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

• DOI: 10.3389/fonc.2022.935373
• Publication Date: 2022-07-07T06:23:54Z
• Authors: Magdalena Samborska; Małgorzata Barańska; Jacek Wachowiak; Jolanta Skalska-S...; Sheanda Thambyrajah; Małgorzata Czogała; Walentyna Balwierz; Sylwia Kołtan; Katarzyna Peszyńs...; Mariusz Wysocki; Tomasz Ociepa; Tomasz Urasiński; Grażyna Wróbel; Jadwiga Węcławek-...; Bogna Ukielska; Alicja Chybicka; Anna Kitszel; Maryna Krawczuk-R...; Anna Szmydki-Baran; Iwona Malinowska; Michał Matysiak; Agnieszka Mizia-M...; Renata Tomaszewska; Tomasz Szczepański; Agnieszka Chodała...; Grażyna Karolczyk; Lucyna Maciejka-K...; Ninela Irga-Jaworska; Wanda Badowska; Michał Dopierała; Paweł Kurzawa; Katarzyna Derwich
• Keywords: Myeloid sarcoma

Myeloid sarcoma (MS) is an extramedullary malignant tumor composed of immature myeloid cells. It occurs in patients with acute leukemia (AML), myelodysplastic syndrome (MDS), or chronic (CML). MS may coincide disease diagnosis precede bone marrow involvement by months even years; it can also represent the manifestation a relapse (1, 2).The aim this study to describe clinical characteristics children diagnosed Poland as well analyze diagnostic methods, treatment, and outcomes including overall survival (OS), relapse-free (RFS), event-free (EFS). The attempted identify factors determining treatment outcomes.The group comprised 43 (F=18, M=25) aged 0-18 years (median age, 10.0 mean 8.8 years) based on biopsy immunohistochemistry identification underlying according imaging findings.The data therapeutic methods used were analyzed. A statistical analysis was conducted STATISTICA v. 13 (StatSoft, Inc., Tulsa, OK, USA) curves MedCalc 11.5.1 (MedCalc Software, Ostend, Belgium). Statistical significance considered at p<0.05.In group, most frequently accompanied AML. common site skin, followed orbital region. Skin more age <10 years. frequent genetic abnormality t(8;21)(q22;q22) translocation. 5-year OS probability (pOS), RFS (pRFS), EFS (pEFS) 0.67 ± 0.08, 0.79 0.07, 0.65 respectively. In isolated those concurrent AML/MDS, pOS values 0.56 0.12 0.84 0.09 (p=0.0251), respectively, pEFS 0.82 0.08 (p=0.0247), without translocation, 0.90 0.51 0.14 (p=0.0490), respectively.MS highly variable course. Worse observed compared AML/MDS. Patients translocation found have significantly higher pEFS. location, chemotherapy regimen, surgery, and/or radiotherapy did not significant influence outcomes. Further exploration prognostic indicated.