A previously healthy child presented with bilateral periorbital edema, right greater than left proptosis, with generalized facial swelling and copious nasal discharge, not improved on antibiotics. Imaging revealed multiple extraconal soft tissue masses in the orbits bilaterally with bilateral proptosis, and also showed an extensive process involving calvarium, facial osseous structures with subperiosteal and epidural extension, and extensive lymphadenopathy. Patient underwent right superior orbitotomy with biopsy. Surgical pathology showed hematolymphoid neoplasm. Flow cytometry and bone marrow biopsy confirmed acute myelomonocytic leukemia with CBFB-MYH11 translocation. CSF was negative for malignancy. The patient was initiated on chemotherapy with cytarabine, daunorubicin, dexrazoxane, gemtuzumab, and intrathecal methotrexate, hydrocortisone, and cytarabine. The patient was negative for Trisomy 21. After cycle one of chemotherapy, she underwent repeat LP, Bone marrow biopsy, and MRI, which showed decreased tumor burden and profound improvement of bilateral intraorbital extraconal masses.
Presentation Date: 11/30/2023
Issue Date: 12/15/2023