Clinical Characteristics and Treatment Outcomes of Myeloid Sarcoma in Children: The Experience of the Polish Pediatric Leukemia and Lymphoma Study Group

Magdalena Samborska; Małgorzata Barańska; Jacek Wachowiak; Jolanta Skalska-Sadowska; Sheanda Thambyrajah; Małgorzata Czogała; Walentyna Balwierz; Sylwia Kołtan; Katarzyna Peszyńska-Żelazny; Mariusz Wysocki; Tomasz Ociepa; Tomasz Urasiński; Grażyna Wróbel; Jadwiga Węcławek-Tompol; Bogna Ukielska; Alicja Chybicka; Anna Kitszel; Maryna Krawczuk-Rybak; Anna Szmydki-Baran; Iwona Malinowska; Michał Matysiak; Agnieszka Mizia-Malarz; Renata Tomaszewska; Tomasz Szczepański; Agnieszka Chodała-Grzywacz; Grażyna Karolczyk; Lucyna Maciejka-Kembłowska; Ninela Irga-Jaworska; Wanda Badowska; Michał Dopierała; Paweł Kurzawa; Katarzyna Derwich

doi:10.3389/fonc.2022.935373

Clinical Characteristics and Treatment Outcomes of Myeloid Sarcoma in Children: The Experience of the Polish Pediatric Leukemia and Lymphoma Study Group

Front Oncol. 2022 Jul 7:12:935373. doi: 10.3389/fonc.2022.935373. eCollection 2022.

Authors

Magdalena Samborska¹, Małgorzata Barańska¹, Jacek Wachowiak¹, Jolanta Skalska-Sadowska¹, Sheanda Thambyrajah¹, Małgorzata Czogała², Walentyna Balwierz², Sylwia Kołtan³, Katarzyna Peszyńska-Żelazny³, Mariusz Wysocki³, Tomasz Ociepa⁴, Tomasz Urasiński⁴, Grażyna Wróbel⁵, Jadwiga Węcławek-Tompol⁵, Bogna Ukielska⁵, Alicja Chybicka⁵, Anna Kitszel⁶, Maryna Krawczuk-Rybak⁶, Anna Szmydki-Baran⁷, Iwona Malinowska⁷, Michał Matysiak⁷, Agnieszka Mizia-Malarz⁸, Renata Tomaszewska⁹, Tomasz Szczepański⁹, Agnieszka Chodała-Grzywacz¹⁰, Grażyna Karolczyk¹⁰, Lucyna Maciejka-Kembłowska¹¹, Ninela Irga-Jaworska¹¹, Wanda Badowska¹², Michał Dopierała¹³, Paweł Kurzawa¹³, Katarzyna Derwich¹

Affiliations

¹ Department of Pediatric Oncology, Hematology and Transplantology, Poznań University of Medical Sciences, Poznań, Poland.
² Department of Pediatric Oncology and Hematology, University Children's Hospital, Kraków, Poland.
³ Department of Paediatrics, Hematology and Oncology, Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University in Toruń, Toruń, Poland.
⁴ Department of Pediatrics, Hemato-oncology and Pediatric Gastroenterology, Independent Public Clinical Hospital No. 1, Pomeranian Medical University, Szczecin, Poland.
⁵ Department of Bone Marrow Transplantation, Pediatric Oncology and Hematology, Supraregional Center of Pediatric Oncology "Cape of Hope", Wrocław, Poland.
⁶ Department of Pediatrics, Oncology and Hematology, L. Children's Clinical Hospital, Białystok, Poland.
⁷ Department of Pediatric Oncology and Hematology, Independent Public Children's Teaching Hospital, Warsaw, Poland.
⁸ Department of Oncology, Hematology and Chemotherapy, John Paul II Upper Silesian Child Health Centre, Katowice, Poland.
⁹ Department of Pediatrics, Pediatric Hematology and Oncology in Zabrze, Stanisław Szyszko Independent Public University Hospital No. 1, Medical University of Silesia, Katowice, Poland.
¹⁰ Department of Pediatric Oncology and Hematology, Provincial Integrated Hospital, Kielce, Poland.
¹¹ Department of Pediatrics, Hematology and Oncology, University Clinical Centre, Gdańsk, Poland.
¹² Department of Pediatric Oncology and Hematology, Provincial Specialist Children's Hospital, Olsztyn, Poland.
¹³ Department of Clinical Pathology, Poznań University of Medical Sciences, Poznań, Poland.

Abstract

Introduction: Myeloid sarcoma (MS) is an extramedullary malignant tumor composed of immature myeloid cells. It occurs in patients with acute myeloid leukemia (AML), myelodysplastic syndrome (MDS), or chronic myeloid leukemia (CML). MS may coincide with disease diagnosis or precede bone marrow involvement by months or even years; it can also represent the extramedullary manifestation of a relapse (1, 2).

Aim: The aim of this study is to describe clinical characteristics of children diagnosed with MS in Poland as well as to analyze diagnostic methods, treatment, and outcomes including overall survival (OS), relapse-free survival (RFS), and event-free survival (EFS). The study also attempted to identify factors determining treatment outcomes.

Patients: The study group comprised 43 patients (F=18, M=25) aged 0-18 years (median age, 10.0 years; mean age, 8.8 years) diagnosed with MS based on tumor biopsy and immunohistochemistry or identification of underlying bone marrow disease and extramedullary tumor according to imaging findings.

Methods: The clinical data and diagnostic and therapeutic methods used in the study group were analyzed. A statistical analysis of the treatment outcomes was conducted with STATISTICA v. 13 (StatSoft, Inc., Tulsa, OK, USA) and analysis of survival curves was conducted with MedCalc 11.5.1 (MedCalc Software, Ostend, Belgium). Statistical significance was considered at p<0.05.

Results: In the study group, MS was most frequently accompanied by AML. The most common site of involvement was skin, followed by orbital region. Skin manifestation of MS was more common in the age group <10 years. The most frequent genetic abnormality was the t(8;21)(q22;q22) translocation. The 5-year OS probability (pOS), 5-year RFS probability (pRFS), and 5-year EFS probability (pEFS) were 0.67 ± 0.08, 0.79 ± 0.07, and 0.65 ± 0.08, respectively. In patients with isolated MS and those with concurrent bone marrow involvement by AML/MDS, pOS values were 0.56 ± 0.12 and 0.84 ± 0.09 (p=0.0251), respectively, and pEFS values were 0.56 ± 0.12 and 0.82 ± 0.08 (p=0.0247), respectively. In patients with and without the t(8;21)(q22;q22) translocation, pEFS values were 0.90 ± 0.09 and 0.51 ± 0.14 (p=0.0490), respectively.

Conclusions: MS is a disease with a highly variable clinical course. Worse treatment outcomes were observed in patients with isolated MS compared to those with concurrent bone marrow involvement by AML/MDS. Patients with the t(8;21)(q22;q22) translocation were found to have significantly higher pEFS. MS location, age group, chemotherapy regimen, surgery, and/or radiotherapy did not have a significant influence on treatment outcomes. Further exploration of prognostic factors in children with MS is indicated.

Keywords: acute myeloid leukemia; children; myeloid sarcoma; prognosis; treatment.

Copyright © 2022 Samborska, Barańska, Wachowiak, Skalska-Sadowska, Thambyrajah, Czogała, Balwierz, Kołtan, Peszyńska-Żelazny, Wysocki, Ociepa, Urasiński, Wróbel, Węcławek-Tompol, Ukielska, Chybicka, Kitszel, Krawczuk-Rybak, Szmydki-Baran, Malinowska, Matysiak, Mizia-Malarz, Tomaszewska, Szczepański, Chodała-Grzywacz, Karolczyk, Maciejka-Kembłowska, Irga-Jaworska, Badowska, Dopierała, Kurzawa and Derwich.