ABSTRACT
An extramedullary myeloid tumor or chloroma is an infrequent manifestation of a myeloid neoplasm. It is considered an equivalent to an acute myeloid leukemia. It is confirmed through biopsy, where infiltrating neoplastic myeloid cells distort the parenchyma. A total of twenty-nine cases were diagnosed as MS between 198 and 2023. Upon re-evaluation, only fourteen cases fulfilled the criteria for MS. The most common differential diagnosis were lymphomas, leukemic infiltration, and extramedullary hematopoiesis. Few were isolated cases; the rest were in the context of progression of a myeloid neoplasm. The majority had a myelomonocytic morphology and immunophenotype. The most reliable markers were CD45, HLA-DR, CD68 and CD4. The study highlights the complexity and impact of an accurate diagnosis of a myeloid sarcoma.
Subject(s)
Leukemia, Myeloid, Acute , Sarcoma, Myeloid , Humans , Sarcoma, Myeloid/diagnosis , Sarcoma, Myeloid/pathology , Leukemia, Myeloid, Acute/diagnosis , Diagnosis, Differential , Biopsy , ImmunophenotypingABSTRACT
Myeloid sarcoma (MS) is a rare extramedullary neoplasm of myeloid cells, which can arise before, concurrently with, or following hematolymphoid malignancies. We report 04 such cases of MS, diagnosed in this institute over a period of 6 years, during various phases of their respective myeloid neoplasms/leukemias. These cases include MS occurring as a relapse of AML (Case 1), MS occurring as an initial presentation of CML (Case 2), MS occurring during ongoing chemotherapy in APML (Case 3), and MS presenting as a progression of MDS to AML (Case 4). In the absence of relevant clinical history and unemployment of appropriate immunohistochemical (IHC) studies, these cases have a high risk of being frequently misdiagnosed either as Non-Hodgkin's Lymphoma (NHL) or small round cell tumors or undifferentiated carcinomas, which may further delay their management, making an already bad prognosis worse. This case series has been designed to throw light on the varied presentation of MS and the lineage differentiation of its neoplastic cells through the application of relevant IHC markers along with their clinical correlation.
Subject(s)
Humans , Male , Female , Child, Preschool , Adolescent , Middle Aged , Aged , Sarcoma, Myeloid/pathology , Myelodysplastic Syndromes/pathology , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/pathology , Leukemia, Myeloid, Acute/pathology , Leukemia, Promyelocytic, Acute/pathology , Diagnostic Errors/prevention & controlABSTRACT
Leukemic cells are rarely present in the oral cavity, and there are very few reports regarding such cases. However, we identified some reports of leukemic cells infiltrating tissues in the oral cavity, including gingival involvement. Recurrent painful oral ulcerations and prominent generalized periodontal destruction are the most common oral features of neutrophil disorders, and they may even be the initial symptoms of the disease. The ulcers may affect any part of the oral mucosa, including the tongue and palate. The objective of this report is to describe and discuss a case of myeloid sarcoma in the oral cavity of a 48-year-old male patient.
Subject(s)
Humans , Male , Middle Aged , Leukemia , Sarcoma, Myeloid/pathology , Pathology, OralABSTRACT
OBJECTIVE: Granulocytic sarcoma also called myeloid sarcoma is an extramedullary tumour of immature granulocytic cells. It is a rare entity, and mostly accompanied by acute myeloid leukaemia. It is observed during the course of myeloproliferative disorders especially in chronic myeloid leukaemia and myelodysplastic syndromes. CONCLUSIONS: Here, we report a case of a 60-year-old male with past history of myelofibrosis admitted to the emergency room due ulceronecrotic lesions, fever and dysphagia. We emphasize the importance of recognizing this entity and its severity.
Subject(s)
Primary Myelofibrosis/pathology , Sarcoma, Myeloid/pathology , Humans , Immunohistochemistry , Male , Middle Aged , Primary Myelofibrosis/complications , Sarcoma, Myeloid/complicationsABSTRACT
SUMMARY Granulocytic sarcoma also called myeloid sarcoma is an extramedullary tumour of immature granulocytic cells. It is a rare entity, and mostly accompanied by acute myeloid leukaemia. It is observed during the course of myeloproliferative disorders especially in chronic myeloid leukaemia and myelodysplastic syndromes. Here, we report a case of a 60-year-old male with past history of myelofibrosis admitted to the emergency room due ulceronecrotic lesions, fever and dysphagia. We emphasize the importance of recognizing this entity and its severity.
RESUMO O sarcoma granulocítico, também chamado de sarcoma mieloide, é um tumor extramedular de células granulocíticas ¡maturas. É uma entidade rara, e principalmente acompanhada de leucemia mieloide aguda. É observado durante o transtorno mieloproliferativo, especialmente na leucemia mieloide crónica e síndromes mielodisplásicas. Aqui, relatamos um caso de um homem de 60 anos com antecedente de mielofibrose admitida na sala de emergência devido a lesões ulceronecróticas, febre e disfagia. Enfatizamos a importância de reconhecer essa entidade e sua gravidade.
Subject(s)
Male , Sarcoma, Myeloid/pathology , Primary Myelofibrosis/pathology , Immunohistochemistry , Sarcoma, Myeloid/complications , Primary Myelofibrosis/complications , Middle AgedABSTRACT
Myeloid sarcoma is an extramedullary tumor of malignant myeloid cells often associated with acute myeloid leukemia, chronic myeloproliferative disorders and myelodysplastic syndromes. The skin is one of the most commonly affected sites. We report a rare case of cutaneous myeloid sarcoma associated with chronic myeloid leukemia.
Subject(s)
Leukemia, Myelogenous, Chronic, BCR-ABL Positive/pathology , Neoplasms, Multiple Primary/pathology , Sarcoma, Myeloid/pathology , Skin Neoplasms/pathology , Adult , Biopsy , Bone Marrow/pathology , Female , Humans , Immunohistochemistry , Rare Diseases/complications , Rare Diseases/pathology , Skin/pathologyABSTRACT
Abstract: Myeloid sarcoma is an extramedullary tumor of malignant myeloid cells often associated with acute myeloid leukemia, chronic myeloproliferative disorders and myelodysplastic syndromes. The skin is one of the most commonly affected sites. We report a rare case of cutaneous myeloid sarcoma associated with chronic myeloid leukemia.
Subject(s)
Humans , Female , Adult , Skin Neoplasms/pathology , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/pathology , Sarcoma, Myeloid/pathology , Neoplasms, Multiple Primary/pathology , Skin/pathology , Biopsy , Bone Marrow/pathology , Immunohistochemistry , Rare Diseases/complications , Rare Diseases/pathologyABSTRACT
Two cases of granulocytic sarcoma (GS) with lymph node presentation without marrow involvement are presented because it is a rare presentation of acute myeloid leukemia (AML) that can coexist with or precede it and as initial symptom (case 1) or disease relapse (case 2). The most common differential diagnoses are lymphoma or solid tumor. Biopsy is essential for definitive diagnosis. Imaging studies such as positron emission tomography (PET) are very useful for staging and monitoring. The prognosis and treatment according to the literature is not different from AML. The two cases presented were diagnosed with lymph node biopsy with a bone marrow that did not meet criteria for AML.
Subject(s)
Lymph Nodes/pathology , Sarcoma, Myeloid/pathology , Adult , Diagnosis, Differential , Female , Humans , Male , Middle AgedABSTRACT
A case of granulocytic sarcoma originating from an eosinophilic lineage is described in a 5-year-old, mixed-breed, female pig. The pig had been originally sent to slaughter in a good plane of nutrition and without displaying clinical disease. At gross examination, green masses were observed in several bones, especially vertebrae, sternum, pelvis, and long bones such as femur and humerus. Similar masses were seen in skeletal muscles, lymph nodes, and kidneys. Cytology revealed large numbers of round cells with round nuclei and scant cytoplasm (myelocytes); some of these cells had a fine eosinophilic granularity to their cytoplasm (eosinophil myelocytes). Histologically, the neoplastic cells formed sheets that completely obliterated the normal architecture of subperiosteal bone marrow. The cytoplasm of the neoplastic cells stained strongly by Sirius red stain of eosinophil and was positively marked by immunohistochemistry using an anti-myeloperoxidase antibody. The association of gross examination, cytology, histology, histochemistry, and immunohistochemistry findings is consistent with a diagnosis of eosinophilic granulocytic sarcoma.
Subject(s)
Bone Marrow Neoplasms/veterinary , Eosinophilia/veterinary , Sarcoma, Myeloid/veterinary , Swine Diseases/pathology , Animals , Bone Marrow Neoplasms/pathology , Eosinophilia/pathology , Fatal Outcome , Female , Immunohistochemistry/veterinary , Sarcoma, Myeloid/pathology , SwineABSTRACT
Myeloid sarcoma is a form of extra-medullary myeloid neoplasia. Cytogenetic characterization is hampered in the absence of invasion to the bone marrow, origin of cells that are usually studied in cytogenetic studies. We report a 13 years old mole presenting with a mass in the right shoulder. A biopsy of the tumor disclosed a Myeloid Sarcoma. A conventional cytogenetic study of a bone marrow aspirate did not show t (8;21) translocation. A fluorescent in situ hybridization (FISH) performed in the paraffin embedded biopsy of the tumor, detected a chromosome 11 trisomy.
Subject(s)
Chromosomes, Human, Pair 11 , Sarcoma, Myeloid/genetics , Sarcoma, Myeloid/pathology , Trisomy/diagnosis , Adolescent , Biopsy , Humans , In Situ Hybridization, Fluorescence , MaleABSTRACT
Myeloid sarcoma is a form of extra-medullary myeloid neoplasia. Cytogenetic characterization is hampered in the absence of invasion to the bone marrow, origin of cells that are usually studied in cytogenetic studies. We report a 13years old mole presenting with a mass in the right shoulder. A biopsy of the tumor disclosed a Myeloid Sarcoma. A conventional cytogenetic study of a bone marrow aspirate did not show t (8;21) translocation. A fluorescent in situ hybridization (FISH) performed in the paraffin embedded biopsy of the tumor, detected a chromosome 11 trisomy.
Subject(s)
Adolescent , Humans , Male , Sarcoma, Myeloid/genetics , Sarcoma, Myeloid/pathology , Trisomy/diagnosis , Biopsy , In Situ Hybridization, FluorescenceABSTRACT
An 18-year-old male underwent an allogeneic hematopoietic stem cell transplantation (allo-HSCT) for chronic myeloid leukemia (CML) in the first late chronic phase. On day 132, he was readmitted to the hospital with nausea, vomiting and nodular lesions on endoscopy. A diagnosis of granulocytic sarcoma of the stomach was made. Bone marrow cytogenetic analysis for the Philadelphia chromosome and nested polymerase chain reaction for BCR-ABL1 were both negative. Immunosuppression was abruptly discontinued, and by day 180, all gastric lesions had completely disappeared. However, there were histological signs of graft-versus-host disease. The patient developed progressive anorexia and elevated hepatic enzymes, which prompted the reintroduction of cyclosporine. Considering the risk of another relapse, imatinib mesylate (IM) 600 mg/day was started. The patient Is condition improved, and there was no evidence of disease recurrence at 36 months after relapse. Relapse of CML is the commonest cause of treatment failure after allo-HSCT. On rare occasions, a localized extramedullary presentation is seen. Unless properly treated, other extramedullary relapse sites and/or marrow infiltration usually occur. Withdrawal of immunosuppression, along with IM therapy seems to be an acceptable approach in this setting.
Subject(s)
Antineoplastic Agents/administration & dosage , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/therapy , Neoplasms, Second Primary , Piperazines/administration & dosage , Pyrimidines/administration & dosage , Sarcoma, Myeloid , Stomach Neoplasms , Adolescent , Benzamides , Hematopoietic Stem Cell Transplantation , Humans , Imatinib Mesylate , Immunosuppression Therapy/adverse effects , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/pathology , Male , Neoplasms, Second Primary/drug therapy , Neoplasms, Second Primary/pathology , Sarcoma, Myeloid/drug therapy , Sarcoma, Myeloid/pathology , Stomach Neoplasms/drug therapy , Stomach Neoplasms/pathology , Transplantation, HomologousABSTRACT
OBJECTIVE: To describe 2 cases of myeloid sarcoma of the vagina, in a patient without a history of acute myeloid leukemia (AML) and in another whose condition was previously diagnosed with AML. MATERIALS AND METHODS: The clinical histories of 2 patients whose conditions were diagnosed with myeloid sarcoma of the vagina were obtained from their medical records. RESULTS: Case 1: A 77-year-old woman with no systemic illnesses presented with a vaginal lump. Clinically, there was a 6-cm periurethral mass that was examined by biopsy. The histopathologic specimen was evaluated on routine and immunohistochemical stains, and myeloid sarcoma was diagnosed after extensive immunohistochemical analysis. The patient was treated with pelvic radiation. She developed extensive myeloid sarcoma of the skin and AML 4.5 months later; she died 2 weeks later, 5 months after the initial presentation. Case 2: A 36-year-old woman with a known history of AML who has had multiple leukemic and extramedullary recurrences presented with a pelvic mass. Physical findings revealed large masses in the vagina and rectovaginal septum, which were confirmed as myeloid sarcoma after biopsy and histologic examination. The patient was treated with pelvic/vaginal radiation. Five months later, she had another leukemic relapse and died within 1 day of palliative chemotherapy. CONCLUSIONS: Myeloid sarcoma of the vagina is extremely rare. Most patients have a poor prognosis and either have a history of or will subsequently develop AML.
Subject(s)
Sarcoma, Myeloid/diagnosis , Vagina/pathology , Vaginal Neoplasms/diagnosis , Adult , Aged , Antigens, Neoplasm , Fatal Outcome , Female , Humans , Leukemia, Myeloid, Acute/complications , Sarcoma, Myeloid/pathology , Sarcoma, Myeloid/radiotherapy , Skin Neoplasms/secondary , Vaginal Neoplasms/pathology , Vaginal Neoplasms/radiotherapyABSTRACT
Sclerosing extramedullary hematopoietic tumor has been described as a rare manifestation of chronic myeloproliferative neoplasm. The lack of knowledge about this entity has caused it to be mistaken for many types of nonhematopoietic and hematopoietic tumors. We present the case of a 71-year-old lady with a long history of primary myelofibrosis, which developed multiple abdominal sclerosing extramedullary hematopoietic tumors with good clinical evolution. Nonchronic myeloid leukemia myeloproliferative neoplasm included a JAK2 mutation as part of the diagnosis algorithm. Particularly, idiopathic myelofibrosis is related with a JAK2 mutation in 50% of the cases with a pejorative prognosis. The absence of JAK2 demonstrated in the paraffin samples of the tumors may be related to the unusual evolution in this particular case. Morphologically differential diagnoses considered in the evaluation of this entity and in our case included sarcomas mainly liposarcoma, anaplastic carcinoma, and Hodgkin lymphoma.
Subject(s)
Bone Marrow/pathology , Janus Kinase 2/genetics , Primary Myelofibrosis/diagnosis , Sarcoma, Myeloid/diagnosis , Aged , Biomarkers, Tumor/metabolism , Bone Marrow/metabolism , DNA Mutational Analysis , Diagnosis, Differential , Female , Humans , Immunochemistry , Janus Kinase 2/immunology , Janus Kinase 2/metabolism , Mutation/genetics , Primary Myelofibrosis/complications , Primary Myelofibrosis/genetics , Primary Myelofibrosis/pathology , Primary Myelofibrosis/physiopathology , Prognosis , Sarcoma, Myeloid/etiology , Sarcoma, Myeloid/genetics , Sarcoma, Myeloid/pathology , Sarcoma, Myeloid/physiopathology , Sclerosis , SplenectomyABSTRACT
UNLABELLED: Granulocytic sarcoma (GS) is a localized infiltrate of immature granulocytes in an extramedullary site. This lesion is most frequently associated with leukemia, but can occur associated with other myeloproliferative disorders. GS can affect virtually any part of the body, but oral lesions are rare, with only 37 cases described. CASE DESCRIPTION: We present a case of GS in a 23-year-old female, with a prior history of acute myeloid leukemia, presenting with a solitary mandibular swelling in the region of the erupting lower left 3rd molar. After biopsy, conventional immunohistochemical stains were positive for CD45 (hematological marker) and myeloid markers, such as myeloperoxidase, and CD68, demonstrating a myeloid lineage with monocytic cell differentiation, suggesting the diagnosis of GS associated to AML-M5. CLINICAL IMPLICATION: Although GS is a rare tumor in the oral cavity, and its diagnosis is usually difficult, the clinician must know of its existence in order to make a differential diagnosis.
Subject(s)
Mouth Neoplasms , Sarcoma, Myeloid , Female , Humans , Mouth Neoplasms/pathology , Sarcoma, Myeloid/pathology , Young AdultABSTRACT
Granulocytic sarcoma (GS), an uncommon solid extramedullary tumour, should be considered even in the absence of leukaemia, as delay in diagnosis and treatment worsens the prognosis. We present a GS (single humeral bone lesion) in a non-leukaemia patient, treated with intensive AML (Acute Myeloid Leukaemia) chemotherapy and sequential radiotherapy, in complete response 26 months after diagnosis, confirmed by histopathology and without leukaemia progression.
Subject(s)
Bone Neoplasms/diagnosis , Humerus/pathology , Sarcoma, Myeloid/diagnosis , Aged , Antineoplastic Agents/therapeutic use , Arthroplasty, Replacement , Bone Neoplasms/complications , Bone Neoplasms/drug therapy , Bone Neoplasms/pathology , Bone Neoplasms/radiotherapy , Bone Neoplasms/surgery , Combined Modality Therapy , Cytarabine/administration & dosage , Female , Fractures, Spontaneous/etiology , Fractures, Spontaneous/surgery , Humans , Humerus/surgery , Idarubicin/administration & dosage , Osteolysis/etiology , Remission Induction , Sarcoma, Myeloid/complications , Sarcoma, Myeloid/drug therapy , Sarcoma, Myeloid/pathology , Sarcoma, Myeloid/radiotherapy , Sarcoma, Myeloid/surgery , Shoulder Fractures/etiology , Shoulder Fractures/surgeryABSTRACT
Granulocytic sarcoma is an extramedullary tumor of immature cells of granulocytic series, generally associated to acute myelogenous leukemia. The skin is one of the most commonly affected sites. Granulocytic sarcoma can complicate myelodysplastic syndromes and is considered a sign of poor prognosis. They are often misdiagnosed with non-Hodgkin lymphoma of the lymphoblastic type, Burkitt lymphoma and large cell lymphoma. In children, the differential diagnoses also include small, round cell tumors. It is important to diagnose these lesions early because they can precede peripheral blood and bone marrow transformation to acute myelogenous leukemia. We report a case of an elderly patient with myelodysplastic syndrome who developed multiple cutaneous granulocytic sarcoma lesions and discuss prognostic and treatment implications.
Subject(s)
Myelodysplastic Syndromes/pathology , Sarcoma, Myeloid/pathology , Skin Neoplasms/pathology , Aged, 80 and over , Bone Marrow Cells/pathology , Fatal Outcome , Humans , Male , Myelodysplastic Syndromes/complications , Myelodysplastic Syndromes/therapy , Neoplasms, Multiple Primary , Platelet Transfusion , Sarcoma, Myeloid/complications , Sarcoma, Myeloid/therapy , Skin Neoplasms/complications , Skin Neoplasms/therapyABSTRACT
Sarcoma granulocítico é tumor sólido, constituído por células precursoras de granulócitos, localizado em sítio extra medular. Geralmente surge precedendo uma leucemia mielóide aguda ou concomitantemente a ela, sendo considerado fator de mau prognóstico. Tem como principais modalidades terapêuticas a radioterapia e a quimioterapia. Um caso de sarcoma granulocítico intracraniano ocorrido seis meses após transplante de medula óssea por leucemia mielóide aguda é relatado. A paciente apresentava cefaléia e hemiplegia esquerda, causada por extensa lesäo fronto-parietal direita. Após a ressecçäo completa do tumor, houve total recuperaçäo do déficit neurológico. A paciente completou o tratamento radio e quimioterápico, estando livre de doença após três meses de acompanhamento. O manejo cirúrgico do sarcoma granulocítico é modalidade adjuvante, indicada quando o efeito compressivo tumoral determina déficit neurológico. É o meio mais rápido de descompressäo do tecido nervoso, possibilitando a chance de recuperaçäo funcional, melhorando a qualidade de vida do paciente