer sarkomlar, karsinomlar ve melanom gibi tümörlerle karışabildiği için ayırıcı tanı zor olabilir. Sağ gözün üst dış kısmında 4x5 cm boyutlarında sert, düzgün sınırlı kitle ve proptozis saptandı.
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