Superior outcome of pediatric acute myeloid leukemia patients with orbital and CNS myeloid sarcoma: a report from the Children's Oncology Group

Pediatr Blood Cancer. 2012 Apr;58(4):519-24. doi: 10.1002/pbc.23201. Epub 2011 May 25.

Abstract

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.

Publication types

  • Clinical Trial
  • Comparative Study
  • Multicenter Study
  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage*
  • Antineoplastic Combined Chemotherapy Protocols / adverse effects
  • Central Nervous System Neoplasms* / classification
  • Central Nervous System Neoplasms* / drug therapy
  • Central Nervous System Neoplasms* / mortality
  • Child
  • Child, Preschool
  • Disease-Free Survival
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Leukemia, Myeloid, Acute* / classification
  • Leukemia, Myeloid, Acute* / drug therapy
  • Leukemia, Myeloid, Acute* / mortality
  • Male
  • Middle Aged
  • Neoplasms, Second Primary* / classification
  • Neoplasms, Second Primary* / drug therapy
  • Neoplasms, Second Primary* / mortality
  • Orbital Neoplasms* / classification
  • Orbital Neoplasms* / drug therapy
  • Orbital Neoplasms* / mortality
  • Sarcoma, Myeloid* / classification
  • Sarcoma, Myeloid* / drug therapy
  • Sarcoma, Myeloid* / mortality
  • Survival Rate