One case of myeloid sarcoma with multiple space occupying lesions in the spinal canal / 中华神经科杂志

Myeloid sarcoma (MS) is a tumor mass formed by the proliferation of one or more myeloid primitive cells outside the marrow, which is mostly related to acute myeloid leukemia (AML). It is reported that 2.5% to 9.1% of AML patients have MS, and AML with spinal canal MS is very rare. Spinal canal MS often has an acute onset and is difficult to diagnose. It is easy to cause missed diagnosis and misdiagnosis, which will lead to a delay in accurate diagnosis seriously affecting the treatment and quality of life among these patients. The clinical data, diagnosis and treatment process of a case of MS with multiple space occupying lesions in the spinal canal diagnosed and treated by the Department of Hematology of Peking Union Medical College Hospital are reported, in order to provide reference for clinical workers.

Skin recurrence after remission of primary nasopharyngeal myeloid sarcoma: report of one case and review of literature / 白血病·淋巴瘤

Objective:To improve the awareness of primary myeloid sarcoma.Methods:The clinical data of one primary myeloid sarcoma patient with first symptom involving nasopharynx who was admitted to the Second Affiliated Hospital of Anhui Medical University in June 2017 were retrospectively analyzed, and the related literature was reviewed.Results:The patient presented with nasal congestion with tinnitus; it took half a year for the myeloid sarcoma diagnosis to be clear with biopsy and immunohistochemical examinations for many times. The patient was treated with chemotherapy regimens for acute myeloid leukemia (AML). Isolated myeloid sarcoma relapsed with skin masses after remission for more than 2 years. Further improvement of the bone marrow-related examinations showed that the bone marrow was not involved, and it was still isolated myeloid sarcoma. The skin mass disappeared completely after the induction chemotherapy of AML. Six months after the recurrence, the patient was in stable condition and was still under follow-up treatment.Conclusions:The primary nasopharyngeal myeloid sarcoma lacks specificity and is easy to be misdiagnosed. The prognosis of isolated myeloid sarcoma is better than that of AML. Isolated recurrence after chemotherapy remission is rare, and usually progresses to the leukemia stage quickly. Early and correct diagnosis is very important for the prognosis, and clinicians should improve the awareness of primary myeloid sarcoma.

Myeloid sarcoma in the breast in a patient withacute myeloid leukemia: a case report

Myeloid sarcoma infiltration into the breast of patients with acute myeloid leukemia is rare. The present study reports the case of a 56-year-old woman diagnosed with AML and an incidental finding of a breast tumor. The nodule biopsy raised the suspicion of invasive lobular carcinoma and poorly differentiated angiosarcoma. Subsequent immunohistochemical study concluded the diagnosis of myeloid sarcoma. The varied image presentations, the lack of knowledge of clinical data and complementary propaedeutics, and the histopathological similarity with certain primary breast lesions make it difficult to discover secondary infiltration by myeloid sarcoma in this unusual site

Myeloid sarcoma: an uncommon presentation of myeloid neoplasms; a case series of 4 rare cases reported in a tertiary care institute

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.

MRI features of childhood granulocytic sarcoma and literature review / 中国医学影像技术

Objective: To investigate the MRI findings of childhood granulocytic sarcoma (GS). Methods MRI data of 4 child patients with GS confirmed pathologically and immunohistochemistry were retrospectively analyzed. The location, size, morphology, signal characteristics, bone destruction and enhancement features of the tumor were observed. Results Among 4 patients, 3 cases were multiple, and 1 case was single. The lesion of 1 case was located in T3-T7 epidural intra-spinal canal and vertebral side, double side wall of maxillary sinus and sphenoid sinus, peri-orbit bone, of 1 case was located in L1-L2 epidural intra-spinal canal, of 1 case was located in T2-T4 and T10-T11 epidural intra-spinal canal, of 1 case was located in T11-T12 and L4-S2 level epidural intra-spinal canal, left side of the eye socket, sphenoid bone, and right side of the frontal sinus wall. Totally 4 cases were found soft tissue masses in the local or formed paravertebral lump through intervertebral foramen. The bone marrow signal of 3 cases was inhibited diffusely. MRI showed that T1WI signal slightly higher than normal surrounding muscles, while T2WI fat suppression showed slightly high signal intensity, and mild to moderate homogeneous enhancement. Two cases showed vertebral bone destruction, characterized by T1WI low signal obviously, T2WI fat suppression high signal obviously, and homogenous enhancement. Conclusion MRI features of GS of childhood have some characteristics, and they are helpful for diagnosis of GS.

Clinical characteristics of myeloid sarcoma:analyses of 12 cases / 白血病·淋巴瘤

Objective To investigate the clinical characteristics, curative effect and prognosis of myeloid sarcoma. Methods The clinical data of 12 patients with MS diagnosed at Xijing Hospital of Air Force Medical University from August 2008 to May 2018 were retrospectively analyzed. Their clinical manifestations, diagnosis, treatment and survival were analyzed. Results Twelve patients were 17 to 62 years old. The initial site included lymph node, external auditory canal, eye, buttock, lung, liver, pancreas, breast,skin, vertebra and its surroundings,and cervix. Among 11 patients with peripheral blood classification, bone marrow aspiration and bone marrow biopsy, 6 cases were isolated MS [one of which developed acute myeloid leukemia (AML)], 1 case was chronic myeloid leukemia in chronic phase, 2 cases were AML-M2, and 1 case was myelodysplastic syndrome (MDS), and 1 case was after aplastic anemia (AA) with no infiltration of bone marrow. Immunohistochemical results showed that LCA(+) (7/7), MPO(+) (12/12), CD43(+) (9/9), lysozyme(+) (5/7), CD3(-) (8/8), CD20(-) (9/9), CD34(+) (5/6), CD117(+) (7/7), and Ki-67(+) 30%-90%. Four patients were examined for bone marrow chromosomes, 2 patients with AML had t (8;21), 1 patient with MDS was 47, XX, +8, del(11)(q21), and 1 patient with CML was t(9;22). Two of the 12 patients were lost to follow-up. Among the 10 patients who were followed up, 6 died and 4 survived, and the median survival time was 21 months (2-27 months). Conclusions AA in stable phase with MS and CML in chronic phase with MS are rarely reported. The clinical manifestations of MS patients are varied, of which the common incidence sites are superficial lymph nodes, the infrequent sites are vertebra and its surrounding areas, and the rare sites are eye, pancreas, lung, liver, etc. The median survival time of MS patient is short and the curative effect is poor.

Male with myelofibrosis and ulceronecrotic lesions

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.

Cutaneous myeloid sarcoma associated with chronic myeloid leukemia

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.

A Case of Myeloid Sarcoma of Intestine / 대한소화기학회지

Myeloid sarcoma (MS) is an extramedullary involvement of immature myeloid proliferation. An isolated MS is defined as a myeloblastic tumor when it arises without any concomitant circulating disease. A diagnosis of MS is established using pathologic features including infiltration of myeloblasts and strong myeloperoxidase expression with negative cytokeratin immunohistochemical staining. We report a rare case of colonic MS without any peripheral blood abnormality. If the affected patient were left untreated, the MS could evolve into acute myeloid leukemia (AML) within one year. Several studies recommend the same regimens of chemotherapy as used for circulating AML to treat isolated MS. We focused on the diagnosis of MS in this study. The correct diagnosis of MS is important for adequate treatment. In conclusion, MS should be considered in the differential diagnosis of intestinal tumor.

Compresión medular por sarcoma granulocítico primario / Spinal cord compression due to nonleukemic granulocytic sarcoma

El sarcoma granulocítico (SG) es una lesión poco frecuente asociada a síndromes mielodisplásicos, mieloproliferativos o leucemias, aunque puede ser el primer hallazgo en un paciente previamente sano. Presentamos un SG que comenzó como compresión medular, en un paciente sin patología hematológica previa. Las imágenes radiológicas demostraron una lesión lítica en L1 que precisó cirugía urgente. Fue preciso realizar inmunohistoquímica de la muestra para llegar al diagnóstico. El aspirado medular no mostró evidencia de patología hematológica, siendo el SG la primera manifestación. El paciente recibió posteriormente tratamiento con quimioterapia y radioterapia, falleciendo 20 meses después del diagnóstico de una sepsis Pseudomonas aeruginosa intratratamiento de una leucemia mieloblástica. En resumen, el SG primario es un tumor infrecuente de difícil diagnóstico. Es necesario tener un alto grado de sospecha y solicitar amplios estudios inmunohistoquímicos para un diagnóstico correcto. El tratamiento debe ser precoz, agresivo e individualizado, ya que tiene mal pronóstico.

Granulocytic sarcoma (GS) is an infrequent lesion associated with myelodysplastic or myeloproliferative disorders or leukemia, although it may be the first finding in an otherwise healthy patient. A case of GS is described that presented as spinal cord compression, in a patient with no underlying hematological disorder. Imaging studies disclosed a single lytic lesion in L1, which required emergency surgery. Immunohistochemical staining of the surgical biopsy sample was needed for diagnosis. Bone marrow aspirate was unremarkable. The patient received chemo-radiotherapy, dying 20 months after diagnosis of Pseudomonas aeruginosa sepsis during treatment of acute myelogenous leukemia. In short, primary GS is an infrequent and difficult to diagnose tumor. A high degree of suspicion, along with extensive immunohistochemical studies are necessary for diagnosis. Treatment should be prompt, aggressive and individualized, since the prognosis is very poor.

Myeloid Sarcoma of Peritoneum in Acute Myeloid Leukemia Patient with Inversion of Chromosome 16 / 대한내과학회지

Myeloid sarcoma is an extramedullary myeloid neoplasm that usually involves the skin, soft tissues, and lymph nodes. Myeloid sarcoma is found in 2.5-9.1% of acute myeloid leukemia patients, usually those with t (8;21), while inv (16) is rarely associated with myeloid sarcoma. Consequently, little is known of the characteristics and incidence of inv (16) in myeloid sarcoma. Myeloid sarcoma in acute myeloid leukemia patients with inv (16) is most often found in the abdominal lesions; the intestinal tract is involved most commonly, in the form of a mass. Here, we report an unusual myeloid sarcoma presenting as peritoneal carcinomatosis in acute myeloid leukemia with inv (16) that appeared to be ascites.

Analysis on clinical characteristics of 10 patients with granulocytic sarcoma / 白血病·淋巴瘤

Objective To explore the clinical characteristics,diagnosis,therapy and prognostic features of granulocytic sarcoma (GS).Methods Retrospective analysis was used to analyse 10 cases who were diagnosed with GS.Results Five patients were diagnosed with primary GS.GS was accompanied by APL in one case,CML in 3 cases and MDS in one case.Patients were treated with resection and chemotherapy,or imatinib treatment.Follow-up data were available from 9 patients,with 2 patients being still alive.The survival of one patient who received high-dose cytarabine chemotherapy was 66 months.The other 7patients died of tumor-related diseases,and their survival ranged from 4 to 17 months.Conclusion GS is a rarely diagnosed disease.A correct diagnosis of GS depends on detailed morphological examination and immunohistochemical study.The clinical outcome of patients is poor,and AML-type chemotherapy is the proper treatment for GS.High-intensity chemotherapy and hematopoietic stem cell transplantation might improve long-term survival.

Testicular myeloid sarcoma: case report

Myeloid sarcomas are extramedullary solid tumors composed of immature granulocytic precursor cells. In association with acute myeloid leukemia and other myeloproliferative disorders, they may arise concurrently with compromised bone marrow related to acute myeloid leukemia, as a relapsed presentation, or occur as the first manifestation. The testicles are considered to be an uncommon site for myeloid sarcomas. No therapeutic strategy has been defined as best but may include chemotherapy, radiotherapy and/or hematopoietic stem cell transplantation. This study reports the evolution of a patient with testicular myeloid sarcoma as the first manifestation of acute myeloid leukemia. The patient initially refused medical treatment and died five months after the clinical condition started.

Multiple Jejunal Myeloid Sarcomas Presenting with Intestinal Obstruction in a Non-leukemic Patient: A Case Report with Ultrastructural Observations

Myeloid sarcoma is a rare extramedullary myeloid tumor, which is frequently misdiagnosed when no evidence of leukemia is initially observed. Here, we report on a peculiar case of a 49-year-old man afflicted with multiple masses in the jejunum, the superior mesentery, and the serosa of the transverse colon, without leukemic manifestation. The tumor was composed of undifferentiated small round cells containing eosinophilic cytoplasm, which were negative for myeloperoxidase, nonspecific esterase, lysozyme, terminal deoxynucleotidyl transferase, leukocyte common antigen, CD3, CD4, CD15, CD20, CD30, CD43, CD56, CD68/PG-M1, CD79a, human melanoma black-45, c-kit, and CD34 with positivity only for CD68/KP1, CD99, and vimentin. Under electron microscopy, those cells had abundant membrane-bound cytoplasmic granules that measured 200 to 300 nm in diameter, which were consistent with granulocytic azurophilic granules. The tumor was finally diagnosed as a myeloid sarcoma. The presence of non-leukemic myeloid sarcomas showing immunonegativity for conventional myeloid-leukemic markers necessitated a diagnosis by ultrastructural observation.

Acute promyelocytic leukemia presenting as an extradural mass

Acute promyelocytic leukemia is potentially a highly curable type of leukemia that usually presents with pancytopenia, coagulopathies and bleeding. We describe a case of an unusual presentation of acute promyelocytic leukemia. A 53 year-old male was admitted complaining of pain and weakness in his legs. He presented at examination a spastic paraparesis with a sensitive level at the eighth thoracic medullar (T8) segment. Magnetic resonance imaging showed a posterolateral extradural mass from T6 through T8 segments with medullar compression. A complete blood count showed anemia, thrombocytopenia and the presence of promyelocytes and blasts. Marrow examination was compatible with the diagnosis of acute promyelocytic leukemia by cytogenetics and polymerase chain reaction for the PML-RARα gene. He was treated with all-trans-retinoic acid therapy plus daunorubicin and presented an all-trans-retinoic acid syndrome. Despite hematological remission, the patient presented neurologic deterioration and had to be treated with radiotherapy (total dose 3000 cGy) of the extradural lesion. The patient evolved with severe sepsis and died without any recovery from his neurologic deficit. Extramedullary infiltration is a very rare complication in acute promyelocytic leukemia. Most cases are related to relapse after initial treatment with all-trans-retinoic acid. The skin and the central nervous system are the most frequently involved sites. This is possibly the first case reported of this condition in which the patient had a symptomatic extradural mass.

Sarcoma mieloide: Caso clínico con trisomia 11 / Myeloid sarcoma: Report of one case with trisomy 11

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.

Leucemia Cutis de presentación atípica / An atypical presentation of Leukemia Cutis

El trabajo aporta un nuevo caso de leucemia cutis, raro en frecuencia, que presenta un comienzo clínico muy poco habitual, a tener en cuenta. Fueron necesarias varias biopsias para llegar al diagnóstico de la paciente. La supervivencia de la paciente está por encima de la media en estos casos.

The work presents a new case of leukemia cutis, rare in frequency, which has a very unusual clinical onset to take into account. It took several biopsies for the diagnosis of the patient. The survival of the patient is above average in these cases.

Collision Tumor Composed of a Granulocytic Sarcoma and an Adenocarcinoma of the Stomach: A Case Report

Granulocytic sarcoma, also called chloroma or myeloblastoma, is an extramedullary invasive tumor composed of neoplastic myeloid cells. In this report, we describe a 43-year-old male patient with a collision tumor composed of an adenocarcinoma and a granulocytic sarcoma in the stomach. The coexistence of a granulocytic sarcoma and adenocarcinoma in the stomach has, to the best of our knowledge, not been reported in the literature. The diagnosis of granulocytic sarcoma is very difficult; especially in the absence of concurrent hematologic disease or in the uncommon setting of coexistence with another tumor. Cautious observation is needed when a finding of unusual atypical cells admixed with an adenocarcinoma in the stomach is confronted.