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Clinical characteristics of pediatric patients with myeloid sarcoma without bone marrow involvement in Japan

Takashi Taga1,2, Toshihiko Imamura3,4, Kentaro Nakashima5

  • 1Leukemia/Lymphoma Committee of Japanese Society of Pediatric Hematology and Oncology, Tokyo, Japan. ttaga@belle.shiga-med.ac.jp.

International Journal of Hematology
|July 5, 2018
PubMed

Insights

Pediatric myeloid sarcoma (MS) without bone marrow involvement is rare in Japan. Children with MS responded well to acute myeloid leukemia (AML) chemotherapy, with most achieving long-term survival.

Area of Science:

  • Hematology
  • Pediatric Oncology
  • Rare Diseases

Background:

  • Myeloid sarcoma (MS) is a rare extramedullary tumor of myeloid blasts.
  • MS is frequently associated with acute myeloid leukemia (AML).
  • Pediatric MS, especially in Japan, is understudied.

Purpose of the Study:

  • To analyze the characteristics and outcomes of pediatric MS without bone marrow involvement in Japan.
  • To evaluate treatment responses and survival rates in this specific patient cohort.

Main Methods:

  • Nationwide retrospective analysis of Japanese children diagnosed with MS (age < 20) between 2000-2013.
  • Inclusion criteria: MS diagnosis without concurrent bone marrow involvement.
  • Data collected on demographics, sites of involvement, karyotypes, treatment, and outcomes.

Main Results:

  • Ten pediatric patients were identified, with a male predominance (8:2) and median age of 4 years.
  • Common sites of involvement included skin, head/neck, and multiple sites.
  • Seven out of ten patients survived disease-free for a median of 93 months after chemotherapy for de novo AML; two received HSCT.

Conclusions:

  • Pediatric MS without bone marrow involvement is a very rare condition in Japan.
  • Patients demonstrated favorable responses to standard AML chemotherapy.
  • Hematopoietic stem cell transplantation (HSCT) in first remission was not universally indicated.
Keywords:
AMLChemotherapyChildrenMyeloid sarcoma

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