Myeloid Sarcoma Research Articles

S4175 A Rare Case of Myeloid Sarcoma of the Stomach

S4175 A Rare Case of Myeloid Sarcoma of the Stomach

S3459 A Case of Myeloid Sarcoma With Hepatic Involvement in a Patient With Pancytopenia

S3459 A Case of Myeloid Sarcoma With Hepatic Involvement in a Patient With Pancytopenia

S2066 Chameleonic Chloroma: A Case of Myeloid Sarcoma Presenting as a Pancreatic Head Mass

S2066 Chameleonic Chloroma: A Case of Myeloid Sarcoma Presenting as a Pancreatic Head Mass

Myeloid sarcoma and pathological fracture: a case report and review of literature

Myeloid sarcoma is a rare clinical entity that presents as an isolated proliferation of leukemic cells, concurrently with or at relapse of acute myeloid leukemia (AML), myelodysplastic syndromes/neoplasms (MDS), chronic myeloid leukemia (CML), and myeloproliferative neoplasm (MPN). Myeloid sarcoma disrupts the normal architecture of its surrounding tissues. When it forms in long bones, it can cause their pathological fracture. We recently experienced a rare case of MDS presenting with myeloid sarcoma in the femur that eventually resulted in its pathological fracture. Detailed chromosomal analysis of the bone marrow cells suggested emergence of myeloid sarcoma during the fast-paced progression of MDS just after acquiring trisomy 22. A comprehensive review of previous cases of myeloid sarcoma-associated pathological fracture indicated possible involvement of structural rearrangements of chromosomes 9 and 22. Management of myeloid sarcoma should continue to improve, and clinicians should note that myeloid sarcoma with specific chromosomal alterations needs extra medical attention to prevent pathological fracture.

Isolated Extramedulary Relapse in T-Acute Lymphoblastic Leukemia

Isolated extramedullary acute lymphoblastic leukemia is a rare phenomenon. This event is more common in acute myeloid leukemia as myeloid sarcoma. This terminology is near the lymphoblastic lymphoma. Isolated extramedullary presentations hasn’t bone marrow involvement morphologically .Submicroscopic bone marrow involvement confirmed by a high-resolution flowcytometry detection method.

Nonmeningothelial Dural-Based Lesions: A Histopathological Analysis.

Introduction We report 30 cases of nonmeningothelial dural-based lesions encountered during a 3-year study period. Materials and Methods We retrospectively reviewed pathology records of patients operated for extra-axial, dural-based lesions during the years 2016 to 2018 and included nonmeningothelial lesions as a part of this study. Results Among the 3,243 neurosurgical specimens for histopathologic examination, only 30 (0.93%) were "nonmeningothelial dural-based lesions." Six (20%) patients were in the pediatric age group. Pathologic assessment identified 13 cases of solitary fibrous tumor/hemangiopericytoma (43.3%) and 7 cases of Ewing's sarcoma/primitive neuroectodermal tumor (23.3%). Two cases (6.7%) were of metastasis. Other lesions included a single case each of non-Hodgkin's lymphoma, undifferentiated sarcoma, solitary plasmacytoma, and granulocytic sarcoma. Nonneoplastic lesions included two cases each of Rosai-Dorfman disease and nonspecific inflammatory lesions. Conclusion Nonmeningothelial dural-based lesions being rare, thorough examination of morphological features is a must by the pathologist, to arrive at the accurate diagnosis. Ancillary tests, if required, should be employed in the context of the morphologic picture.

Intracranial Myeloid Sarcoma Mimicking Hypertensive Intracerebral Hemorrhage.

We herein report a case of intracranial myeloid sarcoma mimicking hypertensive intracerebral hemorrhage. A 71-year-old man with a history of acute myeloid leukemia was admitted with acute-onset dysarthria. A hematoma-like lesion was found on computed tomography in the left putamen. Magnetic resonance imaging (MRI) and cerebrospinal fluid cytology confirmed the diagnosis of intracranial myeloid sarcoma. The patient showed a favorable response to chemotherapy, and follow-up MRI revealed shrinkage of the tumor. Since the computed tomography findings resemble those of intracerebral hemorrhage, it is important to suspect intracranial neoplasm, particularly in cases with a history of hematologic diseases.

AML-220 Sinusitis as a Presentation of Acute Myeloid Leukemia (AML) and Myeloid Sarcoma (MS)

AML-220 Sinusitis as a Presentation of Acute Myeloid Leukemia (AML) and Myeloid Sarcoma (MS)

POSTER: AML-220 Sinusitis as a Presentation of Acute Myeloid Leukemia (AML) and Myeloid Sarcoma (MS)

POSTER: AML-220 Sinusitis as a Presentation of Acute Myeloid Leukemia (AML) and Myeloid Sarcoma (MS)

Clinical characteristics, treatment options, and prognosis of myeloid sarcoma: analysis using the SEER database

ABSTRACTBackgroundMyeloid sarcoma (MS) is a very rare hematologic disorder. This study analyzes the early treatment options for patients with different types of MS and explores the prognostic factors of MS.MethodsPatients aged 15 years and older with MS in the SEER database (diagnosed from 2000 to 2018) were selected, excluding those with an unknown first course of treatment, an unknown location of disease, and less than 1 month of follow-up. Statistical methods used a chi-square test to compare clinical characteristics; Kaplan-Meier analysis to compare survival differences; and Cox proportional risk models to identify prognostic factors affecting overall survival (OS).ResultsData were collected from 472 patients: 244 patients with isolated myeloid sarcoma (IMS) and 228 patients with non-isolated myeloid sarcoma (non-IMS). IMS patients mostly chose local treatment, while non-IMS patients mostly chose chemotherapy. There was a significant difference in OS between IMS patients treated with combined treatment and those without treatment. For non-IMS, treated patients had longer OS than untreated, but the difference was not statistically significant. Among adult patients, those younger than 60 years had a better prognosis. Patients with the urinary system, digestive system, reproductive system and chest and abdomen as the initial site had a better prognosis.ConclusionsEarly combination therapy in IMS patients had a longer OS, and chemotherapy combined with radiotherapy/surgery should be the treatment of choice. For non-IMS patients, early combination therapy did not show a significant advantage. Age and location of first presentation were independent factors affecting MS patients’ long-term prognosis.

Real-world experience with selinexor-containing chemotherapy-free or low-dose chemotherapy regimens for patients with relapsed/refractory acute myeloid leukemia and myeloid sarcoma.

Introduction: 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. Methods: 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. Results: 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. Conclusion: 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.

FLT3-ITD and NPM1 Double Mutation Acute Myeloid Leukemia Case Presenting with Diffuse Skin Granulocytic Sarcoma

Objective: Acute myeloid leukemia (AML) is characterized by leukemic blasts that are not limited to the bone marrow or peripheral blood, may be presented with granulocytic sarcoma, and leukemic cells outside of the blood or bone marrow are called extramedullary involvement (EMI). Skin, bone, and lymph nodes are the most prevalent locations of extramedullary illness. Granulocytic sarcoma (GS) should be considered in the differential diagnosis of nodules, pustules, or plaque-like lesions, especially in patients with suspected hematological disease. No EMI-specific treatment regimens have been established; patients who are suitable for intensive therapy are typically treated with anthracycline and cytarabine-containing regimens. The most common genetic aberration in adult AML is somatic mutations in exon 12 of the NPM gene (NPM1), which affect up to 60% of individuals with normal karyotype AML and around 35% of all cases. Patients with NPM1 mutations are twice as likely to also have a FMS-like tyrosine kinase internal transmembrane duplications (FLT3-ITD) mutation as patients without NPM1 mutations. AML frequently harbors FLT3 mutations that result in (FLT3-ITD) or alterations to the kinase domain's activating loop (FLT3-TKD). Short remissions and unfavorable outcomes are linked to leukemia cells that have a high mutation proportion of FLT3-ITD molecules on their surface. Case: Here in this study, we report a patient diagnosed with FLT3-ITD and NPM1 double mutation AML (FAB classification M0, M1), admitted with diffuse granulocytic sarcoma.

Pancreatic follicular dendritic cell sarcoma: a rare case report and systematic literature review of 7 cases

IntroductionPancreatic follicular dendritic cell sarcoma (FDCS) is an exceptionally rare and low-to-moderate malignancy, with only seven reported cases to date. Clinical diagnosis of FDCS is challenging due to the lack of distinct biological and radiographic features.Case presentationA 67-year-old woman presented to the hospital with a 4-day history of severe abdominal pain. Imaging studies (CT and MRI) revealed a large cystic mass located at the tail of the pancreas, which was suspected to be myeloid sarcoma (MS) based on EUS and CT-guided pancreatic puncture. Postoperative pathology and immunohistochemistry confirmed the diagnosis of pancreatic FDCS. After the diagnosis was confirmed, the patient received postoperative chemotherapy with the CHOP regimen. At 11 months of follow-up, there was no evidence of recurrence. Seven published cases have been reviewed to comprehensively summarize the clinical characteristics, diagnosis, and treatment options of FDCS.ConclusionWhile imaging can be useful in detecting pancreatic FDCS, it should be interpreted with caution as it can be challenging to differentiate from other pancreatic tumors. Pathology and immunohistochemistry are considered the gold standard for diagnosis, with CD21, CD23, and CD35 being specific tumor cell markers. However, preoperative diagnosis of pancreatic FDCS remains difficult, and the pancreatic puncture may further increase the risk of misdiagnosis. The disease is highly prone to recurrence and metastasis, and surgery is the preferred method for both diagnosis and treatment of localized disease.

Myeloid Sarcoma: A Mediastinal Masquerader

Mediastinal Myeloid Sarcoma is a rare entity. Here we present a case of a 28-year-old gentleman, who first presented with a mediastinal mass and was clinically considered lymphoblastic lymphoma. He was diagnosed initially as having T lymphoblastic lymphoma on a mediastinal trucut biopsy. After one month patient was diagnosed with Acute myeloid leukemia on peripheral smear and bone marrow examination and was confirmed with the help of flow cytometry. The biopsy slides from the mediastinal mass were reviewed and based on the smear findings additional immunohistochemical markers were added and diagnosed as Myeloid Sarcoma. Keywords: Myeloid sarcoma, Mediastinum, Acute myeloid leukemia

Isolated myeloid sarcoma of the pancreas: a case report

Myeloid sarcoma (MS) is a rare solid tumor, and the diagnose is ambiguity. On account of the rarity of MS, it is often misdiagnosed. In order to promote clinicians to have a better understanding of the disease, a case of isolated myeloid sarcoma of the pancreas has been admitted by the Center for Pancreas, the First Affiliated Hospital of Nanjing Medical University. A 36-year-old male patient presented to our department with recurrent postprandial abdominal distension and pain, and weight loss. The initial radiologic diagnose of the lesion was pancreatic cancer and a pancreatectomy was performed. Eventually, the histopathology result confirmed the final diagnosis: myeloid sarcoma (MS). To date, this type of disease has hardly been reported in the literature. This case report describes the diagnostic and treatment process and discusses the better way for diagnosis, which will guide such diseases in the future.

Rare cause of biliary obstruction from myeloid sarcoma has better prognosis, if timely diagnosed: case report with literature review

Extramedullary myeloid sarcoma (EMMS) involving the biliary tract is extremely rare. We describe, a case of a 40-year-old gentleman who presented with obstructive jaundice and features of malignant biliary stricture on ERCP and MRCP. Histopathology revealed myeloblasts, while peripheral blood and bone marrow did not reveal any evidence of leukemia.

Intracranial myeloid sarcoma as the first presentation of acute myeloid leukemia and literature review.

Intracranial myeloid sarcoma is a rare extramedullary presentation of acute myeloid leukemia (AML). Itcan involve the meninges and ependyma presenting as extra-axial mass lesion. Rarely, it can also invade the brainparenchyma. It is commonly seen in children. It is usually misdiagnosed due to its close resemblance to otherintracranial tumors (meningioma, metastasis, Ewing's sarcomas, and lymphoma). These are underdiagnosed ifthey precede the diagnosis of leukemia. A 7-year-old boy with isolated intracranial myeloid sarcoma who presented with raised intracranialpressure (ICP) which was successfully managed by surgical excision. Isolated intracranial myeloid sarcoma is a rare presentation of AML. Leukemia can be diagnosed earlyduring the postoperative period and can be started on therapy timely. These patients requires regular follow-ups(clinical, laboratory and radiological) to detect relapses early.

Intracranial hemorrhage (ICH) in acute leukemia (AL): A single-center study from a general hospital with two cases managed with middle meningeal artery (MMA) embolization.

e19033 Background: ICH is a devastating and potentially fatal complication in patients with AL. Contributing factors for ICH include thrombocytopenia, hyperleukocytosis, and coagulopathy. Acute promyelocytic leukemia (APL) has a higher risk of disseminated intravascular coagulation (DIC) and fatal ICH, contributing to early deaths from the disease. At our institution, we have incorporated MMA embolization into traditional methods of observation and surgery for the management of ICH in AL patients. Methods: We performed a single-center retrospective study of adult AL patients with ICH at Memorial Hermann Hospital Texas Medical Center from January 1, 2013, to December 31, 2022. Data collected included: age at diagnosis, type of AL, white blood cell count (WBC), platelet count, coagulation profile, Glasgow Coma Scale score at presentation, response to platelet transfusion, need for surgical, minimally invasive intervention, and mortality rates at 72 hours and 30 days. Descriptive statistics were used to analyze the data. Results: Among 160 consecutive AL patients, we identified 22 (13.75%) with ICH at presentation (APL = 11, AML = 6, B-ALL = 3, Burkitt leukemia = 1, myeloid sarcoma = 1). All were newly diagnosed except for one (an intracranial relapse of AML). Median age was 54 (range, 20–82) years; 60% were women. Subject races included white (n = 10), Asian (n = 5), Hispanic (n = 2), African American (n = 2), and others (n = 3). Median WBC count at admission was 23 (range, 0.2–400) × 109/L. Thrombocytopenia (platelet levels < 50,000/μL) was present in 16 patients (72%), and 3 had platelet counts < 10,000/μL. Twelve patients (54%) had evidence of DIC. Sites of ICH included intraparenchymal (n = 8), subarachnoid (n = 3), subdural (n = 10), cerebellum (n = 1). In 3 patients, ICH extended into the ventricles. Three patients had ≥2 types of ICH. Three patients had middle cerebral artery ischemic stroke in addition to ICH. One patient with myelofibrosis and myeloid sarcoma had bilateral subdural hematomas (SDHs) on admission and developed a fatal basal ganglia ICH during induction chemotherapy. All APL patients were promptly started on all-trans retinoic acid. Two patients had refractory thrombocytopenia. Five patients required craniotomy, with 2 deaths from recurrent ICH. Two patients with SDH underwent MMA embolization with favorable outcomes. Four patients (18%) died within 72 hours of admission (APL = 3, AML = 1). The 30-day mortality rate among patients presenting with ICH was 31% (n = 7). Conclusions: ICH in AL continues to be a major cause of mortality and morbidity. Early diagnosis, transfusion support, correction of coagulopathy, and neurosurgical expertise are critical in improving outcomes in AL-associated ICH. MMA embolization is a new, minimally invasive strategy for the management of SDH in these critically ill patients.

Myeloid sarcoma of the larynx as manifestation of the terminal stage of myeloproliferative disease

A rare clinical observation of advanced myeloid sarcoma developed through blast transformation of post-polycythemia vera myelofibrosis as extramedullary manifestation of acute myeloid leukemia affecting larynx, laryngopharynx, trachea, soft tissues of the neck, pleura, and skeletal bones is presented.

Phase 1 study of ziftomenib (KO-539) in combination with venetoclax or venetoclax/azacitidine or standard induction cytarabine/daunorubicin (7+3) chemotherapy for the treatment of patients with acute myeloid leukemia.

TPS7079 Background: Relapsed or refractory (R/R) acute myeloid leukemia (AML) with nucleophosmin 1-mutations ( NPM1-m) or KMT2A-rearrangment ( KMT2A-r) represent a high unmet need. Ziftomenib is a potent and selective inhibitor that targets the menin-mixed-lineage leukemia (MLL) lysine[K]-specific methyltransferase 2A ( KMT2A) interaction driving leukemogenesis in these subtypes. An ongoing Phase 1/2 study (KO-MEN 001) has demonstrated meaningful clinical activity and tolerability with ziftomenib monotherapy in R/R AML. In preclinical testing, ziftomenib combined with venetoclax induced synergistic lethality in KMT2A-r and NPM1-m human AML cell lines and patient-derived cells. Moreover, treatment with ziftomenib plus venetoclax/azacitidine has induced prolonged durable remissions in mice with KMT2A-r AML xenografts. Therefore, administration of ziftomenib in combination with standard-of-care (SOC) therapies may provide additional clinical benefit for patients with newly diagnosed or R/R NPM1-m and KMT2A-r menin-dependent AML. Methods: KOMET-007 (KO-MEN-007, NCT# ) is a 2-part dose escalation (Phase 1a) and expansion (Phase 1b) study to evaluate the safety, tolerability, and preliminary antileukemic activity of ziftomenib in combination with non-intensive chemotherapy (NIC) and intensive chemotherapy (IC) in patients with NPM1-m and KMT2A-r AML. During Phase 1a, the ziftomenib dose will be escalated with standard doses of either venetoclax and azacitidine (zifto/ven/aza) or cytarabine and daunorubicin (zifto/7+3) in defined genetic cohorts ( NPM1-m and KMT2A-r) using a rule-based approach (n = 6 per cohort/dose level) to select ziftomenib doses for expansion/validation in Phase 1b. The Phase 1b portion will also evaluate zifto/ven/aza in newly diagnosed AML ( KMT2A-r and NPM1-m) and zifto/ven in R/R AML ( NPM1-m only). The overall primary objectives are to establish the minimum biologically effective, dose limiting toxicities, and recommended Phase 2 dose of ziftomenib combined with NIC and IC backbones in each genetically defined cohort. Additional secondary endpoints include preliminary antileukemic efficacy (including MRD-negative responses and number of patients able to transition to subsequent allogeneic stem cell transplant), pharmacokinetic, and pharmacodynamic data. Exploratory end points include biomarkers for efficacy and resistance, pharmacodynamic biomarkers and activity in isolated myeloid sarcoma. Clinical trial information: NCT04067336 .