One Peak is Worth Twenty Finesses - Video

FNAB was performed and demonstrated a small blue cell population with negative Immunohistochemical staining for CD3, CD19. CD79a, CD23, TDT, Yf4 , desmin and S100. There was staining of FLI1 and significant staining for SMI33, as well as focal patchy staining for NSE and synaptophysin. FISH demonstrated an intact SYT gene. The patient was diagnosed as having a Ewing's like sarcoma and started on chemotherapy with vincristine, Cytoxan, and doxorubicin. Ewing sarcoma frequently involves bone, but has rarely involved the orbit. In Reese's book, Orbital Tumors (1st edition (1951) 2nd edition (1963)) fails to mention any patient with a Ewing sarcoma involving the orbit. Fred Jacobiec's book on Ocular and Adnexal Tumor (1978) discussed metastatic sarcomas but there were no cases that were identified as Ewing sarcoma. Henderson's Tumors of the Eye (1994) reviewed the literature, but added no patients with presumed Ewings. A specific diagnosis has been helped by our recognition of the presence of EWSR1 gene rearrangement as well as FLI1 rearrangement. Recently multiple individual case reports have appeared. These tumors often occur in young patients most frequently involving the long bones and only rarely have they involved the bones of the skull base. The rapid presentation here was a clue of a more aggressive process. The greater wing of the sphenoid was involved. Neurosurgical consultation discussed the possibility of a craniotomy and excision. In the 1980s, Jack Kennerdell introduced fine needle aspiration biopsy to the orbit. Obviously in most children fine needle aspiration biopsy, except under general anesthesia would not have been considered, but in select cooperative patients (such as our case) a specific diagnosis can be made without a trip to the operating room, when combined with histochemical staining.