-
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 -
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 -
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 -
Frontiers in Pediatrics 2022Myeloid sarcoma (MS) is a rare extramedullary mass with myeloid expression, which is easy to be missed and misdiagnosed, especially in the pediatric population. We...
PURPOSE
Myeloid sarcoma (MS) is a rare extramedullary mass with myeloid expression, which is easy to be missed and misdiagnosed, especially in the pediatric population. We analyze the clinicopathological characteristics, immunophenotypic, cytogenetic, and molecular studies, therapeutic approaches, and outcomes, to optimize the management of such patients.
METHODS
A retrospective, single-center, case series study of eleven children diagnosed with MS by pathology was performed.
RESULTS
The male-to-female ratio was 8:3, and the median age at diagnosis was 7 years. The most commonly involved sites were the skin and orbital region, followed by lymph nodes, central nervous system, and testis. Seven cases (64%) with -MS and four cases (36%) presented as -MS. Immunohistochemically, MPO and CD117 were the most commonly expressed markers, followed by CD33, CD43, CD34, CD68, and lysozyme. Chromosomal abnormalities were detected in 4 patients. Two patients had the presence of deleterious mutations (FLT3, ASXL, KIT, and DHX15) on molecular detection. Ten patients were treated with chemotherapy based on AML regimens. The median follow-up time was 33.5 months in eleven patients. Two patients relapsed, one died, and one lost to follow-up. The 2-year overall survival (OS) rate estimated by Kaplan-Meier curves was 90.9% ± 8.7%, and the event-free survival (EFS) rate was 64.9% ± 16.7%.
CONCLUSIONS
MS diagnosis is usually challenging. Adequate tumor biopsy and expanded immunohistochemistry are necessary for the correct diagnosis of MS. Early and regular systemic chemotherapy promises long-term survival.
PubMed: 36545668
DOI: 10.3389/fped.2022.927894 -
South Asian Journal of Cancer Dec 2021Myeloid sarcoma (MS) is a malignant extramedullary tumor consisting of immature cells of myeloid origin. It may precede, present concurrently or follow acute myeloid...
Myeloid sarcoma (MS) is a malignant extramedullary tumor consisting of immature cells of myeloid origin. It may precede, present concurrently or follow acute myeloid leukemia (AML) in de novo case or may also be present and might be the only manifestation of recurrent AML, myelodysplastic syndrome, or chronic myeloid leukemia. It frequently involves skin, orbit, bone, periosteum, lymph nodes, and gastrointestinal tract, soft tissue, central nervous system, and testis. Because of its different localization and symptoms, and the lack of diagnostic algorithm, MS is a real diagnostic challenge particularly in patients without initial bone marrow involvement. The correct diagnosis of MS is important for optimum therapy, which is often delayed because of a high misdiagnosis rate. We reported three cases of MS derived from spine presented with back pain, paraplegia, paraparesis, respectively, and reviewed the relevant literature.
PubMed: 34984205
DOI: 10.1055/s-0041-1742079 -
Cancers Mar 2023-mutant cancers are frequent, metastatic, lethal, and largely undruggable. While interleukin (IL)-1β and nuclear factor (NF)-κB inhibition hold promise against cancer,...
-mutant cancers are frequent, metastatic, lethal, and largely undruggable. While interleukin (IL)-1β and nuclear factor (NF)-κB inhibition hold promise against cancer, untargeted treatments are not effective. Here, we show that human -mutant cancers are addicted to IL-1β via inflammatory versican signaling to macrophage inhibitor of NF-κB kinase (IKK) β. Human pan-cancer and experimental NF-κB reporter, transcriptome, and proteome screens reveal that -mutant tumors trigger macrophage IKKβ activation and IL-1β release via secretory versican. Tumor-specific versican silencing and macrophage-restricted IKKβ deletion prevents myeloid NF-κB activation and metastasis. Versican and IKKβ are mutually addicted and/or overexpressed in human cancers and possess diagnostic and prognostic power. Non-oncogene /IL-1β addiction is abolished by IL-1β and TLR1/2 inhibition, indicating cardinal and actionable roles for versican and IKKβ in metastasis.
PubMed: 36980752
DOI: 10.3390/cancers15061866 -
Life Science Alliance Dec 2023The tumor microenvironment is a dynamic network of stromal, cancer, and immune cells that interact and compete for resources. We have previously identified the Vanin1...
The tumor microenvironment is a dynamic network of stromal, cancer, and immune cells that interact and compete for resources. We have previously identified the Vanin1 pathway as a tumor suppressor of sarcoma development via vitamin B5 and coenzyme A regeneration. Using an aggressive sarcoma cell line that lacks Vnn1 expression, we showed that the administration of pantethine, a vitamin B5 precursor, attenuates tumor growth in immunocompetent but not nude mice. Pantethine boosts antitumor immunity, including the polarization of myeloid and dendritic cells towards enhanced IFNγ-driven antigen presentation pathways and improved the development of hypermetabolic effector CD8 T cells endowed with potential antitumor activity. At later stages of treatment, the effect of pantethine was limited by the development of immune cell exhaustion. Nevertheless, its activity was comparable with that of anti-PD1 treatment in sensitive tumors. In humans, expression correlates with improved survival and immune cell infiltration in soft-tissue sarcomas, but not in osteosarcomas. Pantethine could be a potential therapeutic immunoadjuvant for the development of antitumor immunity.
Topics: Humans; Mice; Animals; CD8-Positive T-Lymphocytes; Coenzyme A; Pantothenic Acid; Sarcoma; Tumor Microenvironment
PubMed: 37833072
DOI: 10.26508/lsa.202302200 -
BMC Cancer Nov 2019Myeloid sarcoma (MS), also known as chloroma, is an extramedullary manifestation of malignant primitive myeloid cells. Previously, only small studies investigated...
BACKGROUND
Myeloid sarcoma (MS), also known as chloroma, is an extramedullary manifestation of malignant primitive myeloid cells. Previously, only small studies investigated clinical and imaging features of MS. The purpose of this study was to elucidate clinical and imaging features of MS based upon a multicenter patient sample.
METHODS
Patient records of radiological databases of 4 German university hospitals were retrospectively screened for MS in the time period 01/2001 and 06/2019. Overall, 151 cases/76 females (50.3%) with a mean age of 55.5 ± 15.1 years and 183 histopathological confirmation or clinically suspicious lesions of MS were included into this study. The underlying hematological disease, localizations, and clinical symptoms as well as imaging features on CT and MRI were investigated.
RESULTS
In 15 patients (9.9% of all 151 cases) the manifestation of MS preceded the systemic hematological disease. In 43 cases (28.4%), first presentation of MS occurred simultaneously with the initial diagnosis of leukemia, and 92 (60.9%) patients presented MS after the initial diagnosis. In 37 patients (24.5%), the diagnosis was made incidentally by imaging. Clinically, cutaneous lesions were detected in 35 of 151 cases (23.2%). Other leading symptoms were pain (n = 28/151, 18.5%), neurological deficit (n = 27/151, 17.9%), swelling (n = 14/151, 9.3%) and dysfunction of the affected organ (n = 10/151, 6.0%). Most commonly, skin was affected (n = 30/151, 16.6%), followed by bone (n = 29/151, 16.0%) and lymphatic tissue (n = 21/151, 11.4%). Other localizations were rare. On CT, most lesions were homogenous. On T2-weighted imaging, most of the lesions were hyperintense. On T1-weighted images, MS was hypointense in n = 22/54 (40.7%) and isointense in n = 30/54 (55.6%). A diffusion restriction was identified in most cases with a mean ADC value of 0.76 ± 0.19 × 10 mm/s.
CONCLUSIONS
The present study shows clinical and imaging features of MS based upon a large patient sample in a multicenter design. MS occurs in most cases meta-chronous to the hematological disease and most commonly affects the cutis. One fourth of cases were identified incidentally on imaging, which needs awareness of the radiologists for possible diagnosis of MS.
Topics: Adult; Aged; Diagnostic Imaging; Female; Germany; Humans; Image Processing, Computer-Assisted; Magnetic Resonance Imaging; Male; Middle Aged; Proportional Hazards Models; Retrospective Studies; Sarcoma, Myeloid; Symptom Assessment; Tomography, X-Ray Computed
PubMed: 31775680
DOI: 10.1186/s12885-019-6357-y -
Current Opinion in Virology Oct 2022Kaposi's sarcoma-associated herpesvirus (KSHV) causes primary effusion lymphoma (PEL). Here, we review what is known about human gene essentiality in PEL-derived cell... (Review)
Review
Kaposi's sarcoma-associated herpesvirus (KSHV) causes primary effusion lymphoma (PEL). Here, we review what is known about human gene essentiality in PEL-derived cell lines. We provide an updated list of PEL-specific human gene dependencies, based on the improved definition of core essential genes across human cancer types. The requirements of PEL cell lines for interferon regulatory factor 4 (IRF4), basic leukine zipper ATF-like transcription factor (BATF), G1/S cyclin D2 (CCND2), CASP8 and FADD like apoptosis regulator (CFLAR), MCL1 apoptosis regulator (MCL1), and murine double minute 2 (MDM2) have been confirmed experimentally. KSHV co-opts IRF4 and BATF to drive superenhancer (SE)-mediated expression of IRF4 itself, MYC, and CCND2. IRF4 dependency of SE-mediated gene expression is shared with Epstein-Barr virus-transformed lymphoblastoid cell lines (LCLs) and human T-cell leukemia virus type 1-transformed adult T-cell leukemia/lymphoma (ATLL) cell lines, as well as several B-cell lymphomas of nonviral etiology. LCLs and ATLL cell lines similarly share dependencies on CCND2 and CFLAR with PEL, but also have distinct gene dependencies. Genetic dependencies could be exploited for therapeutic intervention in PEL and other cancers.
Topics: Adult; Humans; Animals; Mice; Lymphoma, Primary Effusion; Leukemia-Lymphoma, Adult T-Cell; Epstein-Barr Virus Infections; Myeloid Cell Leukemia Sequence 1 Protein; Herpesvirus 4, Human; Herpesvirus 8, Human; Neoplasms
PubMed: 36182745
DOI: 10.1016/j.coviro.2022.101270