Congenital Orbital Rhabdomyosarcoma

Ocular Oncology and Pathology
Masoomeh EghtedariMohammad Reza Bordbar

Abstract

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.

References

Jan 3, 2001·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·O OberlinUNKNOWN International Society of Paediatric Oncology Sarcoma Committee, Intergroup Rhabdomyosarcoma Study Group, Italian Cooperative
Oct 6, 2009·Ophthalmology·Arun D SinghLynn Schoenfield
May 31, 2011·Current Topics in Developmental Biology·Ken KikuchiCharles Keller
Apr 12, 2013·Journal of Oncology·Ajay A RaoHema L Ramkumar

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