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• W4385655228 abstract "Topic: 4. Acute myeloid leukemia - Clinical Background: Myeloid sarcoma (MS), also known as granulocytic sarcoma (GS), is a rare tumor mass composed of myeloid blasts that occurs outside of the bone marrow. It can be isolated, but often associated with acute myeloid leukemia (AML).The incidence of MS is higher in pediatric population and it may affect any organ, leading to various clinical presentations. Aims: The objective of this study is to report the clinical features, cytogenetic and molecular characteristics, therapeutic approaches and outcomes of pediatric MS to improve understanding of the disease. Methods: This was a retrospective study that evaluated a series of 9 children diagnosed with MS in hematology pediatric department of Aziza Othmana hospital from January 2006 to December 2022. Results: All patients were Class I-MS given its association with AML (according to the WHO). Among them, 5 patients were LAM2 (56%), 3 patients were LAM5 (33%) and 1 patient was LAM7 (11%). The sex ratio was 2 and the median age at diagnosis was 10 years [2:17]. Organs that were commonly involved in this series were cranial and endocranial sites with extension to the orbital region in 5 cases (56%) and spinal canal in 4 cases (44%). Symptoms of orbital masses were proptosis (4 cases), facial nerve paralysis (1 case), ptosis (1 case) and canthus tumefaction (1 case). Spinal canal myeloid sarcoma was revealed by spinal canal compression in 4 cases. The initial diagnosis was based on Magnetic Resonance Imaging in 6 cases (66%) and only 3 patients were diagnosed by mass biopsy. 8 patients had t(8,21) in karyotype. 6 of them had fusion genes AML1/ETO on molecular testing, cytogenetic and molecular evaluation were not available in other patients. All patients received chemotherapy according to ELAM02 protocol. Only 3 patients received local therapy: 2 decompressive laminectomy and 1 decompressive radiotherapy. All patients had cytologic complete remission of AML in post induction evaluation. However, MS had totally disappeared in 8 patients and one patient had partial regression in post induction evaluation. Prophylactic lumbar puncture was performed for all patients. 3 patients were treated with Allo Hematopoietic Stem Cell Transplantation (HSHCT). Therapy related mortality was 22%: 1 patient died of septic shock during third consolidation, the other one died of toxicity following HSHCT. The 5-year overall survival rate and the 5-year event free-survival rate were 45%.The 5-year free relapse survival rate was 50%. Summary/Conclusion Due to its low incidence, MS is a challenging diagnostic and therapeutic disease and most descriptions of the pathology are case reports. Patients with myeloid sarcoma have usually an unfavorable outcomes. Keywords: Children, Acute myeloid leukemia" @default.
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• W4385655228 date "2023-08-01" @default.
• W4385655228 modified "2023-09-27" @default.
• W4385655228 title "PB1878: MYELOID SARCOMA IN CHILDREN: ABOUT 9 CASES" @default.
• W4385655228 doi "https://doi.org/10.1097/01.hs9.0000974340.89719.df" @default.
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