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Antibodies (Basel, Switzerland) Mar 2024Ewing sarcoma is a rare tumor of the bone or soft tissues characterized by diffuse membranous staining for CD99. As this tumor remains incurable in the metastatic,...
BACKGROUND
Ewing sarcoma is a rare tumor of the bone or soft tissues characterized by diffuse membranous staining for CD99. As this tumor remains incurable in the metastatic, relapsed, and refractory settings, we explored the downstream immune implications of targeting CD99.
METHODS
We discovered a human anti-CD99 antibody (NOA2) by phagemid panning and investigated NOA2 immune cell-mediated cytotoxicity in vitro and in vivo focusing on the myeloid cell compartment, given that M2 macrophages are present in human tumors and associated with a poor prognosis.
RESULTS
NOA2 is capable of inducing immune effector cell-mediated Ewing death in vitro via engagement of macrophages. Mice with metastatic Ewing tumors, treated with NOA2, experience tumor growth arrest and an associated increase in intratumoral macrophages. Further, incubation of macrophages and Ewing cells with NOA2, in conjunction with anti-PILRα antibody blockade in vitro, results in the reactivation of previously dormant macrophages possibly due to interrupted binding of Ewing CD99 to macrophage PILRα.
CONCLUSIONS
These studies are the first to demonstrate the role of human immune effector cells in anti-CD99-mediated Ewing tumor death. We propose that the engagement of CD99 by NOA2 results in the recruitment of intratumoral macrophages. In addition, interruption of the CD99:PILRα checkpoint axis may be a relevant therapeutic approach to activate tumor-associated macrophages.
PubMed: 38534214
DOI: 10.3390/antib13010024 -
Laryngoscope Investigative... Apr 2024Hematologic malignancy involving the trachea is rare. It is even less common for tracheal involvement to be the initial manifestation of this disease. We present a case...
OBJECTIVES
Hematologic malignancy involving the trachea is rare. It is even less common for tracheal involvement to be the initial manifestation of this disease. We present a case report highlighting an unusual diagnosis of acute myeloid leukemia (AML) that first presented with prominent tracheal manifestations. There have been only three other published case reports of extramedullary AML with involvement of the trachea.
METHODS
We discuss direct laryngoscopy and bronchoscopy findings, including pinkish-white irregular lesions, which were similar to findings described in the available literature for tracheal AML.
RESULTS
Laboratory findings from our case are reported, including peripheral smear demonstrating 57% blasts and bone marrow biopsy confirming the diagnosis of AML, and the relevance of these findings is discussed.
CONCLUSION
In patients with unusual airway lesions, laboratory testing and a comprehensive airway evaluation including biopsy are necessary to narrow the differential diagnosis.
LEVEL OF EVIDENCE
5.
PubMed: 38525124
DOI: 10.1002/lio2.1231 -
Case Reports in Gastrointestinal... 2024Myeloid sarcoma (MS) is an extramedullary manifestation of acute myeloid leukemia (AML) and commonly occurs in sites such as the lymph nodes, skin, soft tissues, and...
Myeloid sarcoma (MS) is an extramedullary manifestation of acute myeloid leukemia (AML) and commonly occurs in sites such as the lymph nodes, skin, soft tissues, and bone. It more rarely manifests in the pancreas, with less than 20 cases reported in the literature since 1987. Despite its rarity, MS should be considered in the differential diagnosis of a soft tissue mass causing obstructive jaundice, especially if the patient has a known hematologic disease. Isolated cases of pancreatic MS have been known to progress to AML; therefore, it is crucial to differentiate MS from more common diagnoses, such as pancreatic cancer or pancreatitis. This is a case of a 70-year-old male with symptomatic obstructive jaundice secondary to pancreatic MS, ultimately requiring endoscopic ultrasound (EUS) and endoscopic retrograde cholangiopancreatography (ERCP) for diagnosis and management. Also included is a comprehensive review of previous case reports with similar clinical presentations, management, and treatment of pancreatic MS.
PubMed: 38500609
DOI: 10.1155/2024/5513857 -
Frontiers in Oncology 2024Variations in mutation rates among acute myeloid leukemia (AML) patients with myeloid sarcoma (MS) underscore the need for a thorough examination. This meta-analysis was...
INTRODUCTION
Variations in mutation rates among acute myeloid leukemia (AML) patients with myeloid sarcoma (MS) underscore the need for a thorough examination. This meta-analysis was conducted to fill the information gap concerning mutation frequencies in AML patients presenting with MS.
MATERIALS AND METHODS
This study included retrospective and prospective cohorts. It examined genetic alterations in AML patients with and without MS across all age groups. The search strategy employed terms such as "acute myeloid leukemia," "extramedullary," "granulocytic sarcoma," "myeloid sarcoma," and "leukemic cutis" in the EMBASE, MEDLINE, and Scopus databases. Excluded from the study were reviews, case reports, and case series with fewer than 10 cases. Statistical analyses were performed with Review Manager 5.4 software.
RESULTS
The primary analysis incorporated data from 37 cohorts involving 5646 diagnosed AML patients and revealed a 17.42% incidence of MS. The most prevalent mutation among AML patients with MS was -ITD, with a pooled prevalence of 17.50% (95% CI 12.60% to 22.50%; I 82.48%). The dominant fusion gene was , displaying a pooled prevalence of 28.10% (95% CI 15.10% to 41.20%; I 96.39%). In comparison, no significant intergroup differences were observed for , -ITD, , and mutations. Interestingly, the mutation exhibited protective effects for MS patients, with an odds ratio of 0.51 (95% CI 0.32 to 0.81; I 0%). Conversely, the mutation was associated with an increased risk of MS development, with an odds ratio of 5.07 (95% CI 1.87 to 13.73; I 0%).
CONCLUSION
This meta-analysis sheds light on the prevalence of genetic mutations in AML patients with MS, providing insights into the unique characteristics of the mutations and their frequencies. These discoveries are crucial in informing therapeutic and prognostic decisions for individuals with myeloid sarcoma.
PubMed: 38496752
DOI: 10.3389/fonc.2024.1325431 -
Cancers Feb 2024Non-acute myeloid neoplasms (MNs) with mutations (mut-MNs) pose a diagnostic and therapeutic dilemma, primarily manifesting as chronic myelomonocytic leukemia (CMML)... (Review)
Review
Non-acute myeloid neoplasms (MNs) with mutations (mut-MNs) pose a diagnostic and therapeutic dilemma, primarily manifesting as chronic myelomonocytic leukemia (CMML) and myelodysplastic syndromes (MDS). The classification and treatment approach for these conditions as acute myeloid leukemia (AML) are debated. We describe eight cases of atypical mut-MNs from our institution and review the literature. We include a rare case of concurrent prostate carcinoma and MN consistent with chronic eosinophilic leukemia, progressing to myeloid sarcoma of the skin. Of the remaining seven cases, five were CMML and two were MDS. mutations occur in 3-5% of CMML and 1-6% of MDS, with an increased likelihood of rapid evolution to AML. Their influence on disease progression varies, and their prognostic significance in non-acute MNs is less established than in AML. Non-acute MNs with mutations may display an aggressive clinical course, emphasizing the need for a comprehensive diagnosis integrating clinical and biological data. Tailoring patient management on an individualized basis, favoring intensive treatment aligned with AML protocols, is crucial, regardless of blast percentage. Research on the impact of mutations in non-acute myeloid neoplasms is ongoing, requiring challenging prospective studies with substantial patient cohorts and extended follow-up periods for validation.
PubMed: 38398096
DOI: 10.3390/cancers16040705 -
Radiology Case Reports May 2024This report presents a unique case of a 42-year-old female with a history of acute myeloid leukemia (AML) who exhibited an extramedullary relapse in the breast. Given...
This report presents a unique case of a 42-year-old female with a history of acute myeloid leukemia (AML) who exhibited an extramedullary relapse in the breast. Given the rarity of such presentations, this case underscores the importance of considering AML in the differential diagnosis of breast lesions, especially in patients with a pertinent medical history. Additionally, this case highlights the radiological and pathological challenges in distinguishing AML from other breast malignancies. The importance of timely diagnosis and the clinical implications of such a presentation are also discussed.
PubMed: 38384694
DOI: 10.1016/j.radcr.2024.01.067 -
Cureus Jan 2024Myeloid sarcoma (MS) represents a unique clinical presentation of acute myeloid leukemia (AML). This report describes a case of MS in a 66-year-old man who presented...
Myeloid sarcoma (MS) represents a unique clinical presentation of acute myeloid leukemia (AML). This report describes a case of MS in a 66-year-old man who presented with dysphagia, nausea, vomiting, anorexia, and fatigue. Generalized lymphadenopathy was noted on physical exam and confirmed by CT scans which also showed diffuse esophageal wall thickening. Axillary lymph node biopsy was positive for MS. Bone marrow biopsy confirmed AML with 88% blasts. The patient received induction chemotherapy with decitabine and venetoclax and was planned for four cycles of treatment over three months while monitoring the response.
PubMed: 38374841
DOI: 10.7759/cureus.52624 -
Frontiers in Oncology 2023Myeloid sarcoma (MS) is a rare hematological malignancy characterized by the formation of a solid mass of myeloblasts outside the bone marrow, such as in the lymph...
BACKGROUND
Myeloid sarcoma (MS) is a rare hematological malignancy characterized by the formation of a solid mass of myeloblasts outside the bone marrow, such as in the lymph nodes, skin, or bone. MS may arise or concurrently with acute myeloid leukemia (AML), myeloproliferative neoplasm (MPN), or myelodysplastic syndrome (MDS). MS accounts for less than 1% of extramedullary acute myeloid leukemia cases. Phyllodes tumors (PTs) are a rare fibroepithelial breast tumor that can be benign, malignant, or borderline, and account for less than 1% of all breast cancers.
CASE PRESENTATION
We present a unique case of a 50-year-old woman with both breast MS and borderline PT with malignant features, which presented a diagnostic challenge. The patient initially presented with a mass in her right breast, and the initial fine-needle biopsy revealed the presence of immature myeloperoxidase (MPO) myeloid cells consistent with MS. Subsequent pathological analysis of tumor tissues after neoadjuvant radiotherapy and chemotherapy showed a borderline PT with malignant features. Following excision of the tumor, the patient experienced a local recurrence, which was also surgically removed. At 8 months post-surgery, the patient remains free of recurrence under close follow-up.
CONCLUSION
This case highlights the importance of considering the possibility of concurrent malignancies in the differential diagnosis of complex breast masses and underscores the challenges involved in diagnosing and managing such cases. Additionally, we also emphasize the value of neoadjuvant radiotherapy and chemotherapy in MS.
PubMed: 38313212
DOI: 10.3389/fonc.2023.1268617 -
Clinical Case Reports Feb 2024We report an unusual case presentation of a patient with necrotic tissue changes of the right second and third fingers, found to have myeloid sarcoma with -positive...
We report an unusual case presentation of a patient with necrotic tissue changes of the right second and third fingers, found to have myeloid sarcoma with -positive tenosynovitis and underlying acute myeloid leukemia, to highlight the importance of comprehensive evaluation in patients with atypical wounds.
PubMed: 38292221
DOI: 10.1002/ccr3.8465 -
Cureus Jan 2024This case report details a rare case of small bowel myeloid sarcoma (MS) in an otherwise fit and well 49-year-old male presenting initially with vague obstructive...
This case report details a rare case of small bowel myeloid sarcoma (MS) in an otherwise fit and well 49-year-old male presenting initially with vague obstructive symptoms and weight loss. The patient ultimately required an operation for a small bowel obstruction where a laparotomy and small bowel resection were performed due to three cicatrising completely obstructing lesions in the mid-jejunum. Fewer than 1% of patients with acute myeloid leukaemia (AML) present with MS as an initial diagnosis, and only 6.5% of these are intestinal in origin. This report adds to the current body of literature on this rare condition, emphasises the diagnostic challenges resulting in delays to diagnosis, and discusses the crucial role of early and accurate identification for optimal treatment and prognosis. Surgery may be warranted in patients with complications such as obstruction; however, systemic chemotherapy tailored to AML is the primary therapeutic approach for MS patients.
PubMed: 38283782
DOI: 10.7759/cureus.52956