Sporadic form of isolated orbital Burkitt lymphoma presenting with fulminant proptosis and dystopia

Abstract

This is a rare case report of fulminant proptosis with dystopia caused by isolated orbital involvement of nonendemic Burkitt lymphoma. We present the case of a 6-year-old Egyptian child who suffered from progressive, painful proptosis and dystopia in his left eye for 10 days. We discovered a noncompressible, soft-tissue mass at the superotemporal part of the orbit. The presence of an orbital inflammatory pseudotumor was suspected when the patient was administered with systemic corticosteroids and antibiotics. Histopathological examination of the obtained specimen revealed the “starry-sky” appearance of histiocytes, which is characteristic of Burkitt lymphoma. The patient underwent chemotherapy with remarkable improvement of his dystopia and proptosis. Although orbital Burkitt lymphoma is a rare tumor, ophthalmologists should be aware of its differential diagnosis of rapidly progressive proptosis with dystopia in children, which may initially masquerade as orbital cellulitis or a pseudotumor.

Keywords

• burkitt lymphoma
• dystopia
• proptosis