Ewing's Sarcoma of the Cavernous Sinus: Case Report

OBJECTIVE AND IMPORTANCE: The Ewing's sarcoma (ES) family of tumors is a relatively rare entity, presenting most commonly in children. While the most common sites for this group of tumors are the trunk and the extremities, this case is unique in that we describe a long-term follow-up with an ES presenting in the cavernous sinus. CLINICAL PRESENTATION: We report the case of a 13-year-old girl presenting with the symptoms of classic orbital apex syndrome. Magnetic resonance imaging disclosed a cavernous sinus mass engulfing the optic nerve and extending into the superior orbital fissure. INTERVENTION: Evaluation of this patient included intracranial magnetic resonance imaging angiography and biopsy of the cavernous sinus mass lesion. Histopathological findings yielded a diagnosis of ES. Further work-up demonstrated that the patient's thyroid was also involved. The tumor was treated with radiation therapy to both sites and, subsequently, after confirmed metastases to other sites, 11 cycles of doxorubicin-based chemotherapy, as well as bone marrow transplantation. However, the patient succumbed to the illness 18 months after her initial diagnosis. CONCLUSION: The cavernous sinus is an unusual site for ES, but given the vascularity and the frequency of this tumor in childhood, the diagnosis should be entertained. This patient with an ES of cavernous sinus may be the first reported case with a long-term follow-up in the literature.