Granulocytic Sarcoma in Childhood Acute Myelogenous Leukemia: Clinical Characteristics and Management

Oh, Chan Kyun; Kook, Hoon; Kim, Seok Joo; Noh, Ha Young; Sohn, Kyoung Ran; Baek, Hee Jo; Hwang, Tai Ju

Abstract

PURPOSE
Granulocytic sarcoma (GS), an extramedullary tumor consisting of primitive myeloid cells, is a rare manifestation of acute myelogenous leukemia (AML). However, GS can occasionally precede the development of systemic leukemia by weeks to years. The objectives of this study are to describe the frequency, clinical characteristics and survival of AML children with GS from a single Korean institute. METHODS: Retrospective review of all the AML children who presented between January, 1995 and June, 2003 was undertaken. RESULTS: GS developed in 9 children among 118 AML patients (incidence, 7.6%). The median age at diagnosis of AML was 82 months (8 months~13 years) with equal sexual distribution. The sites of GS were scalp (n=4), skull (n=3), paranasal sinuses (n=1), external auditory canal (n=1), spinal epidura (n=1), and spinal intramedulla (n=1). The symptoms related with GS were scalp mass (n=4), paraparesis (n=3), facial nerve palsy (n=3), hearing impairment (n=2), and exophthalmos (n=1). In the case with spinal epidural mass, GS preceded the diagnosis of AML by 15 months. Cytogenetics were available in 8 cases, and t (8; 21) was found in five cases. All cases received systemic chemotherapy, with surgical decompression and radiotherapy for 2 patients involving spine. Seven cases received stem cell transplantations (3, allogeneic bone marrow; 4, autologous peripheral blood). The 5-yr event-free survival was 35.0% by Kaplan-Meier method. All 3 allografted patients are alive (86 mo, 5 mo, 1 mo), while 3 of 4 autografted patients had either died or relapsed. CONCLUSION: GS should be considered in patients with or even without AML who have palpable mass or neurological manifestation. Effective treatment, including allogeneic stem cell transplantation, should be considered to achieve a durable disease control.