Sarcoma granulocitico dell'orbita: Case report

Granulocytic sarcoma is a rare solid tumor formed by primitive myeloid cells. It represents an expression of acute myeloid leukaemia or, less frequently, of chronic ones or of myeloproliferative diseases. Besides, it can appears some weeks or years before the development of such diseases. The authors describe the case of an orbital chloroma in a patient with a clinical history of progressive exophthalmos and diplopia, without signs and symptoms in relation to a systemic disease. The lesion was studied by CT and MRI and then was operated; histopathological diagnosis was of orbital granulocytic sarcoma. After the patient underwent molecular investigations on peripherical blood and bone marrow biopsy which allowed to do the diagnosis of a minor myeloproliferative syndrome. Therefore, the patient was submitted to a chemotherapeutic treatment in according of ICE scheme. At 1-year clinical and radiological follow-up, there was no evidence of local regrowth of disease. The authors explain clinical aspects and compare them with literature data; they outline the importance of an early diagnosis to begin an aggressive chemotherapeutic regimen above all in the cases where sarcoma appears before the development of myeloproliferative disease.