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Seminars in Cancer Biology Feb 2020The development of a myeloid neoplasm is a step-wise process that originates from leukemic stem cells (LSC) and includes pre-leukemic stages, overt leukemia and a... (Review)
Review
The development of a myeloid neoplasm is a step-wise process that originates from leukemic stem cells (LSC) and includes pre-leukemic stages, overt leukemia and a drug-resistant terminal phase. Organ-invasion may occur in any stage, but is usually associated with advanced disease and a poor prognosis. Sometimes, extra-medullary organ invasion shows a metastasis-like or even sarcoma-like destructive growth of neoplastic cells in local tissue sites. Examples are myeloid sarcoma, mast cell sarcoma and localized blast phase of chronic myeloid leukemia. So far, little is known about mechanisms underlying re-distribution and extramedullary dissemination of LSC in myeloid neoplasms. In this article, we discuss mechanisms through which LSC can mobilize out of the bone marrow niche, can transmigrate from the blood stream into extramedullary organs, can invade local tissue sites and can potentially create or support the formation of local stem cell niches. In addition, we discuss strategies to interfere with LSC expansion and organ invasion by targeted drug therapies.
Topics: Animals; Biomarkers; Bone Marrow; Cell Communication; Cell Movement; Humans; Immunophenotyping; Leukemia, Myeloid; Neoplasm Staging; Neoplastic Stem Cells; Phenotype; Recurrence; Transendothelial and Transepithelial Migration; Tumor Microenvironment
PubMed: 31408723
DOI: 10.1016/j.semcancer.2019.07.025 -
Frontiers in Pharmacology 2023Treatment of relapsed or refractory acute myeloid leukemia (R/R AML) and myeloid sarcoma (MS) has presented challenges for decades. Studies on selinexor in combination...
Real-world experience with selinexor-containing chemotherapy-free or low-dose chemotherapy regimens for patients with relapsed/refractory acute myeloid leukemia and myeloid sarcoma.
Treatment of relapsed or refractory acute myeloid leukemia (R/R AML) and myeloid sarcoma (MS) has presented challenges for decades. Studies on selinexor in combination with various standard or intensive chemotherapy regimens for the treatment of R/R AML have demonstrated promising results. This study aimed to evaluate the efficacy and safety of chemotherapy-free or low-dose chemotherapy regimens with selinexor for R/R AML and MS patients. Ten patients with R/R AML or MS who received chemotherapy-free or low-dose chemotherapy regimens in combination with selinexor at Tongji Hospital from October 2021 to August 2022 were included in this study. The primary endpoint was overall response rate (ORR) and secondary endpoints included complete remission (CR), CR with incomplete hematological recovery (CRi), partial remission (PR), transplantation rate, and safety. All patients were evaluable for response, achieving CR in four (40.0%) patients and CRi in two (20.0%) patients for a total CR/CRi of 60.0%. The ORR was 80.0% when patients with PR were included. Five (50.0%) patients underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) after treatment with selinexor-containing regimens. At the end of the follow-up, seven (70.0%) patients were alive, and three patients died of transplant-related complications or disease progression. The most frequently reported nonhematologic adverse events (AEs) in patients were grade 1 or 2 asymptomatic hyponatremia. The chemotherapy-free or low-dose chemotherapy regimens in combination with selinexor for R/R AML are feasible and tolerable and provide an opportunity for patients to receive transplantation.
PubMed: 37601075
DOI: 10.3389/fphar.2023.1217701 -
Chinese Clinical Oncology Jun 2020Pain in abdomen has wide differentials and narrowing down the clinical possibilities depends on type of pain, location, characterization which is usually assisted by... (Review)
Review
Pain in abdomen has wide differentials and narrowing down the clinical possibilities depends on type of pain, location, characterization which is usually assisted by imaging studies. Cholecystitis and cholelithiasis are amongst the common causes of acute abdomen. This study reviews the literature for the clinical characteristics, differential diagnosis, treatment and prognosis of reported cases of gallbladder myeloid sarcoma (GB-MS) who presented with abdominal symptoms. A total of 17 cases of GB-MS were studied. The median age was 52 years with age range of 23 to 84 years. All except 1 patient presented with abdominal symptoms. Based on imaging or pathological studies, 3 cases were initially confused with gallbladder lymphoma or cancer. Only 5 patients were treated with AML like chemotherapy. Treatment given included combinations of surgery, chemotherapy, and radiotherapy. None of the cases underwent HSCT for GB-MS. Seven patients were alive till the time of last F/U, 9 succumbed to death while F/U of 1 patient was not available. Irrespective of treatment protocol followed suggesting the poor prognosis in GB-MS cases. In conclusion, acute abdomen complicating blood malignancies is life threatening and can be devastating if not detected and treated in a timely fashion.
Topics: Adult; Aged; Aged, 80 and over; Female; Gallbladder Neoplasms; Humans; Male; Middle Aged; Sarcoma, Myeloid; Young Adult
PubMed: 32434344
DOI: 10.21037/cco-19-250 -
Indian Journal of Hematology & Blood... Dec 2015Myeloid sarcoma is an extramedullary tumor seen most commonly in patients with acute myeloid leukemia and less frequently in chronic myeloid leukemia, myelodysplastic... (Review)
Review
Myeloid sarcoma is an extramedullary tumor seen most commonly in patients with acute myeloid leukemia and less frequently in chronic myeloid leukemia, myelodysplastic syndrome and rarely, in an isolated form without any other underlying malignancy. Malignant pleural effusion in hematological malignancies is rare when compared with solid tumors. We present an unusual case of myeloid sarcoma in which a mediastinal mass with pleural effusion was the initial presentation. A 27 year old gentleman presented with complaints of fever, chest pain and swelling in the anterior chest wall for 6 months. Examination revealed a lump measuring 5 × 5 cm on the left side of the chest wall. Hematological evaluation showed hemoglobin-14.2 g/dL, platelet count-233 × 10(9)/L, TLC-117 × 10(6)/L with normal differential counts. Contrast enhanced computerised tomography (CECT) confirmed the presence of a soft tissue mass in the superior mediastinum abutting against the chest wall. Core biopsy was suggestive of myeloid sarcoma and immunohistochemistry was positive for myeloperoxidase and negative for CD3, CD 20 and CD 23. Pleural fluid analysis showed the presence of malignant cells. Bone marrow examination did not show an excess of blasts. A final diagnosis of extramedullary myeloid sarcoma with malignant pleural effusion was made. The patient was given induction chemotherapy (3 + 7 regimen) with daunorubicin and cytosine arabinoside. Repeat CECT done on day 28 showed complete resolution of pleural effusion and significant reduction in the size of mediastinal mass. The patient has successfully completed three cycles of consolidation therapy following which there has been complete resolution of the mass. He remains asymptomatic on close follow up.
PubMed: 26306072
DOI: 10.1007/s12288-015-0536-z -
Autopsy & Case Reports May 2020Leukemic cells are rarely present in the oral cavity, and there are very few reports regarding such cases. However, we identified some reports of leukemic cells...
Leukemic cells are rarely present in the oral cavity, and there are very few reports regarding such cases. However, we identified some reports of leukemic cells infiltrating tissues in the oral cavity, including gingival involvement. Recurrent painful oral ulcerations and prominent generalized periodontal destruction are the most common oral features of neutrophil disorders, and they may even be the initial symptoms of the disease. The ulcers may affect any part of the oral mucosa, including the tongue and palate. The objective of this report is to describe and discuss a case of myeloid sarcoma in the oral cavity of a 48-year-old male patient.
PubMed: 33344279
DOI: 10.4322/acr.2020.160 -
British Journal of Haematology Mar 2018
Topics: Adult; Aged; Antineoplastic Combined Chemotherapy Protocols; Bone Marrow Neoplasms; Eye Neoplasms; Female; Humans; Leukemia, Myeloid; Magnetic Resonance Imaging; Male; Middle Aged; Young Adult
PubMed: 27879987
DOI: 10.1111/bjh.14430 -
Oncology Letters Jun 2020Myeloid sarcoma (MS) carries a poor prognosis, and information on epigenetic modifications in MS is currently limited. In the present study, 214 ten-eleven...
Myeloid sarcoma (MS) carries a poor prognosis, and information on epigenetic modifications in MS is currently limited. In the present study, 214 ten-eleven translocation-2 () mice were successfully constructed. In addition, 436 patients with myelodysplastic syndrome (MDS) and 354 with acute myeloid leukemia (AML) patients were recruited. The incidence of MS in mice and patients with deficiency was examined, and the efficiency of hypomethylating agents (HMAs) was also be evaluated. A total of 93% of the mice developed myeloid malignancies, 5.5% of which were accompanied by MS (n=11). The survival of these mice ranged between 3 and 25 months. No significant difference was observed between the survival of MS and non-MS mice with loss (P>0.05). In addition, MS cells were transplantable, and their recipients exhibited myeloproliferative characteristics, such as increased white blood cell counts, monocytosis, low erythrocyte counts and hepatosplenomegaly. Their median survival duration was 94.8 days. In the clinical setting, 9.7% of MDS and 11.6% of AML patients with deficiency developed MS, which was higher compared with previous reports (1.5-9.1%). The median age of the MS patients was 44 years old. 5-Aza-2'-deoxycytidine (5-Aza-dC) reduced the incidence of MS in mice, and decitabine was a suitable treatment strategy for MS patients. These data indicate that deficiency plays a key role in MS and its prognostic significance requires further investigation. HMAs may be a useful treatment for MS patients with mutations.
PubMed: 32382331
DOI: 10.3892/ol.2020.11479 -
Cureus Jan 2022Myeloid sarcoma (MS)/granulocytic sarcoma/myeloblastoma/chloroma is a rare extramedullary proliferation of blast cells of one or more myeloid lineages along with the...
Myeloid sarcoma (MS)/granulocytic sarcoma/myeloblastoma/chloroma is a rare extramedullary proliferation of blast cells of one or more myeloid lineages along with the destruction of the normal architecture of adjacent tissue. Isolated MS is a rare entity with an incidence of 0.7 out of 1 million children and 2 out of 1 million adults. Varied clinical presentation, the rarity of the diagnosis, inadequate immunophenotyping, and lack of available literature makes the disease difficult to manage. Here, we report a case of MS in a 44-year-old male with an initial presentation of testicular mass without bone marrow involvement, causing diagnostic challenges. In this case report, we discuss the pathogenesis, diagnostic challenges, and therapeutic options of MS.
PubMed: 35165636
DOI: 10.7759/cureus.21200 -
The British Journal of Radiology Jul 2017Chloroma refers to the extramedullary proliferation of immature myeloid precursors occurring in a gamut of myeloproliferative and myelodysplastic conditions; acute... (Review)
Review
Chloroma refers to the extramedullary proliferation of immature myeloid precursors occurring in a gamut of myeloproliferative and myelodysplastic conditions; acute myeloid leukaemia being the commonest. With non-specific clinical and imaging manifestations, it runs a high risk of misdiagnosis which may significantly affect the outcome of an otherwise treatable lesion. Also with these lesions heralding impending blast crises, awareness of the imaging findings becomes imperative. Imaging not only helps raise the suspicion but also guides further confirmation by demonstration of specific immunohistochemistry markers, ensuring timely institution of chemotherapy. In general, solid enhancing lesions in any haematological disorder could be chloromas, especially if multifocal with mass effect.
Topics: Biomarkers, Tumor; Contrast Media; Diagnosis, Differential; Humans; Immunohistochemistry; Radiopharmaceuticals; Risk Factors; Sarcoma, Myeloid
PubMed: 28445074
DOI: 10.1259/bjr.20160710 -
Hematology Reports May 2018We present an unusual case of myeloid sarcoma with ascites and abdominal pain in which initial clinical, laboratory, and imaging studies suggested a gastrointestinal...
We present an unusual case of myeloid sarcoma with ascites and abdominal pain in which initial clinical, laboratory, and imaging studies suggested a gastrointestinal malignancy or lymphoma. Subsequent detection of leukemic ascites and blasts in a gastric, small bowel, and skin biopsy supported a diagnosis of myeloid sarcoma. Bone marrow biopsy revealed 15% blasts, and cytogenetics with an inversion 16 rearrangement was diagnostic of acute myeloid leukemia (AML). Positron emission tomography-computed tomography performed at presentation to stage a presumptive lymphoma found later utility in following the burden of extramedullary disease. Standard AML induction chemotherapy resulted in complete remission and was followed by three rounds of high dose cytarabine consolidation. The patient unfortunately relapsed leading to re-induction followed by allogeneic stem cell transplantation. This report describes the presentation, assessment, and management of myeloid sarcoma.
PubMed: 30046410
DOI: 10.4081/hr.2018.7040