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Wydanie 4/2006

Mięsak mięśni prążkowanych (rhabdomyosarcoma) oczodołu u dzieci i młodzieży - problemy diagnostyczne i terapeutyczne

Orbital Rhabdomyosarcoma in Children and Adolescents - Diagnostic and Therapeutic Problems

Mirosława Grałek^1,2, Barbara Chipczyńska¹, Krystyna Kanigowska¹, Wojciech Hautz¹

¹ Klinika Okulistyki, Instytut ?Pomnik ? Centrum Zdrowia Dziecka?? w Warszawie
Kierownik: prof. dr hab. n. med. Mirosława Grałek
² Klinika Okulistyki Katedry Pediatrii Zabiegowej Uniwersytetu Medycznego w Łodzi
P.o. kierownik: dr n. med. Anna Niwald

Summary: This article presents the current knowledge on pathology, diagnosis and treatment of orbital rhabdomyosarcoma (RMS) in children. Orbital rhabdomyosarcoma is the most often occurring primary common soft-tissue sarcoma in the first decade of life. This tumor originates from primitive fetal mesenchyma and often is not directly linked to the skeletal muscle. Histologically, the tumor resembles fetal striated muscle. There are four differentiated types of tumor: embryonal, alveolar, botryoid and pleomorphic. These tumors can disseminate hematogenously and via lymphatic system. Orbital RMS can manifest itself through proptosis, globe displacement, chemosis, or mass on the eyelid or conjunctiva. The diagnosis is based on biopsy, CT and MRI. Treatment includes chemotherapy, radiation and surgery. In general, orbital RMS in children has the best prognosis.

Keywords: rhabdomyosarcoma, orbit, children, clinical signs, histopathology, imaging diagnostic, treatment.

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