Myeloid sarcoma, spectrum of clinical characteristics, prognostic impact, and treatment outcome

Abstract

Background

Acute leukemia may present with extramedullary (EM) tissues. Myeloid sarcoma (MS) and leukemia cutis (LC) are considered extramedullary diseases. This study aims to evaluate the incidence, clinical characteristics, and prognostic factors affecting the outcome of pediatric patients with myeloid sarcoma at the pediatric oncology center from July 2007 to December 2017. Radiological imaging was used to stage the tumor, biopsy was done for pathological diagnosis, and bone marrow aspirate for morphology, flow cytometry, cytogenetics, and molecular analysis. Patients received chemotherapy protocols based on those used by the Children's Cancer Group for acute myeloid leukemia (AML).

Result

The study included 91 (13.2%) of 687 pediatric patients with acute myeloid leukemia, with a male-to-female ratio of 1.9 to 1. Prognostic factors that improved the patient’s 5-year overall survival (OS) were age > 5 years old, molecular and cytogenetic detection of t (8; 21), inv 16, presence of a single and small size lesion < 5 cm, negative CNS lesion, and achieved radiological response in isolated disease or radiological and marrow complete remission in disseminated disease post induction 1 with significant P value. Relapse, particularly early relapse, worsens the OS and EFS by 10% and 7.7%, respectively.

Conclusion

Patients over five with low-risk disease based on cytogenetics, a small, single, negative CNS lesion, and a complete response post induction 1 had better outcomes, with no significant difference between those with isolated extramedullary disease and those with marrow dissemination. Early relapse harms the outcome. The study group’s 5-year OS and EFS rates are 51.4% and 49.4%, respectively.