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Clinical Lymphoma, Myeloma & Leukemia May 2017Myeloid sarcoma is an extramedullary tumor of immature granulocytic cells. It is a rare condition, most often associated with acute myeloid leukemia (AML), although in... (Review)
Review
Myeloid sarcoma is an extramedullary tumor of immature granulocytic cells. It is a rare condition, most often associated with acute myeloid leukemia (AML), although in some rare cases it may present in nonleukemic patients. It should therefore be considered as a differential diagnosis of any atypical cellular infiltrate. It may occur at any site, leading to very varied clinical presentations. Diagnosis is challenging and relies on a high index of suspicion as well as radiology, histology, immunophenotyping, and molecular analyses, which also are essential for risk stratification and treatment planning. Systemic chemotherapy using AML-like regimens should be commenced early, even in nonleukemic disease. Surgery and/or radiotherapy may be indicated for symptomatic lesions or tumors causing local organ dysfunction or obstruction. Allogeneic hematopoietic stem cell transplantation has demonstrated promising results, particularly in patients who achieved complete remission with AML-induction protocols, and recent advances in genetic profiling may enable the development of novel targeted therapies. Prospective multicenter controlled trials are required to further refine management decisions and investigate the role of novel targeted therapies.
Topics: Antineoplastic Agents; Hematopoietic Stem Cell Transplantation; Humans; Sarcoma, Myeloid
PubMed: 28342811
DOI: 10.1016/j.clml.2017.02.027 -
Oncology Research and Treatment 2019Hematological malignancies can manifest as extramedullary soft tissue masses in relatively rare cases. The rarity of it causes a diagnostic and therapeutic challenge.... (Review)
Review
Hematological malignancies can manifest as extramedullary soft tissue masses in relatively rare cases. The rarity of it causes a diagnostic and therapeutic challenge. One of the rarest manifestations is myeloid sarcoma (MS). MS develops as part of acute myeloid leukemia, myeloproliferative neoplasm, or myelodysplastic syndrome or at relapse, especially following allogeneic hematopoietic stem cell transplant. The tumor displays high myeloperoxidase expression, hence the color green, and is called chloroma. It most commonly appears in lymph nodes, skin and soft tissues, bone, testes, gastrointestinal tract, and peritoneum. Immunohistochemistry shows CD68-KP1 as the most commonly expressed marker, then myeloperoxidase, CD117, CD99, CD68/PG-M1, lysozyme, CD34, terminal deoxynucleotidyl transferase, CD56, CD61, CD30, glycophorin A, and CD4. Different chromosomal abnormalities including MLL rearrangement, t(8; 21), monosomy 7, trisomy 8, trisomy 11, trisomy 4, inversion (16), monosomy 16,16q deletion, 5q deletion, and 20q deletion were reported. Most of the literature about MS are case reports and small retrospective studies, thus there is limited clinical knowledge of the cases and their presentation and management plans. Here, we provide a review of what has been reported in the literature about MS in the light of our experiences.
Topics: Female; Hematopoietic Stem Cell Transplantation; Humans; Immunohistochemistry; Leukemia, Myeloid, Acute; Male; Sarcoma, Myeloid
PubMed: 30840960
DOI: 10.1159/000497210 -
Seminars in Diagnostic Pathology May 2023Myeloid Sarcoma (MS) is a high grade, hematological malignancy defined as an extramedullary tumor mass of myeloid blasts with or without maturation that effaces tissue... (Review)
Review
Myeloid Sarcoma (MS) is a high grade, hematological malignancy defined as an extramedullary tumor mass of myeloid blasts with or without maturation that effaces tissue architecture. It is a highly heterogenous condition that represents a variety of myeloid neoplasms. This heterogeneity of MS, together with its rarity, have greatly hampered our understanding of the condition. Diagnosis requires tumor biopsy, which should be accompanied by bone marrow evaluation for medullary disease. It is presently recommended that MS be treated similar to AML. Additionally, ablative radiotherapy and novel targeted therapies may also be beneficial. Genetic profiling has identified recurrent genetic abnormalities including gene mutations associated with MS, supporting its etiology similar to AML. However, the mechanisms by which MS homes to specific organs is unclear. This review provides an overview of pathogenesis, pathological and genetic findings, treatment, and prognosis. Improving the management and outcomes of MS patients requires a better understanding of its pathogenesis and its response to various therapeutic approaches.
Topics: Humans; Sarcoma, Myeloid; Prognosis; Myeloproliferative Disorders; Mutation; Leukemia, Myeloid, Acute
PubMed: 37149396
DOI: 10.1053/j.semdp.2023.04.009 -
Annals of Hematology Aug 2023Myeloid sarcoma (MS) is a distinct entity among myeloid neoplasms defined as a tumour mass of myeloid blasts occurring at an anatomical site other than the bone marrow,... (Review)
Review
Myeloid sarcoma (MS) is a distinct entity among myeloid neoplasms defined as a tumour mass of myeloid blasts occurring at an anatomical site other than the bone marrow, in most cases concomitant with acute myeloid leukaemia (AML), rarely without bone marrow involvement. MS may also represent the blast phase of chronic myeloproliferative neoplasms (MPN) and myelodysplastic syndromes (MDS). However, the clinical and molecular heterogeneity of AML, as highlighted by the 2022 World Health Organization (WHO) and International Consensus (ICC) classifications, indirectly define MS more as a set of heterogeneous and proteiform diseases, rather than a homogeneous single entity. Diagnosis is challenging and relies mainly on histopathology, immunohistochemistry, and imaging. Molecular and cytogenetic analysis of MS tissue, particularly in isolated cases, should be performed to refine the diagnosis, and thus assign prognosis guiding treatment decisions. If feasible, systemic therapies used in AML remission induction should be employed, even in isolated MS. Role and type of consolidation therapy are not univocally acknowledged, and systemic therapies, radiotherapy, or allogeneic hematopoietic stem cell transplantation (allo-HSCT) should be considered. In the present review, we discuss recent information on MS, focusing on diagnosis, molecular findings, and treatments also considering targetable mutations by recently approved AML drugs.
Topics: Humans; Sarcoma, Myeloid; Leukemia, Myeloid, Acute; Myelodysplastic Syndromes; Myeloproliferative Disorders; Hematopoietic Stem Cell Transplantation
PubMed: 37286874
DOI: 10.1007/s00277-023-05288-1 -
RoFo : Fortschritte Auf Dem Gebiete Der... Jan 2021
Review
Topics: Contrast Media; Diagnosis, Differential; Humans; Sarcoma, Myeloid
PubMed: 32353885
DOI: 10.1055/a-1150-8131 -
Journal of Computer Assisted TomographyMyeloid sarcoma (MS) is a rare extramedullary neoplasm that can present in association with acute myeloid leukemia, most commonly in children younger than 15 years. This... (Review)
Review
Myeloid sarcoma (MS) is a rare extramedullary neoplasm that can present in association with acute myeloid leukemia, most commonly in children younger than 15 years. This unique extramedullary malignancy may involve a variety of different organ systems and can present following, preceding, simultaneous with, or in insolation to acute myeloid leukemia. Common areas of extramedullary involvement include soft tissues, bones, lymph nodes, and the peritoneum. Imaging plays a critical role in the diagnosis and management of MS, with commonly used modalities including positron emission tomography-computed tomography, magnetic resonance imaging, computerized tomography, and ultrasound. The purpose of this review article is to provide radiologists with a comprehensive guide summarizing the relevant imaging and clinical features of MS, with emphasis on the role of imaging in the diagnosis, treatment, and follow-up of patients with MS. The relevant pathophysiology, epidemiology, clinical presentations, and differential diagnosis of MS will be reviewed. The relevance of different imaging modalities in diagnosis, monitoring of treatment response, and assessment of treatment-related complications will also be outlined. Through summarizing these topics, this review article aims to provide radiologists with a guide for understanding the existing knowledge of MS in the literature and the current role of imaging in the management of this unique malignancy.
Topics: Child; Humans; Sarcoma, Myeloid; Leukemia, Myeloid, Acute; Tomography, X-Ray Computed; Magnetic Resonance Imaging; Radiologists
PubMed: 37185013
DOI: 10.1097/RCT.0000000000001440 -
Blood Reviews May 2021The World Health Organization classification and definition of "myeloid sarcoma" is imprecise and misleading. A more accurate term is "extramedullary acute myeloid... (Review)
Review
The World Health Organization classification and definition of "myeloid sarcoma" is imprecise and misleading. A more accurate term is "extramedullary acute myeloid leukemia tumor (eAML)." The pathogenesis of eAML has been associated with aberrancy of cellular adhesion molecules, chemokine receptors/ligands and RAS-MAPK/ERK signaling. eAML can present with or without synchronous or metachronous intramedullary acute myeloid leukemia (AML) so a bone marrow evaluation is always recommended. Accurate diagnosis of eAML requires tissue biopsy. eAML confined to one or a few sites is frequently treated with local therapy such as radiotherapy. About 75-90% of patients with isolated eAML will develop metachronous intramedullary AML with a median latency period ranging from 4 to 12 months; thus, patients with isolated eAML may also be treated with systemic anti-leukemia therapy. eAML does not appear to have an independent prognostic impact; selection of post-remission therapy including allogeneic hematopoietic cell transplant (alloHCT) is typically guided by intramedullary disease risk. Management of isolated eAML should be individualized based on patient characteristics as well as eAML location and cytogenetic/molecular features. The role of PET/CT in eAML is also currently being elucidated. Improving outcomes of patients with eAML requires further knowledge of its etiology and mechanism(s) as well as therapeutic approaches beyond conventional chemotherapy, ideally in the context of controlled trials.
Topics: Allografts; Hematopoietic Stem Cell Transplantation; Humans; Leukemia, Myeloid, Acute; MAP Kinase Signaling System; Neoplasm Proteins; Positron Emission Tomography Computed Tomography; Radiotherapy; Sarcoma, Myeloid
PubMed: 33213985
DOI: 10.1016/j.blre.2020.100773 -
Journal of Breast Imaging Jul 2023Myeloid sarcoma (MS) is a rare extramedullary solid tumor arising most often in patients with current or subsequent acute myeloid leukemia (AML). Patients of all ages... (Review)
Review
Myeloid sarcoma (MS) is a rare extramedullary solid tumor arising most often in patients with current or subsequent acute myeloid leukemia (AML). Patients of all ages may present with involvement of the skin, lymph nodes, intestinal tract, bone, and/or central nervous system. Isolated involvement of the breast is rare, and only a small number of cases have been described in the literature. Breast MS may present as a palpable mass on clinical evaluation. In this broad literature review from 2010 to 2022, the most common findings on mammography are either solitary or multiple masses, followed by architectural distortion and, less commonly, no discrete findings. Sonography may demonstrate hypoechoic or mixed echogenicity mass(es) with circumscribed or indistinct, not discrete margins. Myeloid sarcoma may present as an enhancing mass or nonmass enhancement on breast MRI and is typically moderately radiotracer avid on 18F-fluorodeoxyglucose-PET. At histopathology, MS is characterized by myeloid blasts in varying stages of granulocytic or neutrophilic maturation; diagnosis typically requires immunophenotyping. There is no consensus for treatment of MS, although systemic chemotherapy for AML is often used as MS is considered the tissue equivalent of AML. This article will discuss and illustrate imaging and pathology findings when the breast is involved by MS.
Topics: Female; Humans; Breast Neoplasms; Leukemia, Myeloid, Acute; Magnetic Resonance Imaging; Mammography; Sarcoma, Myeloid
PubMed: 38416899
DOI: 10.1093/jbi/wbad019 -
International Journal of Hematology Dec 2023Myeloid sarcoma is a rare clinical entity that presents as an isolated proliferation of leukemic cells, concurrently with or at relapse of acute myeloid leukemia (AML),... (Review)
Review
Myeloid sarcoma is a rare clinical entity that presents as an isolated proliferation of leukemic cells, concurrently with or at relapse of acute myeloid leukemia (AML), myelodysplastic syndromes/neoplasms (MDS), chronic myeloid leukemia (CML), and myeloproliferative neoplasm (MPN). Myeloid sarcoma disrupts the normal architecture of its surrounding tissues. When it forms in long bones, it can cause their pathological fracture. We recently experienced a rare case of MDS presenting with myeloid sarcoma in the femur that eventually resulted in its pathological fracture. Detailed chromosomal analysis of the bone marrow cells suggested emergence of myeloid sarcoma during the fast-paced progression of MDS just after acquiring trisomy 22. A comprehensive review of previous cases of myeloid sarcoma-associated pathological fracture indicated possible involvement of structural rearrangements of chromosomes 9 and 22. Management of myeloid sarcoma should continue to improve, and clinicians should note that myeloid sarcoma with specific chromosomal alterations needs extra medical attention to prevent pathological fracture.
Topics: Humans; Sarcoma, Myeloid; Fractures, Spontaneous; Myeloproliferative Disorders; Myelodysplastic Syndromes; Leukemia, Myeloid, Acute
PubMed: 37707761
DOI: 10.1007/s12185-023-03656-1 -
Diagnostic Cytopathology Nov 2022
Topics: Cell Biology; Humans; Sarcoma, Myeloid
PubMed: 35960136
DOI: 10.1002/dc.25034