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Medicine Jan 2024With the advancement of diagnostic technology, true acute undifferentiated leukemia (AUL) is becoming more rare, and AUL with extramedullary sarcoma has not been... (Review)
Review
BACKGROUND
With the advancement of diagnostic technology, true acute undifferentiated leukemia (AUL) is becoming more rare, and AUL with extramedullary sarcoma has not been reported.
CASE PRESENTATION
This article reports a case of AUL with extramedullary sarcoma. Flow cytometric analysis of the bone marrow and lymph nodes indicated that the tumor cells of both were of the same origin and mainly expressed stem cell markers and CD7, no myeloid-specific markers, T-lymphoblastic-related markers, and B-lymphoblastic-related markers. Although the priming regimen combined with azacitidine was ineffective, complete remission was achieved by switching to azacitidine combined with HIA (homoharringtonine, idarubicin plus Ara-C).
CONCLUSION
To diagnosis de novo acute leukemia with extensive and comprehensive cellular immune maker detection is available and credible, the expression of a single relatively nonspecific myeloid antigen as a immune maker to detect AUL or AUL associated with sarcoma is precise and effective in our case, which patient was benefit from HIA regiment.
Topics: Humans; Sarcoma, Myeloid; Leukemia, Myeloid, Acute; Leukemia; Bone Marrow; Acute Disease; Azacitidine
PubMed: 38277531
DOI: 10.1097/MD.0000000000036948 -
Journal of the Chinese Medical... Apr 2013Myeloid sarcoma (MS) is a rare, extramedullary malignant tumor composed of immature myeloid precursor cells and myeloblast. Most MSs occur in the subperiosteal region of...
Myeloid sarcoma (MS) is a rare, extramedullary malignant tumor composed of immature myeloid precursor cells and myeloblast. Most MSs occur in the subperiosteal region of the bone, with the skull, sternum, ribs, and proximal portions of the long bones being the common sites of involvement. It is thought that the MS tumor originates in the bone marrow, and traverses the Haversian canals to reach the subperiosteum. Various reports have also described the involvement of the liver, spleen, brain, heart, pharynx, uterus, vagina, skin, kidney, and other soft tissues in the formation of the tumor.
Topics: Cheek; Humans; Male; Maxillary Sinus Neoplasms; Middle Aged; Mouth Neoplasms; Sarcoma, Myeloid
PubMed: 23557893
DOI: 10.1016/j.jcma.2012.12.005 -
The Neuroradiology Journal Jun 2017Introduction A 74-year-old man presented to hospital with a headache, thrombocytopaenia and an acute deterioration in cognition on a background of acute monocytic...
Introduction A 74-year-old man presented to hospital with a headache, thrombocytopaenia and an acute deterioration in cognition on a background of acute monocytic leukaemia in remission. Method This is a case report with computed tomography (CT), magnetic resonance (MR) and histopathology imaging. Results Preoperative CT and limited MR demonstrated a subdural lesion with marked midline shift. Craniotomy performed for evacuation of the presumed subdural haematoma revealed a solid tumour-like lesion. Histopathology identified the presence of a myeloid sarcoma (chloroma). Postoperative MRI with contrast revealed the solid nature of the mass. Conclusion The use of contrast is critical in the assessment of intracranial lesions to distinguish myeloid sarcoma from subdural haematoma in the context of leukaemia and a neurologically deteriorating patient.
Topics: Aged; Brain Neoplasms; Contrast Media; Craniotomy; Diagnosis, Differential; Hematoma, Subdural; Humans; Magnetic Resonance Imaging; Male; Sarcoma, Myeloid; Tomography, X-Ray Computed
PubMed: 28134024
DOI: 10.1177/1971400916689343 -
Medicine May 2022Isolated myeloid sarcoma (MS) is characterized by the rapid proliferation of myeloblasts of acute myeloid leukemia (AML), without any blood or bone marrow involvement....
RATIONALE
Isolated myeloid sarcoma (MS) is characterized by the rapid proliferation of myeloblasts of acute myeloid leukemia (AML), without any blood or bone marrow involvement. This disease can manifest with extramedullary organ involvement, such as the skin, lymph nodes, bone, brain, breast cervix, and visceral organs, while the occurrence of myeloid sarcomas in the stomach is rare. Isolated MS has been associated with acute myeloid leukemia (AML), but the rapid progression of MS to acute myeloid leukemia with a complex karyotype and TLS-ERG fusion gene is even rarer.
PATIENT CONCERNS
A 33-year-old woman suffered from persistent epigastric pain accompanied by two months of anorexia and nausea, as well as 1-week of melena.
DIAGNOSIS
This patient was initially diagnosed with gastric MS that eventually transformed into AML with a complex karyotype and TLS-ERG fusion gene, 4 months later.
INTERVENTIONS
Only palliative care, including nutrition support, antacids, blood transfusion, anti-infection methods were used on this patient to determine the cachexia status and the family's requirement.
OUTCOMES
Routine follow-up results demonstrated this patient had died due to cerebral hemorrhage five months after the diagnosis of MS.
LESSONS
Comprehensive integration of patient history, imaging features, mass and bone marrow biopsy, and molecular cytogenetic may provide insights that could help us avoid the misdiagnosis of gastric MS. Isolated gastric MS can rapidly progress to AML with a poor prognosis if the patient does not receive appropriate treatment.
Topics: Adult; Female; Gene Fusion; Humans; Karyotype; Leukemia, Myeloid, Acute; Oncogene Proteins, Fusion; RNA-Binding Protein FUS; Sarcoma, Myeloid; Soft Tissue Neoplasms; Stomach Neoplasms; Transcriptional Regulator ERG
PubMed: 35623083
DOI: 10.1097/MD.0000000000029475 -
AME Case Reports 2021Granulocytic sarcoma, chloroma, myeloblastoma, or here referred as myeloid sarcoma (MS), is a rare extramedullary tumor composed of immature myeloid cells called...
Granulocytic sarcoma, chloroma, myeloblastoma, or here referred as myeloid sarcoma (MS), is a rare extramedullary tumor composed of immature myeloid cells called myeloblasts. MS is seen most commonly in patients with acute myeloid leukemia and less frequently in chronic myeloid leukemia, myelodysplastic syndrome. In rarer instances, MS has been shown to precede the development of myeloid tumors by acute myeloblastic leukemia (AML). In particular, isolated MS involving spine is extremely rare. We herein present a rare case of isolated spinal MS in non-leukemic patient. This is a previously relatively healthy 47-year-old man who presented with signs of lumbar spinal cord compression, initially reported as schwannoma on imaging, later diagnosed with spinal MS on pathology. Further workup did not reveal any evidence of bone marrow or other hematological involvement. The patient successfully treated by L4/L5 laminectomy and debulking with subsequent radiation resulting in substantial decrease in size of tumor with significant improvement in symptoms during follow up. This case not only describes a rare case of isolated MS of lumbar spine, but also highlights the potential treatment challenges of such a rare diagnosis. We review the available literature, discuss available treatment options, and highlight the need for further investigations along with increased clinician awareness.
PubMed: 34312606
DOI: 10.21037/acr-20-110 -
Neoplasia (New York, N.Y.) Jul 2022The molecular mechanism of myeloid sarcoma (MS) formation remains nuclear. Our clinical and mouse model findings from a previous study revealed that cooperation of KMT2A...
Hoxa11-mediated reduction of cell migration contributes to myeloid sarcoma formation induced by cooperation of MLL/AF10 with activating KRAS mutation in a mouse transplantation model: Hoxa11 in myeloid sarcoma formation.
The molecular mechanism of myeloid sarcoma (MS) formation remains nuclear. Our clinical and mouse model findings from a previous study revealed that cooperation of KMT2A (MLL) translocation (MLL-t) with activating N-/K-RAS mutations promoted MS formation in a shorter latency. To improve the understanding of MS formation, in this study, we performed imaging cell trafficking analysis and demonstrated that cells harboring cooperating mutations migrated more slowly to omental adipose tissues and more cells were retained in adipose tissues in vivo. Comparison of transcriptome profiling among three pairs of mouse MLL/AF10(OM-LZ) leukemia cell lines harboring activating and wild-type KRAS identified 77 differentially expressed genes (DEGs) with >1.5-fold change. Functional annotation of these 77 DEGs using Gene Ontology (GO) enrichment analysis followed by cluster analysis revealed that GO terms related to development/differentiation have the highest enrichment score. The roles of Hoxa10 and Hoxa11, two genes which mapped to this cluster, were further characterized. Silencing Hoxa10 and Hoxa11 in cells harboring cooperating mutations prolonged the survival and reduced MS formation, respectively, in the recipient mice. Data of imaging cell trafficking as well as competitive engraftment and clonal expansion analyses indicated that silencing or overexpressing Hoxa11 in mouse leukemia cells affected cell migration and retention in omental adipose tissue. Although silencing Hoxa11 in leukemia cells did not affect Cxcr4 expression, it resulted in increased transwell migration, motility in confined spaces 3 μm in size, and cell protrusion. Our results revealed that Hoxa10 plays an important role in survival and Hoxa11 contributes to MS formation in MLL-t acute myeloid leukemia with activating KRAS mutation.
Topics: Animals; Cell Movement; Homeodomain Proteins; Humans; Leukemia, Myeloid, Acute; Mice; Mutation; Oncogene Proteins, Fusion; Proto-Oncogene Proteins p21(ras); Sarcoma, Myeloid; Transcription Factors
PubMed: 35500545
DOI: 10.1016/j.neo.2022.100802 -
Journal of Clinical and Diagnostic... Jul 2017Myeloid sarcoma is an extramedullary manifestation of Acute Myeloid Leukaemia and sometimes is the only indicator of the disease. The incidence varies between 3-9.1% of...
Myeloid sarcoma is an extramedullary manifestation of Acute Myeloid Leukaemia and sometimes is the only indicator of the disease. The incidence varies between 3-9.1% of acute leukaemia cases. The blast infiltration is seen most commonly in skin, lymph node, gastrointestinal tract, bone, soft tissue though can involve any body site usually as a solitary lesion and is rarely seen in nasal cavity. We present two cases of myeloid sarcoma presenting as a nasal mass in a six year old girl and other as orbital mass in 32-year-old as an initial manifestation of acute myeloid leukaemia. Histopathological examination along with immunohistochemistry clinched the diagnosis of myeloid sarcoma. Examination of bone marrow aspirate revealed blasts which fulfilled the criteria for acute leukaemia. These cases are usually misdiagnosed because often lymphoma and granulocytic sarcoma is not considered in initial list of differential diagnoses. These rare cases are being presented here as early recognition and diagnosis will ensure rapid treatment of the condition and improve the survival.
PubMed: 28892915
DOI: 10.7860/JCDR/2017/20494.10256 -
Journal of Korean Neurosurgical Society Mar 2011Myeloid sarcoma is a solid, extramedullary tumor composed of leukemic myeloblasts or immature myeloid cells. Intraparenchymal myeloid sarcoma without the involvement of...
Myeloid sarcoma is a solid, extramedullary tumor composed of leukemic myeloblasts or immature myeloid cells. Intraparenchymal myeloid sarcoma without the involvement of the skull or meninges is extremely rare. Here, we present the case of a 49-year-old man who developed intraparenchymal myeloid sarcoma on the left cerebellum after allogeneic bone marrow transplantation (BMT). He received radiotherapy after complete removal of intraparenchymal myeloid sarcoma, but he was diagnosed spinal myeloid sarcoma three month later. Nine months after the operation, new intracranial and spinal myeloid sarcoma were diagnosed and the patient's condition had been worsened rapidly. Although the spinal myeloid sarcoma was not histologically diagnosed, this report provides valuable insights into the clinical course of progression of intraparenchymal myeloid sarcoma.
PubMed: 21556238
DOI: 10.3340/jkns.2011.49.3.171 -
The Tohoku Journal of Experimental... Jun 2021Myeloid sarcoma is a rare disease entity of extramedullary myeloid neoplasm that can occur both as an initial isolated myeloid sarcoma without leukemic cell invasion in...
Myeloid sarcoma is a rare disease entity of extramedullary myeloid neoplasm that can occur both as an initial isolated myeloid sarcoma without leukemic cell invasion in the peripheral blood and bone marrow, and as the secondary lesion of acute and chronic myeloid leukemias, myelodysplastic syndrome and chronic myeloproliferative neoplasms. Due to its rarity and its frequent emergence as the recurrent lesion after intensive systemic therapy, including allogeneic hematopoietic stem cell transplantation, the standard treatment has not been established for myeloid sarcoma. In this report, we presented an 84-year-old female patient with isolated myeloid sarcoma which progressed to myelodysplastic syndrome and systemic myeloid sarcoma despite various types of conventional anti-leukemic chemotherapies. However, the patient got a durable partial response by the monotherapy of azacitidine, a hypomethylating agent. She received thirteen courses of azacitidine therapy without progression. We discuss the possibility that hypomethylating agents are the novel effective and feasible therapeutic options for myeloid sarcoma, even in cases refractory to or relapsed after intensive systemic treatment. We also discuss the possible future development of hypomethylating agent-containing combinatory therapeutic strategy for myeloid sarcoma, given its direct anti-leukemic effect and immunomodulatory effect.
Topics: Aged, 80 and over; Azacitidine; Female; Hematopoietic Stem Cell Transplantation; Humans; Leukemia, Myeloid, Acute; Myelodysplastic Syndromes; Sarcoma, Myeloid
PubMed: 34148918
DOI: 10.1620/tjem.254.101 -
Medicine Sep 2017Myeloid sarcoma (MS) and leukemia cutis (LC) are extramedullary tumors comprising myeloid blasts. They can occur de novo or concurrently with hematological disorders,... (Review)
Review
RATIONALE
Myeloid sarcoma (MS) and leukemia cutis (LC) are extramedullary tumors comprising myeloid blasts. They can occur de novo or concurrently with hematological disorders, usually acute myeloid leukemia (AML). AML chemotherapy is generally the initial therapy for MS and LC, and hematopoietic stem cell transplantation (HSCT) can be considered as additional therapy. However, treatment for older patients who are unable to continue intensive chemotherapy is not currently standardized.
PATIENT CONCERNS
A 71-year-old Japanese woman was diagnosed with multiple MSs associated with myelodysplastic syndrome (MDS), using bone marrow aspiration and lymph node biopsy.
DIAGNOSES
Additionally, LC was diagnosed by skin biopsy. Extramedullary MS and LC lesions were formed by massive infiltration of myeloblastic cells.
INTERVENTIONS
Twenty courses of 5-azacytidine (5-Aza) were administrated as maintenance therapy after induction therapy with daunorubicin and cytarabine.
OUTCOMES
Myeloblasts decreased in the bone marrow and the LC disappeared after induction therapy. The MSs completely disappeared, except for the palatine tonsil lesion, after 5-Aza maintenance therapy. 5-Aza treatment provided long-term partial response for more than 21 months.
LESSONS
5-Aza was well tolerated and may be a good option for the treatment of MS and LC associated with MDS, especially in older patients who cannot receive HSCT.
Topics: Aged; Antimetabolites, Antineoplastic; Azacitidine; Diagnosis, Differential; Female; Humans; Leukemia; Myelodysplastic Syndromes; Sarcoma, Myeloid
PubMed: 28885352
DOI: 10.1097/MD.0000000000007975