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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 -
Nature Medicine Oct 2018Impaired immunity in patients with late-stage cancer is not limited to antitumor responses, as demonstrated by poor vaccination protection and high susceptibility to...
Impaired immunity in patients with late-stage cancer is not limited to antitumor responses, as demonstrated by poor vaccination protection and high susceptibility to infection. This has been largely attributed to chemotherapy-induced impairment of innate immunity, such as neutropenia, whereas systemic effects of tumors on hematopoiesis and adoptive immunity remain incompletely understood. Here we observed anemia associated with severe deficiency of CD8 T cell responses against pathogens in treatment-naive mice bearing large tumors. Specifically, we identify CD45 erythroid progenitor cells (CD71TER119; EPCs) as robust immunosuppressors. CD45 EPCs, induced by tumor growth-associated extramedullary hematopoiesis, accumulate in the spleen to become a major population, outnumbering regulatory T cells (Ts) and myeloid-derived suppressor cells (MDSCs). The CD45 EPC transcriptome closely resembles that of MDSCs, and, like MDSCs, reactive oxygen species production is a major mechanism underlying CD45 EPC-mediated immunosuppression. Similarly, an immunosuppressive CD45 EPC population was detected in patients with cancer who have anemia. These findings identify a major population of immunosuppressive cells that likely contributes to the impaired T cell responses commonly observed in patients with advanced cancer.
Topics: Anemia; Animals; Antigens, CD; CD8-Positive T-Lymphocytes; Disease Models, Animal; Erythroid Precursor Cells; Humans; Immune Tolerance; Immunity, Innate; Leukocyte Common Antigens; Mice; Myeloid-Derived Suppressor Cells; Neoplasm Staging; Reactive Oxygen Species; Receptors, Transferrin; Sarcoma, Myeloid; T-Lymphocytes, Regulatory; Xenograft Model Antitumor Assays
PubMed: 30297899
DOI: 10.1038/s41591-018-0205-5 -
Cureus Jun 2020Myeloid sarcoma is an isolated extramedullary tumor mass consisting of immature myeloid cells. It is characterized by highly variable outcomes and usually disrupts the...
Myeloid sarcoma is an isolated extramedullary tumor mass consisting of immature myeloid cells. It is characterized by highly variable outcomes and usually disrupts the normal architecture of the normal tissue in which it originates. It may occur de novo or be associated with other hematological malignancies. Clinical presentation of myeloid sarcomas can be highly variable based on the tumor site, size, and extent of tissue involvement. The diagnosis of myeloid sarcoma is challenging and requires a high index of suspicion. Tissue sampling followed by the use of auxiliary studies is essential for diagnosis. Moreover, bone marrow sampling is necessary to exclude morrow involvement. Currently, the recommended therapeutic regimens for myeloid sarcoma are similar to those for acute myeloid leukemia. Much work remains to be accomplished as myeloid sarcomas, if initially missed or misdiagnosed, have poor overall survival rates. Furthermore, prognostic factors for this malignancy remain poorly understood.
PubMed: 32528784
DOI: 10.7759/cureus.8462 -
Internal Medicine (Tokyo, Japan) 2020
PubMed: 32115522
DOI: 10.2169/internalmedicine.3560-19 -
Mediterranean Journal of Hematology and... 2021Myeloid sarcomas can be detected in up to 30% of acute myeloid leukemia cases or occur de-novo without bone marrow involvement. The most frequent localization of myeloid... (Review)
Review
Myeloid sarcomas can be detected in up to 30% of acute myeloid leukemia cases or occur de-novo without bone marrow involvement. The most frequent localization of myeloid sarcomas in the abdominal cavity is the small intestine, and gastric presentations are infrequent, frequently misdiagnosed, and a high level of suspicion should exist when the characteristic histomorphology features are present. The current review features a case report with gastric presentation of myeloid sarcoma in a patient with a diagnosis of acute myeloid leukemia with trisomy 8. In addition, a review of the literature of intestinal-type myeloid sarcomas shows that less than 15% of these cases have been reported in the stomach. The most common molecular aberrancy detected in intestinal myeloid sarcomas is the fusion protein CBFB-MYH11. A review of several large studies demonstrates that the presence of myeloid sarcoma does not constitute an independent prognostic factor. The therapeutic approach will be tailored to the specific genetic abnormalities present, and systemic chemotherapy with hematopoietic stem cell transplant is the most efficient strategy.
PubMed: 34804441
DOI: 10.4084/MJHID.2021.067 -
Leukemia Research Reports 2019Myeloid Sarcoma (MS) is diagnosed by an extramedullary proliferation of immature granulocytic cells. Its association with chronic myeloid leukemia (CML) is rare. CML is...
Myeloid Sarcoma (MS) is diagnosed by an extramedullary proliferation of immature granulocytic cells. Its association with chronic myeloid leukemia (CML) is rare. CML is characterized by gene rearrangement and therapies with tyrosine kinase inhibitors (TKI) are very effective. However, TKI resistance may occur secondary to the development of mutations. T315I is a common mutation that accounts for ∼20% clinical resistance to TKIs. We report the first case of a patient with T315I mutated myeloid sarcoma that occurred after complete cytogenetic response with dasatinib of a chronic phase CML. The patient was successfully treated with induction chemotherapy and ponatinib.
PubMed: 31485411
DOI: 10.1016/j.lrr.2019.100184 -
European Journal of Case Reports in... 2022A myeloid sarcoma is an extramedullary tumour arising from infiltration by leukemic cells at an anatomic site other than the bone marrow. Most commonly it precedes acute...
UNLABELLED
A myeloid sarcoma is an extramedullary tumour arising from infiltration by leukemic cells at an anatomic site other than the bone marrow. Most commonly it precedes acute myeloid leukaemia but occasionally occurs simultaneously. It may also be associated with myeloproliferative neoplasms, myelodysplastic syndrome and the blast phase of chronic myeloid leukaemia. The most common sites for extramedullary tumours are bone, periosteum, soft tissue, lymph node and skin. Although this disease can affect a wide range of body sites, there are very few reports of peritoneal myeloid sarcoma or cavity effusion. The authors present the case of a 68-year-old man with myelodysplasia-related acute myeloid leukaemia and peritoneal myeloid sarcoma with myeloid ascites. The definitive diagnosis is challenging, requires a high level of suspicion, and relies on the exclusion of all alternative diagnoses and especially on complementary tests such as flow cytometry and immunohistochemistry analysis of ascitic fluid in order to detect the immature myeloid cells.
LEARNING POINTS
Myeloid sarcomas are extramedullary leukemic tumours that occur before or simultaneously with acute myeloid leukaemia, other myeloproliferative neoplasms or myelodysplastic syndrome.Myeloid sarcomas are most often seen in bone, soft tissue, lymph node and skin, but can present in most locations.Peritoneal myeloid sarcoma and leukemic ascites, although very rare, must be searched for when a patient with acute leukaemia presents with newly diagnosed ascites, through ascitic fluid flow cytometry and immunophenotypic analysis.
PubMed: 35265554
DOI: 10.12890/2022_003184 -
Proceedings (Baylor University. Medical... 2022Myeloid sarcoma is a tumor mass of immature myeloid or monocytic cells (rarely erythroid or megakaryocytic) occurring in an extramedullary site. A de novo promyelocytic...
Myeloid sarcoma is a tumor mass of immature myeloid or monocytic cells (rarely erythroid or megakaryocytic) occurring in an extramedullary site. A de novo promyelocytic granulocytic sarcoma is a very rare tumor. We report a case of a young man presenting with a paraspinal myeloid sarcoma of promyelocytic origin.
PubMed: 35261471
DOI: 10.1080/08998280.2021.1995108 -
Journal of Clinical and Diagnostic... Mar 2017Granulocytic Sarcomas (GS) also called as Myeloid Sarcomas (MS) or chloromas are the representatives of extramedullary infiltrates of immature myeloid cells including... (Review)
Review
Granulocytic Sarcomas (GS) also called as Myeloid Sarcomas (MS) or chloromas are the representatives of extramedullary infiltrates of immature myeloid cells including myeloblasts, promyelocytes and myelocytes. Primary cardiac malignancies per se are rare and infiltration of cardiac muscles by secondary malignant cells is also an uncommon finding. Out of these cardiac tumors, contribution of Cardiac Myeloid Sarcoma (CMS) is even more smaller thereby limiting our knowledge about this rare entity. Because of its very lower incidence, an exact guideline for diagnosis and management is still missing and usually haematologists around the world are treating CMS based on their clinical acumen. Aim of this review is to briefly discuss the presenting clinical feature, differential diagnosis, diagnostic workup and management based on published articles related to CMS till date.
PubMed: 28511492
DOI: 10.7860/JCDR/2017/23241.9499 -
Contemporary Oncology (Poznan, Poland) 2016Myeloid sarcoma (MS) is a malignant extramedullary tumour, which consists of immature cells of myeloid origin. It may occur , concurrently or precede the diagnosis of... (Review)
Review
Myeloid sarcoma (MS) is a malignant extramedullary tumour, which consists of immature cells of myeloid origin. It may occur , concurrently or precede the diagnosis of acute myeloid leukemia (AML), myelodysplastic syndrome (MDS) or chronic myeloid leukemia (CML). MS can also be a manifestation of the relapse of the disease. The more frequent sites of involvement are the skin, orbit, bone, periosteum, lymph nodes, gastrointestinal tract, soft tissue, central nervous system and testis. Because of its different localization and symptoms, and the lack of diagnostics algorithm, myeloid sarcoma is a real diagnostic challenge, in particular in patients without initial bone marrow involvement. The correct diagnosis of MS is important for adequate therapy, which is often delayed because of a high misdiagnosis rate. In the paper, the role of immunohistochemistry, cytogenetic and molecular genetic analyses is emphasized as well as the breadth of unclear aspects of this disorder in children.
PubMed: 28239280
DOI: 10.5114/wo.2016.65602