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PATHOLOGY TEACH AND TELL: SOLID VARIANT ALVEOLAR RHABDOMYOSARCOMA OF THE ORBIT

The case refers to an 11-year-old African American boy who was diagnosed 2 years prior to admission with a progressive right orbital rhabdomyosarcoma. The patient initially presented in 2003 with proptosis. He completed a 45-week chemotherapy course with vincristine and actinomycin-D and subsequently developed progression of his disease. He then was started on chemotherapy with cytoxan and doxorubicin, which failed to arrest his disease progression, and later he was started on irinotean and vinorelbine. Subsequently, a brain computed tomography (Figure 1) scan showed a mass in the right orbit with extensive heterogeneous enhancement with consequent proptosis and destruction of the surrounding bony structures. On review of systems, the patient reported right eye pain and headache and had poor oral intake. Review of systems was negative for fever, bone pain, chest pain, shortness of breath, vomiting, diarrhea, or rashes. His medications included septra 80 mg twice daily 3 days a week, azithromycin ointment every 4 hr to the right eye, neosporin ointment every 4 hr to the right eye, and cortaid 15 mg every 6 hr as needed for itching. On physical examination, the patient was alert and in no acute distress. He had a very dramatic right orbital proptosis with a huge mass protruding from the right eye. The left eye was normal with intact extraocular movements. Examination of the neck, chest, heart, abdomen, and extremities was unremarkable. A complete blood count demonstrated a mildly increased white blood cell count of 12,200 and an increased platelet count of 510,000. Hemoglobin and hematocrit were within normal range. The