Dr.Sonal Chaugule
Purpose: To report clinical, radiological, histopathological features and outcome after multimodal management in a rare case of orbital ATRT
Case Report: A 3-year-old girl presented with subacute proptosis of left eye with features of compressive optic neuropathy. Neuro-imaging showed a large intraconal mass with variegated intensity in close association with the optic nerve, and extending to the orbital apex. An incision biopsy, followed by histopathology and immunohistochemistry confirmed the diagnosis of extrarenal, noncerebral ATRT. She underwent stereotactic EBRT (4600 cGy) sandwiched between 2 sessions of 3 cycles of chemotherapy with Vincristine, Adriamycin and Cisplatin, each. After 17 months of follow up, she remains stable with PET proven inactive scar and without any ocular or systemic complications
Conclusion: Multimodal treatment involving chemotherapy and radiation can achieve eye as well as life salvage in rare tumors such as atypical teratoid rhabdoid tumour.
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