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Superior outcome of pediatric acute myeloid leukemia patients with orbital and CNS myeloid sarcoma: A report from the Children's Oncology Group
Author(s) -
Johnston Donna L.,
Alonzo Todd A.,
Gerbing Robert B.,
Lange Beverly J.,
Woods William G.
Publication year - 2012
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.23201
Subject(s) - medicine , myeloid leukemia , myeloid sarcoma , leukemia , cerebrospinal fluid , myeloid , chemotherapy , oncology , gastroenterology , pathology
Background Extramedullary leukemia (EML) is common in pediatric acute myeloid leukemia (AML) and occurs as leukemia cells within the cerebrospinal fluid (CSF) or as a solid tumor (myeloid sarcoma—MS). The effect of MS on survival is unknown. Methods Patients on CCG protocols 2861, 2891, 2941, and 2961 being treated for AML with intensive‐timing chemotherapy were classified for the presence of EML (CSF leukemia, CNS‐MS, orbital‐MS, or non‐CNS MS). CSF leukemia was classified as CNS3 ( > 5 WBC in the CSF with blasts) and non‐CSF leukemia as CNS1/2 (<5 WBC in the CSF with or without blasts). Characteristics and outcomes of these patients were compared. Results Of the 1,459 total patients, 1,206 (82%) had no EML, 154 (11%) had CSF leukemia, 19 (1%) had CNS‐MS, 23 (2%) had orbital‐MS, and 57 (4%) had non‐CNS MS. The CR rate was significantly higher in patients with orbital‐MS and CNS‐MS than in those with non‐MS and non‐CNS MS (96% and 95% vs. 78% and 78%, P = 0.034). Patients with orbital‐MS and CNS‐MS had significantly higher overall survival than patients with non‐CNS MS (92% and 73% vs. 38%, P < 0.001), CNS3 patients (92% and 73% vs. 51, P < 0.001), and CNS1/2 patients (92% and 73% vs. 50%, P < 0.001). Patients with orbital‐MS had a significantly lower relapse rate. Conclusion Patients with MS involving orbital and CNS sites had a significantly better survival than patients with non‐CNS MS, with CSF leukemia, or with no EML. Pediatr Blood Cancer 2012; 58: 519–524. © 2011 Wiley Periodicals, Inc.