Heart of Darkness - Path PPT

Systemic steroids were restarted and orbital radiation was initiated. That month, vision worsened to LP and optic nerve pallor developed. Despite aggressive medical and radiation therapy, worsening clinical course prompted family and team to proceed with pterional craniotomy for intracranial tumor resection. Microscopic examination demonstrated a mass of spindle cells, focally arranged in intersecting fascicles. EBV-encoded RNA (EBER) stain was EBV-positive. Immunohistochemical studies revealed smooth-muscle-actin (SMA) positive lesional cells. EBV-SMT are rare (122 reported since 1970) and typically manifest under immunosuppression (commonly post-solid-organ transplantation, also with HIV). 29 cases have been reported in children following solid-organ transplantation. 1 In contrast to other EBV-associated-malignancies (Burkitt lymphoma, Hodgkin disease, nasopharyngeal carcinoma), malignant potential of these tumors is uncertain. Mean age of onset is 39 years, 68% of patients have multiple tumors, which are typically well-differentiated, exhibit slow mitotic activity, and are locally invasive. It is unclear if these represent metastases or loci of infection. 2 Liver, lungs, brain, and GI tract are typical sites for EBV-SMT, though orbital cases have been reported. They include: two HIV-positive patients in their 40s - one with a fusiform orbital mass surrounding the optic nerve, the other with a retro-orbital mass; a 52-year old renal transplant patient with two iris lesions; and a 12-month-old status-post two liver transplants with a posterior orbital mass extending intracranially. 3 No established treatment pattern exists, but it may include surgical resection, chemotherapy/radiation, antiviral therapy, and reduced immunosuppression. Patient remains NLP in the left eye. Repeat MRI is scheduled.