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Leukemia & Lymphoma May 2023Myeloid sarcoma (MS) is currently considered equivalent to de novo acute myeloid leukemia (AML); however, the relationship between these entities is poorly understood....
Myeloid sarcoma (MS) is currently considered equivalent to de novo acute myeloid leukemia (AML); however, the relationship between these entities is poorly understood. This retrospective multi-institutional cohort study compared 43 MS with mutation to 106 AML with mutation. Compared to AML, MS had more frequent cytogenetic abnormalities including complex karyotype ( = .009 and = .007, respectively) and was enriched in mutations of genes involved in histone modification, including ( = .007 and = .008, respectively). AML harbored a higher average number of gene mutations ( = .002) including more frequent mutations ( < .001) and mutations of DNA-methylating genes including and (both < .001). MS had significantly shorter overall survival (OS) than AML (median OS: 44.9 vs. 93.2 months, respectively, = .037). MS with mutation has a unique genetic landscape, and poorer OS, compared to AML with mutation.
Topics: Humans; Bone Marrow; Nuclear Proteins; Nucleophosmin; Sarcoma, Myeloid; Retrospective Studies; Cohort Studies; Leukemia, Myeloid, Acute; Mutation; Prognosis
PubMed: 36960680
DOI: 10.1080/10428194.2023.2185091 -
Clinical Neuroradiology Dec 2019
Review
Topics: Brain Neoplasms; Fatal Outcome; Humans; Infant; Magnetic Resonance Imaging; Male; Sarcoma, Myeloid
PubMed: 30941450
DOI: 10.1007/s00062-019-00779-z -
Journal of Cutaneous Pathology Mar 2013Myeloid sarcoma is a rare tumor of immature myeloid cells in an extramedullary site. Myeloid sarcoma may present in a variety of locations; skin is one of the common... (Review)
Review
Myeloid sarcoma is a rare tumor of immature myeloid cells in an extramedullary site. Myeloid sarcoma may present in a variety of locations; skin is one of the common sites. It may precede or occur concurrently with acute myeloid leukemia, chronic myeloid leukemia, other forms of myeloproliferative disorders/myelodysplastic syndrome or de novo. We report a case of a 4-month-old female who presented with cutaneous lesions without evidence of leukemia, determined to be de novo myeloid sarcoma. She had erythematous nodules in multiple skin sites. Biopsy revealed a diffuse atypical mononuclear cell infiltrate involving the entire dermis and extending to the subcutis. The infiltrate was diffusely positive for lysozyme, CD43, CD15, CD33, CD68 and CD117 and was negative for CD3, CD20, CD34, CD56, CD79a, CD99, myeloperoxidase, desmin, chromogranin and synaptophysin, supporting a diagnosis of myeloid sarcoma. No leukemic involvement was found on evaluation of peripheral blood or bone marrow aspiration. Chromosomal abnormalities were found at chromosomes 7, 10 and 11. The skin lesions resolved following multiple chemotherapy courses, then recurred requiring additional treatment. De novo myeloid sarcoma involving skin without evidence of leukemia can occur in an infant and may present a diagnostic challenge.
Topics: Antigens, CD; Fatal Outcome; Female; Gene Expression Regulation; Humans; Infant; Neoplasm Proteins; Sarcoma, Myeloid; Skin Neoplasms
PubMed: 23095217
DOI: 10.1111/cup.12027 -
Gastroenterologia Y Hepatologia Dec 2019
Review
Topics: Aged; Biomarkers, Tumor; Endoscopic Ultrasound-Guided Fine Needle Aspiration; Fatal Outcome; Humans; Male; Pancreatic Neoplasms; Sarcoma, Myeloid
PubMed: 31324462
DOI: 10.1016/j.gastrohep.2019.04.008 -
Leukemia & Lymphoma Dec 2021Myeloid sarcoma (MS) in the setting of concomitant medullary AML is relatively well described, while much less is known about patients presenting with MS with <20% bone...
Myeloid sarcoma (MS) in the setting of concomitant medullary AML is relatively well described, while much less is known about patients presenting with MS with <20% bone marrow blasts. We conducted a retrospective analysis of 56 patients with MS with <20% marrow blasts seen at MD Anderson between 2005 and 2020. The prevalence of MS without medullary AML was 1.4% among all newly diagnosed AML patients. The majority (75%) of patients had a single known anatomic site involved, with the skin (34%) being the most frequent. The most common histologic subtype was monocytic, and 11% of patients had a known history of an antecedent hematologic disorder. The majority of patients (70%) received frontline intensive chemotherapy induction, with 75% of those evaluable attaining complete or partial responses. The median overall survival (OS) and event-free survival (EFS) were 3.41 and 3.07 years, respectively. Patients with bone marrow blasts of ≥5% or medullary relapse had inferior outcomes, while age (>60 years) was not associated with outcomes. There was a suggestion that patients with isolated leukemia cutis may have had better outcomes compared to patients with other organ involvement, but this did not reach statistical significance. Most patients who had cytogenetic analysis had a diploid karyotype within their MS and bone marrow pathway mutations were enriched in MS at diagnosis, and at time of medullary relapse. Our study provides a large dataset summarizing the clinical and molecular analysis of patients with MS with <20% BM blasts and suggests that monitoring for medullary leukemia is important for early detection of relapse.
Topics: Bone Marrow; Humans; Leukemia, Myeloid, Acute; Middle Aged; Prognosis; Recurrence; Retrospective Studies; Sarcoma, Myeloid
PubMed: 34380367
DOI: 10.1080/10428194.2021.1961235 -
Indian Journal of Pathology &... 2019Myeloid sarcoma is an extra medullary manifestation of acute myeloid leukemia (AML). Primary involvement of the biliary tract with myeloid sarcoma presenting as...
Myeloid sarcoma is an extra medullary manifestation of acute myeloid leukemia (AML). Primary involvement of the biliary tract with myeloid sarcoma presenting as obstructive jaundice without evidence of leukemia is very rare. Here we present a case of 72 year old lady, who initially presented with features of biliary obstruction and was clinically considered as cholangiocarcinoma. She was diagnosed as myeloid sarcoma involving right and left hepatic duct, common bile duct (CBD) on histopathological and immunohistochemistry (IHC) examination after surgical resection. Since she did not show evidence of leukemia on peripheral blood and bone marrow examination treatment was deferred. However, she developed full blown picture of AML within two months and succumbed to her disease. We conclude that obstructive jaundice can be the presenting symptom in myeloid sarcoma without evidence of AML in peripheral blood and bone marrow. However, these cases have to be treated aggressively to obtain remission.
Topics: Aged; Bile Ducts; Diagnosis, Differential; Fatal Outcome; Female; Humans; Jaundice, Obstructive; Leukemia, Myeloid, Acute; Sarcoma, Myeloid
PubMed: 31611450
DOI: 10.4103/IJPM.IJPM_371_18 -
Clinical and Experimental Medicine May 2020Splenomegaly is a key clinical manifestation of myelofibrosis, and splenectomy is currently indicated in patients with drug refractory, symptomatic splenomegaly or with... (Review)
Review
Splenomegaly is a key clinical manifestation of myelofibrosis, and splenectomy is currently indicated in patients with drug refractory, symptomatic splenomegaly or with the aim of improving refractory cytopenias. Transformation to acute myeloid leukemia occurs in up to 20% of patients with myelofibrosis, while cases of myeloid sarcoma have been reported very unfrequently. In this manuscript, we report the case of a 60-year-old man with a history of primary myelofibrosis who underwent splenectomy because of drug-refractory massive splenomegaly, systemic symptoms and anemia. At the opening of the peritoneal cavity, the spleen resulted massively enlarged and tenaciously entrapped by a pervasive neoplastic-like tissue. The extensive involvement of the abdomen fatally complicated the surgical procedure. At postmortem examination, the spleen showed a diffuse infiltration of immature cells that were also found in the peritoneum, bowel, liver, lungs and myocardium. After immunohistochemical, cytogenetic, flow cytometric and molecular characterization of neoplastic population, a diagnosis of disseminated myeloid sarcoma of the spleen was made. This case report highlights a very unusual case of myeloid sarcoma originating from the spleen in a patient with myelofibrosis who had no evidence of bone marrow or peripheral blood involvement by leukemic cells. Molecular characterization showed that leukemic cells originated from the founding clone of the chronic phase. The sarcoma could not be suspected based on clinical findings and was diagnosed only at the time of surgical procedure and autopsy. This case suggests that leukemic transformation of myelofibrosis can originate outside the bone marrow and, presumably rarely, present as a granulocytic sarcoma.
Topics: Humans; Male; Middle Aged; Primary Myelofibrosis; Sarcoma, Myeloid; Splenomegaly
PubMed: 32065308
DOI: 10.1007/s10238-020-00616-5 -
Pediatric Dermatology 2023Cutaneous myeloid sarcoma is rarely present prior to the diagnosis of congenital acute myeloid leukemia (AML); the former is typically diagnosed with or after the...
Cutaneous myeloid sarcoma is rarely present prior to the diagnosis of congenital acute myeloid leukemia (AML); the former is typically diagnosed with or after the leukemia. We report a 2-day-old male born with multiple cutaneous red to violaceous nodules. Histopathologic and immunohistochemistry findings from a skin nodule were suspicious for myeloid sarcoma. Bone marrow biopsy was initially negative for aberrant blasts; however, at age 4 months, AML with a KMT2A gene rearrangement was identified via bone marrow biopsy.
Topics: Humans; Infant; Infant, Newborn; Male; Bone Marrow; Leukemia, Myeloid, Acute; Sarcoma, Myeloid; Skin; Skin Neoplasms
PubMed: 37029447
DOI: 10.1111/pde.15313 -
International Journal of Gynecological... Mar 2020Vulvar malignancies are rare and have diverse origins and presentations. The majority of these tumors are squamous cell carcinoma. An ulcerating vulvar tumor was found...
Vulvar malignancies are rare and have diverse origins and presentations. The majority of these tumors are squamous cell carcinoma. An ulcerating vulvar tumor was found in a 74-yr-old woman presenting with fatigue and leukocytosis. Tumor biopsy revealed infiltration of blastoid cells from myeloid origin. Through bone marrow aspiration chronic myelomonocytic leukemia was diagnosed, of which the vulvar tumor was a rare extramedullary disease manifestation termed myeloid sarcoma. Limited palliative treatment was instated with a focus on the patient's quality of life. Myeloid sarcoma (chloroma; granulocytic sarcoma) is a mass of myeloblasts occurring in cases of myeloid disease. Manifestations in most organ systems have been described. Presentation in gynecologic areas is reported in a few case reports, to which we now add our experience.
Topics: Aged; Female; Humans; Sarcoma, Myeloid; Vulvar Neoplasms
PubMed: 30829759
DOI: 10.1097/PGP.0000000000000595 -
Orbit (Amsterdam, Netherlands) Dec 2012[corrected] To report two rare cases of orbital myeloid sarcoma in adults and discuss special features relevant to this entity. (Review)
Review
BACKGROUND/AIMS
[corrected] To report two rare cases of orbital myeloid sarcoma in adults and discuss special features relevant to this entity.
METHODS
Complete ophthalmologic and systemic examinations followed by incisional biopsy and pathological examination.
RESULTS
Two cases of myeloid sarcoma with orbital involvement were identified. Both cases presented with a clinical picture and imaging which demonstrated infiltrative orbital masses. When combined with 8 previous reported cases, 8/10 demonstrated systemic AML involvement.
CONCLUSIONS
Orbital myeloid sarcoma is a rare entity in adults and highly associated with myelodysplastic syndromes, especially acute myelogenous leukemia (AML). It may present at any stage of AML and requires appropriate workup and treatment, though generally carries a poor prognosis.
Topics: Aged, 80 and over; Biopsy; Diagnosis, Differential; Fatal Outcome; Humans; Immunohistochemistry; Magnetic Resonance Imaging; Male; Middle Aged; Orbital Neoplasms; Sarcoma, Myeloid
PubMed: 23088361
DOI: 10.3109/01676830.2012.723784