%0 Journal Article
%T Congenital Orbital Rhabdomyosarcoma
%A Amir Reza Farsiani
%A Masoomeh Eghtedari
%A Mohammad Reza Bordbar
%J Archive of "Ocular Oncology and Pathology".
%D 2018
%R 10.1159/000481533
%X Rhabdomyosarcoma (RMS) is the most common malignant tumor of the orbit in children, but it is rarely present at birth. We report a large congenital orbital RMS with intracranial extension in a newborn baby. A newborn baby girl was referred to our hospital due to severe right-eye exophthalmia. Imaging studies showed an orbital mass with intracranial extension. Treatment was started with a course of neoadjuvant chemotherapy followed by right orbital exenteration and intracranial resection of the tumor. Histologic examination of the mass showed undifferentiated malignant small-cell tumor. Immunohistochemical study proved it to be RMS. The patient was categorized as intermediate-risk RMS and chemotherapy was continued accordingly with VAC regimen. In spite of treatment, the infant developed intracranial recurrence at the age of 6 months and died 1 month later. Congenital orbital RMS has a poor prognosis. Reconstruction surgery should be deferred due to high rate of recurrence and low chance of survival. A multidisciplinary approach might increase the survival of these patients
%K Rhabdomyosarcoma
%K Orbit
%K Congenital tumor
%K Exenteration
%U https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5939714/