Granulocytic Sarcoma as the Presenting Manifestation of Acute Leukemia in Childhood

Abstract

This report describes six cases of acute leukemia in childhood manifested initially by granulocytic sarcoma. Solid tumor was associated with acute monocytic leukemia in one patient and with acute myelogenous leukemia in other patients. In two patients, generalized lymphadenopathy was the predominant feature preceding other signs and symptoms of acute leukemia. One case revealed the clinical course of smoldering acute leukemia characterized by insidious onset and a small number of myeloblasts in the bone marrow. One case showed Mikulicz's syndrome preceding the systemic manifestation of leukemia, and the peripheral blood was normal at the time of admission. In another patient with an orbital tumor, three months after the biopsy which was reported to be reticulum cell sarcoma, blood and marrow were typical of acute leukemia. In this case, peripheral blood picture was normal at the time of tumor formation, but marrow examination was not performed. The last case was unusual in that myeloblastoma preceded the blood and marrow evidence of acute leukemia by 38 months. Review of literature revealed 6 well-documented cases of granulocytic sarcoma preceding the marrow evidence of leukemia. The importance of cytological examination by Peroxidase reaction and May-Giemsa staining on imprints prepared from biopsy specimen together with histological examination in differentiating acute leukemia from reticulum cell sarcoma was stressed. Chloromas are green tumor masses composed of primitive white blood cells, usually associated with acute myelocytic or myelomonocytic leukemia, occurring more often in childhood than in adults. Myeloblastoma—tumorous masses histologically identical to chloroma but without greenish pigmentation—is often used as a synonym. But the term granulocytic sarcoma may be more appropriate to describe these tumors (Rappaport, 1966). Occasionally, tumor formation may be the earliest manifestation preceding other symptoms, signs and bone marrow evidence of acute leukemia.