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NR. 4/2006
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Mięsak mięśni
prążkowanych (rhabdomyosarcoma) oczodołu u dzieci i młodzieży –
problemy diagnostyczne i terapeutyczne
Orbital Rhabdomyosarcoma in Children and Adolescents –
Diagnostic and Therapeutic Problems
Mirosława Grałek1,2,
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 |
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| 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. |
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| Key words: |
rhabdomyosarcoma, orbit,
children, clinical signs, histopathology, imaging
diagnostic, treatment. |
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