Orbital granulocytic sarcoma in a myelodysplastic syndrome

Abstract

PURPOSE . To report an elderly patient with a primary myelodysplastic syndrome (MDS) type refractory anemia with excess of blasts (RAEB) who developed an orbital granulocytic sarcoma (GS) as a harbinger of an acute myelogenous leukemia (AML). METHODS . A 77-year-old man was diagnosed as having a MDS type RAEB. Eight months later he developed a progressive painless proptosis in his left orbit. A computed tomography (CT) scan revealed a large irregular mass involving the orbit. It showed heterogeneous soft tissue density and no osseous cortical destruction was observed. A diagnostic lateral orbitotomy with an excisional biopsy were performed. RESULTS . Histopathology showed sheets of immature granulocytic cells. Immunohistochemical staining was positive for markers for myeloperoxidase, which supported the diagnosis of GS. CONCLUSIONS . Reports of a GS complicating the course of a MDS are few. Sites of detection of the extramedullary tumors (EMT) in MDS vary, although cutaneous sites predominate. An orbital site is a very rare localization of this tumor in adults. The case we report is the first one with an orbital granulocytic sarcoma in an MDS-type RAEB in an elderly patient.