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Case Reports in Oncology 2024Myeloid sarcoma (MS) is also known as chloroma, extramedullary acute myeloid leukemia (AML), or granulocytic sarcoma. MS is a rare extramedullary infiltration of myeloid...
INTRODUCTION
Myeloid sarcoma (MS) is also known as chloroma, extramedullary acute myeloid leukemia (AML), or granulocytic sarcoma. MS is a rare extramedullary infiltration of myeloid cells, most commonly collecting in the skin and causing a small number of localized lesions. It is strongly associated with AML; however, MS more commonly occurs after diagnosis of AML is previously established or after previous treatment of AML.
CASE PRESENTATION
This case describes a patient with an atypical presentation of MS with no known history of AML and up to 18 lesions identified on CT scan that were previously being monitored for months by her primary care physician. She presented with sepsis attributed to choledocholithiasis versus bacteremia from scattered abscesses versus osteomyelitis of her left knee; nonetheless, lactic acid failed to improve after common bile duct stent with biliary sphincterotomy/dilation or with incision and drainage and empiric antibiotics. Core needle biopsy of her left abdominal sidewall was eventually positive for MS, but she unfortunately developed multiorgan failure with symptomatic hypercalcemia refractory to treatment and ultimately decided to go to comfort care rather than pursue further workup and treatment. Although bone marrow biopsy was ultimately not performed to rule out synchronous AML, this is likely a case of isolated MS due to her scattered skin lesions being present for months prior to hospitalization and acute illness.
CONCLUSION
This case highlights the importance of maintaining MS in the differential diagnosis and the importance of early diagnostic core needle biopsy for patients with persistent skin lesions of unknown origin.
PubMed: 38188484
DOI: 10.1159/000535600 -
Journal of the College of Physicians... Jan 2024Null.
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Journal of Hematopathology Mar 2024Agarose-based cell block (CB) technique can be modified to be combined with the frozen section technique for the preparation of a high-quality frozen-embedded CB (F-CB)...
Agarose-based cell block (CB) technique can be modified to be combined with the frozen section technique for the preparation of a high-quality frozen-embedded CB (F-CB) from an effusion or fine-needle aspiration (FNA) cytology sample. This combined technique can be effectively used for the immunocharacterization of the hematolymphoid cells on F-CB. To demonstrate the applicability of performing diagnostic ICC on F-CB, we have analyzed the immunophenotype of the hematolymphoid cells in a series of eight cases of effusions and eight cases of FNA cytology specimens by using CB-ICC on sections cut from frozen-embedded CBs. The SurePath residue or cytologic material scraped off from the FNA cytology smear that was diagnostic for or suspicious of hematolymphoid malignancy was pelleted and pre-embedded in agarose. Half of the agarose-embedded pellet was frozen-embedded in OCT compound for the preparation of F-CB, while the other half was processed for the preparation of paraffin-embedded CB. Sections cut from the F-CB and P-CB were used for CB-ICC. Panels of ICC on the F-CBs could enable the immunocytochemical differential diagnosis of large cell hematologic malignancies that encompass anaplastic large cell lymphoma and other forms of large-cell hematolymphoid malignancies such as large B-cell lymphomas, anaplastic plasma cell myeloma, myeloid sarcoma, and T-lymphoblastic lymphoma. It also appeared that the small B-cell lymphomas in the effusions or FNAs could be differentially diagnosed with the aid of CB-ICC on the F-CB. A modified agarose-based CB technique can be combined with the frozen-embedded CB method for the preparation of F-CB that can be directly used for the immunocytochemical differential diagnosis of hematolymphoid cytology samples.
Topics: Humans; Immunohistochemistry; Sepharose; Biopsy, Fine-Needle; Multiple Myeloma; Lymphoma, Large B-Cell, Diffuse
PubMed: 38175379
DOI: 10.1007/s12308-023-00569-0 -
Biomedicine & Pharmacotherapy =... Feb 2024Kirsten rats sarcoma viral oncogene (KRAS), the first discovered human oncogene, has long been recognized as "undruggable". KRAS mutations frequently occur in multiple... (Review)
Review
Kirsten rats sarcoma viral oncogene (KRAS), the first discovered human oncogene, has long been recognized as "undruggable". KRAS mutations frequently occur in multiple human cancers including non-small cell lung cancer(NSCLC), colorectal cancer(CRC) and pancreatic ductal adenocarcinoma(PDAC), functioning as a "molecule switch" determining the activation of various oncogenic signaling pathways. Except for its intrinsic pro-tumorigenic role, KRAS alteration also exhibits an unique immune signature characterized by elevated PD-L1 level and high tumor mutational burden(TMB). KRAS mutation shape an immune suppressive microenvironment by impeding effective T cells infiltration and recruiting suppressive immune cells including myeloid-derived suppressor cells(MDSCs), regulatory T cells(Tregs), cancer associated fibroblasts(CAFs). In immune checkpoint inhibitor(ICI) era, NSCLC patients with mutated KRAS tend to be more responsive to ICI than patients with intact KRAS. The hallmark for KRAS mutation is the existence of multiple kinds of co-mutations. Different types of co-alterations have distinct tumor microenvironment(TME) signatures and responses to ICI. TP53 co-mutation possess a "hot" TME and achieve higher response to immunotherapy while other loss of function mutation correlated with a "colder" TME and a poor outcome to ICI-based therapy. The groundbreaking discovery of KRAS G12C inhibitors significantly improved outcomes for this KRAS subtype even though efficacy was limited to NSCLC patients. KRAS G12C inhibitors also restore the suppressive TME, creating an opportunity for combinations with ICI. However, an inevitable challenge to KRAS inhibitors is drug resistance. Promising combination strategies such as combination with SHP2 is an approach deserve further exploration because of their immune modulatory effect.
Topics: Animals; Humans; Carcinoma, Non-Small-Cell Lung; Lung Neoplasms; Mutation; Pancreatic Neoplasms; Proto-Oncogene Proteins p21(ras); Tumor Microenvironment
PubMed: 38171240
DOI: 10.1016/j.biopha.2023.116058 -
Clinical Nuclear Medicine Feb 2024Myeloid sarcoma is a neoplastic mass formed by the infiltration of primitive or immature myeloid cells into organs and tissues outside the bone marrow. It may occur...
Myeloid sarcoma is a neoplastic mass formed by the infiltration of primitive or immature myeloid cells into organs and tissues outside the bone marrow. It may occur before, at the same time, or manifest as the recurrence of acute myeloid leukemia, myelodysplastic syndromes, and chronic myeloproliferative syndromes. It may involve any organ or tissue, including skin, soft tissue, lymph nodes, and gastrointestinal tract and bone. Isolated humerus involvement is extremely rare. Herein, we present the FDG PET/CT findings of a rare case of isolate myeloid sarcoma in the right humerus, which showed only increased bone density with moderate FDG uptake.
Topics: Humans; Sarcoma, Myeloid; Fluorodeoxyglucose F18; Positron Emission Tomography Computed Tomography; Positron-Emission Tomography; Humerus
PubMed: 38170922
DOI: 10.1097/RLU.0000000000005009 -
Cureus Nov 2023Acute myeloid leukemia (AML) is the most prevalent form of leukemia in adults, with rising global incidence rates. AML usually presents with non-specific clinical...
Acute myeloid leukemia (AML) is the most prevalent form of leukemia in adults, with rising global incidence rates. AML usually presents with non-specific clinical features such as pallor, fever, and bleeding. This case report discusses a unique presentation of AML, where a 25-year-old female with a history of hypertension presented with unilateral facial swelling, chest pain, and shortness of breath. Radiologic investigations revealed a mediastinal mass encasing the superior vena cava (SVC), confirming the suspicion of SVC syndrome. Upon testing with a biopsy, the mass was found to be composed of immature myeloid cells confirming the diagnosis of myeloid sarcoma-associated AML. The patient's treatment involved a combination of surgical debridement, induction chemotherapy, supportive care, and management of complications. This case highlights that despite its common occurrence, AML may present with atypical clinical manifestations such as SVC syndrome, posing challenges in its diagnosis and timely management.
PubMed: 38161934
DOI: 10.7759/cureus.49616 -
Asian Journal of Surgery Mar 2024
Topics: Male; Humans; Sarcoma, Myeloid; Prostate; Sarcoma; Soft Tissue Neoplasms; Pelvis
PubMed: 38161133
DOI: 10.1016/j.asjsur.2023.12.076 -
BMJ Case Reports Dec 2023This case report describes the case of a man in his seventies presenting with a nasopharyngeal deposit of myeloid sarcoma associated with acute monomyelocytic leukaemia....
This case report describes the case of a man in his seventies presenting with a nasopharyngeal deposit of myeloid sarcoma associated with acute monomyelocytic leukaemia. He presented with right nasal obstruction associated with unilateral pulsatile tinnitus. CT and MRI scans of sinuses identified a moderately restricting mucosal swelling of the right torus tubarius, and a biopsy of the lesion diagnosed a nasal deposit of myeloid sarcoma.
Topics: Humans; Male; Leukemia, Myeloid, Acute; Nasopharynx; Nose; Sarcoma, Myeloid; Tinnitus; Aged
PubMed: 38123315
DOI: 10.1136/bcr-2022-251681 -
Cell Reports. Medicine Dec 2023Chronic myelomonocytic leukemia (CMML) is frequently associated with mutations in the rat sarcoma gene (RAS), leading to worse prognosis. RAS mutations result in active...
Chronic myelomonocytic leukemia (CMML) is frequently associated with mutations in the rat sarcoma gene (RAS), leading to worse prognosis. RAS mutations result in active RAS-GTP proteins, favoring myeloid cell proliferation and survival and inducing the NLRP3 inflammasome together with the apoptosis-associated speck-like protein containing a caspase recruitment domain (ASC), which promote caspase-1 activation and interleukin (IL)-1β release. Here, we report, in a cohort of CMML patients with mutations in KRAS, a constitutive activation of the NLRP3 inflammasome in monocytes, evidenced by ASC oligomerization and IL-1β release, as well as a specific inflammatory cytokine signature. Treatment of a CMML patient with a KRAS mutation using the IL-1 receptor blocker anakinra inhibits NLRP3 inflammasome activation, reduces monocyte count, and improves the patient's clinical status, enabling a stem cell transplant. This reveals a basal inflammasome activation in RAS-mutated CMML patients and suggests potential therapeutic applications of NLRP3 and IL-1 blockers.
Topics: Humans; Inflammasomes; NLR Family, Pyrin Domain-Containing 3 Protein; Proto-Oncogene Proteins p21(ras); Leukemia, Myelomonocytic, Chronic; Symptom Burden; Interleukin-1
PubMed: 38118408
DOI: 10.1016/j.xcrm.2023.101329 -
Journal Francais D'ophtalmologie Mar 2024
Topics: Child; Humans; Sarcoma, Myeloid
PubMed: 38114380
DOI: 10.1016/j.jfo.2023.07.004