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Myeloid sarcoma (MS) is considered as an extramedullary manifestation of acute myeloid leukemia (AML) with or without concurrent AML. It can present at any age and any site, however, nasopharynx being an extremely rare site of manifestation. MS may precede AML by weeks, months or years, thereby necessitating an early diagnosis and timely intervention and treatment. We report a case of MS in a young female who presented with nasal obstruction and epistaxis for 3 months. The present case also highlights the significance of judicious use of immunohistochemistry panel while dealing with a hematolymphoid neoplasm devoid of expression of B-cell or T cell specific markers in head and neck region.
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Myeloid sarcoma (MS) is an extramedullary proliferation of immature myeloid cells which may occur as a progression of myelodysplastic syndrome (MDS), myeloproliferative neoplasm (MPN), or myelodysplastic syndrome/myeloproliferative neoplasm (MDS/MPN) and as acute myeloid leukemia (AML) relapse. Rarely may it be de novo. Lymph nodes, skin, lungs, intestine are the commonly involved sites. However, an isolated pancreatic MS is seldom reported in the literature. Herein, we report one such case which was misdiagnosed as pancreatic adenocarcinoma on the clinico-radiological examination which misled us away from preoperative diagnostic sampling, and a Whipple pancreaticoduodenectomy was performed. Histopathological examination in conjunction with immunohistochemistry revealed the final diagnosis of isolated MS of the pancreas. We emphasize that although rare, a clinical suspicion along with preoperative histopathological examination may lead to early diagnosis, targeted management, and a better clinical outcome in such cases.
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Objective:To explore the clinical characteristics, treatment and prognosis of myeloid sarcoma(MS).Methods:From January 2010 to May 2019, clinical data were reviewed for 89 MS cases. Age, gender, site of onset, type, comorbid diseases, lymphatic characteristics and disease remission status were analyzed. And 1-year survival rates were explored for different treatments including whether or not chemotherapy, transplantation and using hypomethylated drugs(HMAs)for maintenance after transplantation.Results:Among them, 21 cases had the data of chromosome karyotypic analysis and next generation sequencing and 8 patients underwent allogeneic hematopoietic stem cell transplantation(allo-HSCT). The 1-year overall survival rates(OS)of primary MS, MS with intramedullary disease and MS relapse after leukemic remission were 16.0%, 37.5% and 36.9% respectively( P=0.013). The 1-year OS of local treatment(surgical resection, intrathecal injection and local radiotherapy), chemotherapy plus local treatment and chemotherapy plus allo-HSCT was 0, 28.1% and 72.9% respectively( P=0.003). After two courses of treatment, the 1-year OS of patients with complete and incomplete remissions were 34.9% and 10.0% respectively( P=0.008). Half(4/8)MS patients relapsed within 1 year after transplantation and had a short survival.Three patients received decitabine after HSCT and all of them survived for a long time. Conclusions:Chemotherapy plus HSCT is efficacious for MS. Decitabine maintenance treatment after transplantation may prolong recurrence-free survival. However, a larger sample size is required for further clinical verifications.
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granulocytic sarcoma (GS) is rare in the breast, histologically and immunophenotypically similar to myelogenous leukemia, radiologically lacking specificity, similar to breast cancer or mammary abscess. It should not be misdiagnosed as invasive cancer, especially in the case of frozen diagnosis, leading to excessive surgery. Three cases of GS were collected, specimens were fully drawn, microscopic pathologic examinations and immunohistochemistry (SP method) granulocytic sarcoma of breast were performed. The clinicopathological, immunohistochemical features, diagnosis and prognosis of GS are discussed to improve the awareness of the disease.
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PURPOSE: To report two cases with recurrent involvement of the optic nerve as the initial sign of acute leukemic relapse.CASE SUMMARY: An 8-year-old male with acute lymphoblastic leukemia on the maintenance chemotherapy was referred for a decrease in visual acuity in the right eye. The visual acuity and optic disc swelling were completely resolved with high-dose steroid therapy. Two months after the initial presentation, the symptoms recurred and brain/orbit magnetic resonance imaging (MRI) showed high intensity along the right optic nerve from the retrobulbar area to the optic chiasm. The visual acuity was restored after high-dose steroid therapy. One month after the second attack, the symptoms recurred and the cerebrospinal fluid cytology was positive for lymphoblasts. Three weeks after the intrathecal chemotherapy, the visual acuity improved fully, but optic disc atrophy developed. A 45-year-old male, who received allogenic peripheral blood stem cell transplantation for acute myeloid leukemia, presented with a decrease in visual acuity in the left eye. The left optic disc swelling improved with high-dose steroid therapy, but the medication was restarted due to the recurrence of symptoms 3 weeks later. Brain MRI showed a mass lesion compressing the left optic nerve, presumed to be a myeloid sarcoma. One month after local irradiation, the visual acuity was no light perception in the left eye.CONCLUSIONS: In patients with a prior history of acute leukemia, the recurrent involvement of the optic nerve should be considered as a central nerve system relapse, regardless of improvement with steroid treatment.
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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.
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@#Myeloid sarcoma (MS) is an uncommon type of malignancy, and its diagnosis is comparable to acute myeloid leukaemia (AML). In the rare circumstances in which MS does present without AML, it is known as MS de novo. We report a case of a 10-month old child who presented with bilateral proptosis and a pelvic mass due to synchronous primary MS de novo. She was initially misdiagnosed with neuroblastoma, which has this typical presentation. The histopathological result from the biopsy of the orbital mass also showed a small blue round cell tumour (SBRCT) as seen in cases of neuroblastoma. However, the diagnosis of MS was confirmed using immunohistochemistry (IHC) from the orbital biopsy specimen, which usually plays a major role in the diagnosis of orbital tumours and as a prognostic indicator. Our patient remains in clinical remission two years after antileukemic treatment, with no relapse or progression to AML.
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Sarcoma, MyeloidABSTRACT
@#AIM: To investigate the clinic opathological features of orbital soft tissue tumors.<p>METHODS: A retrospective analysis of 455 cases of orbital soft tissue tumors in our hospital from 2003-11/2018-11 were performed to observe the clinical features, pathological classification and pathological features of rare tumors.<p>RESULTS:All 455 patients with orbital soft tissue tumors, 421(92.5%)were benign tumors. The top 5 were 258 cases of cavernous hemangioma, 58 cases of capillary hemangioma, 16 cases of neurofibroma, 15 cases of fibroid, and 14 cases of schwannoma; 27 cases(5.9%)of intermediate type, including 23 cases of solitary fibrous tumor(SFT), 2 cases of low-grade malignant fibrous histiocytoma, 2 cases of low-grade mucinous neurofibroma; 7 cases(1.5%)of malignant type, 2 cases of orbital malignant SFT, 2 cases of orbital myeloid sarcoma(MS), mucinous liposarcoma(MLS), spindle cell undifferentiated sarcoma and peripheral primitive neuroectodermal tumor(PNET)1 case. Immunohistochemistry and molecular detection of some cases revealed that the application of new antibodies such as STAT6 and molecular detection techniques can improve the diagnostic accuracy.<p>CONCLUSION:Benign tumors account for the majority of orbital soft tissue tumors. Cavernous hemangioma in vascular tumors is the first, and there are few soft tissue tumor pathological types such as Erdheim-Chester disease(ECD)and granulomatous ossifying fibers. Tumors(POF), leiomyomas and myxomas; intermediate and malignant tumors are rare; pathological types such as SFT, MLS, spindle cell undifferentiated sarcoma and MS are often difficult to diagnose, easy to miss diagnosis and misdiagnosis, new immunity histochemical antibodies and molecular detection techniques can improve the accuracy of the diagnosis.
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Objective@#To investigate the clinical manifestations, pathological features, diagnosis and treatment of myeloid sarcoma, and to improve the understanding of myeloid sarcoma.@*Methods@#The clinical data, diagnosis and treatment of 7 patients with myeloid sarcoma were retrospectively analyzed.@*Results@#Of the 7 patients with myeloid sarcoma, 1 was male and 6 were female. In most patients, the local compression symptoms caused by painless local masses or masses were the first manifestations. One patient had lesions involving the cervix and vaginal bleeding was the first symptom. The lesions were extensive with 19 sites involved. The positive proportion of immunohistochemical staining was 6/6 for CD43, 6/7 for MPO, 4/5 for CD117, 4/4 for LCA, 3/5 for CD34 and 2/2 for CD99. Lymphocyte markers CD3 and CD20 were negative in all 7 patients.@*Conclusions@#Myeloid sarcoma is a rare hematological malignancy. Early diagnosis and active treatment are the key to improve prognosis. Current treatments include systemic chemotherapy, surgical resection, radiation therapy, and hematopoietic stem cell transplantation.
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RESUMEN Introducción y objetivos: El sarcoma mieloide puede ser la primera manifestación de la leucemia mieloide aguda (LMA), presentarse simultáneamente o constituir una forma de recaída. Material y métodos: Presentamos el caso de una paciente con sarcoma mieloide uterino, como forma de recaída de LMA. Resultados: El diagnóstico se basa en los hallazgos histopatológicos, la inmunohistoquímica y el inmunofenotipo. El tratamiento y el pronóstico son similares a LMA. Conclusión: La afectación uterina por leucemia mieloide extramedular es rara pero debe tenerse en cuenta en el diagnostico diferencial de una masa uterina en aquellas pacientes con antecedentes de LMA.
ABSTRACT Introduction and objectives: Myeloid Sarcoma can present as a first MLA sign, concurrently with or at relapse form. Materials and methods: We present the case of a patient with uterine myeloid sarcoma, as a form of relapse of MLA. Results: The diagnostic is based on the histopathology findings along with immunohistochemistry and immunophenotyping. Conclusion: Uterine involvement due to extramedullary myeloid leukemia is rare but it should be taken into account in the differential diagnosis of a uterine mass in those patients with a history of MLA.
Subject(s)
Humans , Female , Aged , Leukemia, Myeloid, Acute/complications , Leukemia, Myeloid, Acute/therapy , Sarcoma, Myeloid/diagnostic imaging , Thrombosis , Immunohistochemistry , Magnetic Resonance Spectroscopy , Leukemia, Myeloid, Acute/pathologyABSTRACT
Objective To observe the efficacy of maintenance treatment with decitabine and dasatinib after allogenic hematopoietic stem cell transplantation for myeloid sarcoma.Methods A 29-year-old male patient was diagnosed with abdominal myeloid sarcoma and acute myeloid leukemia with c-kit mutation and t(8;21).Allogeneic hematopoietic stem cell transplantation was performed after inducted remission.The conditioning regimen was decitabine + FLAG + modified Bu/Cy.Prophylaxis of GVHD was performed with cyclosporine mycophenolate mofetil and short-term methotrexate.The patient received 11.73 × 108 mononucleated cells/kg and 17.59 × 106CD34+ cells/kg from donor.At Day 13 post-transplantation,neutrophils reached 0.5 × 109/L and platelet count was 20 × 109/L.Decitabine was prescribed since Day 50 post-transplantation monthly for 5 courses.And dasatinib was offered orally since Day 100 for 4 months.Results It was followed up to 16 months post-transplantation.There were no obvious abnormalities of bone marrow cytology,AML/ETO fusion gene quantification,cerebrospinal fluid or abdominal enhanced computed tomography (CT).Conclusions Hematopoietic stem cell transplantation is an effective treatment for myeloid sarcoma.Decitabine has some efficacy for myeloid sarcoma and it may be used for maintenance treatment after transplantation.Tyrosine kinase inhibitors reduce recurrence in myeloid sarcoma with c-kit mutation.The treatment of decitabine and dasatinib after allogeneic hematopoietic stem cell transplantation yield excellent outcomes.This is the first report in domestic and foreign literatures.
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Objective: To describe the diagnosis, treatment and prognosis of acute promyelocytic leukemia (APL) patients with myeloid sarcoma (MS). Methods: The clinical data including the diagnosis and treatment of two patients with APL and MS were reported, and the related literature was reviewed. Results: Case 1 was an APL patient with MS as the initial symptom. The puncture biopsy and immunohistochemistry for a right chest wall mass showed it was in accordance with MS. The system treatment with all-trans retinoic acid (ATRA), arsenic trioxide (ATO) and idarubicin was given to this patient. After one cycle of chemotherapy, the mass on the right chest wall disappeared, and the bone marrow examination showed the complete remission. Then this patient maintained treatment until May 2018. Case 2 was diagnosed as extramedullary recurrence of APL with MS 25-years later. The puncture biopsy and immunohistochemistry for a parasternal mass showed it was in accordance with MS. After one cycle of chemothrapy with ATRA, idarubicin and cytarabine, the parasternal mass remarkably decreased. However, this patient stopped using ATRA after discharging from hospital, then died on 3 April 2017, which were known by phone call following-up. Conclusion: The incidence of APL with MS is low, but the degree of malignancy is high. ATRA and ATO combined chemotherapy is effective, but the overall prognosis is poor.
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Objective@#To analyze the diagnosis and treatment of acute myeloid leukemia (AML) complicated with myeloid sarcoma (MS).@*Methods@#The clinical features, diagnosis and therapy of 3 AML who were treated with routine regimen induction and consolidation therapy in Shanghai Beizhan Hospital and showed MS during the marrow remission stage were retrospectively analyzed.@*Results@#Three female patients included 2 cases of CBFβ-MYH11 positive and 1 case of AML1-ETO positive. One patient occurred in the first complete remission(CR1) and 2 patients occurred in CR2. The involved sites included brain, vagina, and breast respectively. Three patients all received short efficacy after chemotherapy, including 1 patient in bone marrow remission stage, 1 patient died of after bone marrow relapse and 1 patient without follow-up.@*Conclusions@#For AML patients in remission with extramedullary lesions, pathological examination is warranted to confirm the diagnosis of the disease. Systemic chemotherapy is still the main treatment for MS. Central nervous system(CNS) leukemia treatment should be performed to the patients with CNS involved. Allogeneic hematopoietic stem cell transplantation may be the best option for the treatment of these patients.
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BACKGROUND: Isolated myeloid sarcoma (MS) is a rare extramedullary tumor mass composed of malignant myeloid precursor cells without any evidence of leukemia in the peripheral blood and bone marrow. We describe the clinical characteristics and outcomes of patients diagnosed with isolated MS at our institution. METHODS: We retrospectively reviewed 9 of 497 acute myeloid leukemia (AML) patients (1.8%) with isolated MS. Isolated MS patients were divided into 2 groups according to the first-line treatment strategy: systemic treatment only (S) or local treatment with or without systemic treatment (LS). RESULTS: The most common site of MS occurrence was the head and neck area (N=4, 44.4%), followed by the anterior mediastinum (N=2, 22.2%) and the gastrointestinal tract (N=2, 22.2%). The tumors of 4 patients (44.4%) eventually evolved to AML, in a median time of 13.4 months (range, 2.4–20.1 mo). The number of patients achieving complete remission after first-line treatment was higher in the LS group (N=5, 83.3%) than in the S group (N=1, 33.3%) (P =0.226). All patients in the LS group survived, but those in the S group died (P=0.012). CONCLUSION: Accurate and rapid diagnosis using various modalities and the early initiation of intensive combined treatment may be the optimal strategies to reduce the risk of isolated MS subsequently evolving to AML. To fully understand the characteristics of isolated MS, a larger number of patients from a multinational study is necessary.
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Humans , Bone Marrow , Diagnosis , Gastrointestinal Tract , Head , Leukemia , Leukemia, Myeloid, Acute , Mediastinum , Neck , Retrospective Studies , Sarcoma, MyeloidABSTRACT
Myeloid sarcoma is a rare tumor mass consisting of immature granulocytic cells occurring in an extramedullary site or in a bone. It has often been observed during the course of an acute leukemia, myelodysplastic syndrome or myeloproliferative neoplasms, and it can involve any site of the body. However, it rarely present in the absence of bone marrow infiltration, especially for the isolated spinal myeloid sarcoma. In this report, we describe a case of isolated myeloid sarcoma that showed spinal compression. A 66-year-old male, with no underlying disease or medication history, presented with a progressive back pain and numbness in bilateral lower extremities that had begun two weeks before. He was diagnosed with myeloid sarcoma with no evidence of bone marrow involvement. Tumor cells were positive for CD34, c-KIT, and Bcl-2 on the immunohistochemical stain. He was treated with systemic chemotherapy with daunorubicin plus cytosine arabinoside and achieved a partial response.
Subject(s)
Aged , Humans , Male , Back Pain , Bone Marrow , Cytarabine , Daunorubicin , Drug Therapy , Hypesthesia , Leukemia , Lower Extremity , Myelodysplastic Syndromes , Sarcoma, Myeloid , Spinal Cord CompressionABSTRACT
PURPOSE: To report a case of an 82-year-old male with acute myeloid leukemia presenting with bilateral isolated conjunctival and eyelid masses. CASE SUMMARY: An 82-year-old male presented with a bilateral conjunctival mass and right eyelid mass occurring 10 days prior. He was diagnosed with prostate cancer 8 years ago and complete recovery was achieved using selective mass chemotherapy. He experienced a stroke 4 years ago and was treated using a carotid artery stent insertion and medication. In the initial laboratory test, hemoglobin was 13.7 g/dL and leukocyte count 5,530/mm3(neutrophil 74.4%, lymphocyte 10%, monocyte 11.8%). Light reflex, movement of extraocular muscle and fundus examination were all normal. Biopsy was performed 1 week after the first visit. Seven days after biopsy, he complained of sudden dyspnea and febrile sense and was admitted to the intensive care unit via the emergency room (ER). The laboratory tests performed in the ER showed hemoglobin was 9.6 g/dL and leukocyte count was 78,020/mm3(neutrophil 0%, lymphocyte 7%, monocyte 5%, promyelocyte 1%, metamyelocyte 4%, myelocyte 6%, blast 67%). The biopsy revealed diffuse proliferation of atypical plasmacytoid cells, consistent with leukemic infiltration. Under the diagnosis of acute myeloid leukemia, chemotherapy was administered. However, the patient died due to aggravated pneumonia. CONCLUSIONS: Even if non-specific findings appear on the peripheral blood tests, eyelid and conjunctival masses should be considered as possible tumors in acute myeloid leukemia.
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Aged, 80 and over , Humans , Male , Biopsy , Carotid Arteries , Conjunctiva , Diagnosis , Drug Therapy , Dyspnea , Emergency Service, Hospital , Eyelids , Granulocyte Precursor Cells , Hematologic Tests , Intensive Care Units , Leukemia, Myeloid, Acute , Leukemic Infiltration , Leukocyte Count , Lymphocytes , Monocytes , Pneumonia , Prostatic Neoplasms , Reflex , Sarcoma, Myeloid , Stents , StrokeABSTRACT
Myeloid sarcoma (MS) is an extramedullary solid neoplasm of immature myeloid cells. These tumours usually develop in concurrence with or following acute leukemia. The breast is an uncommon site for presentation of this tumour, where it is often misdiagnosed as lymphoma or carcinoma.A 33- year-old female presented with a right breast lump in a private hospital, which was diagnosed as ductal carcinoma on lumpectomy. Subsequently she developed a lump in the left breast and a similar diagnosis of carcinoma was made on biopsy. A left mastectomy was performed. Histopathological examination revealed a tumour composed of mononuclear cells arranged in sheets and cords with round to oval vesicular nuclei and occasional prominent nucleoli. IHC for CK was very weak and focal. The tumour cells were immunonegative for ER, PR, Her2neu,epithelial membrane antigen, e-cadherin, CD3 and CD20. Diffuse immunopositivity for myeloperoxidase, CD34 and CD117 established a diagnosis of myeloid sarcoma. A histopathological review of the right breast lesion, with immunohistochemistry, also confirmed the diagnosis of myeloid sarcoma. Investigatory workup for acute myeloid leukemia, including bone marrow aspirate and biopsy and karyotypic studies, proved negative. The patient was treated with high dose cytarabine (HDAC) regimen and was disease free during the 12-month follow-up.Although extremely rare, awareness of such a presentation is crucial. This case also illustrates that careful histopathological review along with an expanded panel of immunohistochemistry is extremely important for recognizing such cases as a misdiagnosis can lead to unnecessary surgery and inappropriate therapy.
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Myeloid Sarcoma1 (also termed as chloroma, granulocytic sarcoma, extra medullary myeloid tumor) is a rare extra medullary tumor composed of immature myeloid cells (myeloblast)2. It is usually associated with leukemia or other myeloproliferative disorder. Myeloid Sarcoma in the central nervous system, around the brain stem is the commonest site of presentation and require high suspicion for diagnosis. We report a forty years male patient with history of dysphagia, dysphonia for last two months. MRI showed chloromas around the brain stem. Laboratory investigations revealed the presence of AML. This is a rare case of Myeloid Sarcoma around the brain stem in a patient of AML.
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10.3969/j.issn.1000-8179.2013.12.013
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Myeloid sarcoma is an uncommon extramedullary tumor of immature myeloid cells or myeloblasts. It may occur alone or concurrently with an underlying hematological malignancy. Although it can develop anywhere in the body, common sites include bones, particularly the skull and vertebra, soft tissues, and lymph nodes. However, there have been few reports of myeloid sarcoma occurring in the respiratory system, especially the large airways. We describe a case of endobronchial relapse of acute myeloid leukemia in a patient who achieved complete remission after allogeneic stem cell transplantation. To our knowledge, this is the first such report in Korea.