Treatment Outcomes of Intracranial Myeloid Sarcomas: A Meta-Analysis.

OBJECTIVE: Intracranial myeloid sarcomas (IMS) are rare central nervous system manifestations of malignant hematopoietic neoplasms of myeloid origin such as acute myeloid leukemia and chronic myeloid leukemia. Reported cases in the literature are limited to primarily case reports. We present a systematic review of this rare central nervous system tumor, characterizing the clinical presentation, tumor location, histopathology, and available treatment modalities. We correlate these variables with mortality, recurrence, and complications to suggest optimal management strategies for IMS. METHODS: A systematic literature search was performed across Ovid MEDLINE, Scopus, and Embase using 14 search terms in accordance to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. This systematic review examines variables such as patient age, tumor location, size, presenting symptoms, treatment modality, extent of resection, and mortality. We performed descriptive analyses to identify bivariate associations between patient characteristics, treatment, and outcomes. RESULTS: The mean age at diagnosis was 34.8 years, and the most common etiology was acute myeloid leukemia (68.8%). The most common presenting symptoms were headache (45.5%), vision complaints (27.3%), and weakness/motor symptoms (21.2%). IMS were most commonly located in the temporal lobe (10.1%), cerebellum (10.1%), or falcine/parasagittal (10.1%) region. Patients who received radiotherapy (P < 0.001) or chemotherapy (P < 0.001) had lower rates of mortality versus those who did not. Surgical treatment and extent of resection were not significantly associated with mortality (P > 0.05). CONCLUSION: The use of adjuvant radiotherapy or chemotherapy for IMS significantly reduces mortality, confirming IMS as a cranial manifestation of a systemic disease. Although surgical treatment is indicated for histopathologic diagnosis and to relieve mass effect, the extent of resection does not predict overall survival.

Nonleukemic granulocytic sarcoma of orbit after blunt trauma: A case report and review of literature.

RATIONALE: Granulocytic sarcoma without invasion of bone marrow or blood is very rare. The diagnosis of it is usually overlooked and the treatment has not reached a consensus. Meanwhile, the onset of this kind of disease is not clear. PATIENT CONCERNS: Diagnose patients in early stage and help choose the right treatment strategies. DIAGNOSES: The ultimate diagnosis was nonleukemic granulocytic sarcoma after blunt trauma. INTERVENTIONS: Surgery was the initial treatment option. Chemotherapy including idarubicin (70 mg, D1-D3) and cytosine arabinoside (100 mg, D1-D7) and radiotherapy of total 3,060 cGy were then administered but failed to control the disease. Hematopoietic stem cell transplantation was finally administered. OUTCOMES: No evidence of disease progression or spread according to the latest follow-up. LESSONS: The etiology of nonleukemic granulocytic still remains unclear, though trauma seems to be a potential predisposing factor and deserves more attention for early diagnosis and timely and proper treatment. Systemic chemotherapy is more effective than radiotherapy or surgery. Hematopoietic stem cell transplantation is an alternative choice after the failure of chemotherapy.

Acute myeloid leukaemia masquerading as a primary CNS tumour.

In children with newly diagnosed acute myeloid leukaemia (AML), myeloid sarcomas (MS) of the central nervous system (CNS) are rare. Since MS involving the CNS are potentially curable, timely recognition is paramount. Establishing a diagnosis may be problematic as they can easily mimic primary CNS neoplasms. We report the case of a 5-year-old boy with AML with t(8;21)(q22;q22) rearrangement who presented with a massive intracranial MS and rapid clinical deterioration suggestive of a meningioma or a primitive neuroectodermal tumour. The peripheral smear showed blasts with Auer rods. Urgent chemotherapy was started for AML with presumptive CNS MS. The mass resolved with chemotherapy, and treatment was consolidated with radiotherapy. Although exceedingly rare, this case highlights the potential for MS to present similarly to a primary CNS tumour. MS should be part of the differential diagnosis as part of a CNS mass, particularly if the complete blood count is abnormal.

Myeloid sarcoma of the skin in a patient with myelodysplastic syndrome.

We report the case of a 76-year-old woman who presented with asymptomatic extensive erythematous. Firm plaques were noted over the right cheek. Complete blood count was normal, as was a peripheral smear. An excision biopsy taken from the cheek showed infiltration of the dermis and hypodermis with atypical cells which were strongly positive for human leukocyte antigen (HLA-DR) and lysozyme and were moderately myeloperoxidase (MPO) enzyme. The results of immunohistochemical staining for CD34, CD117, CD3, CD4, CD8, CD20, CD23, CD56, and ALK-1 were negative. Bone marrow analysis indicated myelodysplastic syndrome RAEB 1 while cytogenetic finding showed tetrasomy 8. It was recommended that the patient undergo local radiotherapy of skin lesions, but she refused and was lost to follow-up.

Is acute myeloid leukemia a liquid tumor?

Extramedullary manifestations of acute myeloid leukemia (AML) were described as early as the 19th century. However, the incidence, clinical significance and pathobiology of extramedullary AML remain ill defined. We reviewed case reports, retrospective case series, pilot studies and imaging studies of extramedullary leukemia (EML) to determine its frequency, characteristics, clinical presentation and significance. EML precedes or accompanies development of AML and occurs during or following treatment, even during remission. Although imaging studies are rarely conducted and the true incidence of EML has yet to be verified, authors have reported several estimates based on retrospective and autopsy studies. The incidence of EML in patients with AML of all ages is estimated to be about 9% and EML in children with AML was detected in 40% of patients at diagnosis. The combination of positron emission tomography and computed tomography were the most sensitive and reliable techniques of detecting and monitoring EML. Based on our literature review, the frequency of EML is likely underreported. The well-documented nature of EML in patients with AML suggests that AML can manifest as a solid tumor. The extent to which EML accompanies AML and whether EML is derived from bone marrow are unknown. Furthermore, questions remain regarding the role of the microenvironment, which may or may not facilitate the survival and proliferation of EML, and the implications of these interactions with regard to minimal residual disease, tumor cell quiescence and relapse. Therefore, prospective studies of detection and characterization of EML in patients with AML are warranted.

Mediastinal granulocytic sarcoma: poor risk AML?

Granulocytic sarcoma (GS), a rare extramedullary manifestation of acute myeloid leukaemia (AML) has been frequently reported in the skin, orbits, gingiva, maxilla and lymph nodes. GS in the mediastinum is often mistaken as lymphoblastic lymphoma or mediastinal germ cell tumour. We presented here three cases of AML with normal cytogenetics who presented with mediastinal masses with superior vena caval (SVC) syndrome. All the three cases summarised in this write-up, received standard AML therapy but had, poor outcome.

Myeloid sarcoma and adenocarcinoma of the large bowel as collision tumors: a case report.

Myeloid sarcoma is a rare tumor composed of myeloid cells, localized in an extramedullary site, which may be associated with a concurrent myeloid neoplasm involving the bone marrow, although such an association is not required. Most patients present with acute myeloid leukemia and their prognosis is poor. We describe the case of a 76-year old woman with an adenocarcinoma of the right colon infiltrating the subserosa synchronous with a myeloid sarcoma at the same site; one pericolic lymph node was infiltrated by both tumors. The peculiarities of this case are the clinical presentation (as an acute abdomen due to subserosa infiltration by the myeloid sarcoma), the coexistence of a myeloid sarcoma with an adenocarcinoma of the right colon, and the absence of progression to acute leukemia. Coexistence of myeloid sarcoma and adenocarcinoma in the colon is probably incidental, and so it appears likely that the two different tumours arose from different mechanisms. However, a possible common background is conceivable. Some authors have found that p53 has a pivotal role in driving the maturation of myeloid stem cells and p53 is, also, involved in colon carcinogenesis. In our case, it may be hypothesized that synchronous heterogeneous mutations occurred in different types of committed cells or in stem cells secondary to p53 loss. Since only one case report has evaluated the correlation between myeloid sarcoma and adenocarcinoma of the large bowel, further immunohistochemical and molecular studies are needed to clarify the pathogenetic relationship between them.

Postsplenectomy sclerosing extramedullary hematopoietic tumor with unexpected good clinical evolution: morphologic, immunohistochemical, and molecular analysis of one case and review of the literature.

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.

Myeloid sarcoma: extramedullary manifestation of myeloid disorders.

Myeloid sarcoma (MS), also termed extramedullary acute myeloid leukemia, extramedullary myeloid tumor, and granulocytic sarcoma or chloroma, is a rare manifestation that is characterized by the occurrence of 1 or more tumor myeloid masses occurring at an extramedullary site. The wide spectrum of this disorder and the conditions that it overlaps diagnostically were well reflected in the 25 cases submitted to the Society for Hematopathology/European Association for Haematopathology Workshop held in Indianapolis, IN, in November 2007. This review, on the one hand, focuses on the definition and most recent achievements on the pathobiology of MS, and on the other, also in the light of the revised World Health Organization classification, summarizes the main features of a representative series of this condition aiming to provide readers a useful document for daily practice.

Disfonía y lumbalgia como presentación de sarcoma granulocítico. Primera manifestación de leucemia mieloide aguda / Dysphonia and lumbago as signs of granulocytic sarcoma. Initial manifestation of acute myeloid leukaemia

El sarcoma granulocítico o cloroma es un tumor de células primitivas de la serie granulocítica que se desarrolla en una localización extramedular. Ha sido descrito en pacientes con leucemia aguda y en síndromes mieloproliferativos crónicos en transformación leucémica, en un porcentaje bajo de casos, generalmente como forma de presentación. Su diagnóstico se basa en la sospecha, casi siempre dificultosa si no se considera dentro del diagnóstico diferencial, dado que habitualmente sus manifestaciones clínicas se refieren a la zona afectada enmascarando la etiología de los mismos. Presentamos el caso de un varón de 78 años que es derivado al servicio de urgencias hospitalarias desde su centro de atención primaria para valoración de disfonía, lumbalgia y malestar general de un mes de evolución, sin respuesta a tratamiento convencional (AU)

Granulocytic sarcoma or chloroma is an extramedullar tumor consisting of primitive granulocytic cells. It has been described in patients with acute leukaemia and chronic myeloproliferative syndromes transforming into leukaemia. It is rare and generally an initial manifestation of leukaemia. Diagnosis is based on suspicion and is always difficult if it is not considered in the differential diagnosis procedure because the clinical manifestations in the affected area often mask their aetiology. We contribute the case of a 78 year old man who was referred to the hospital emergency department from his primary care centre for evaluation. He had suffered from dysphonia, lumbago and general malaise for a month and had not responded to conventional treatment (AU)

Granulocytic sarcoma in a patient with essential thrombocythemia presented as acute spinal cord compression--case report and review of the literature.

Granulocytic sarcoma (GS) is a rare solid tumor of myeloid origin, which usually precedes or occurs concurrently with myeloid leukemia, or with other types of myeloproliferative and myelodysplastic disorders. Spinal affections of GS have been described but are uncommon, particularly in association with essential thrombocythemia. We present a case of a 75-year-old woman with a long history of essential thrombocythemia who developed 2 tumors: 1 in the bodies of T3 - 6 vertebras extending epidurally, and the other in the right frontal lobe, adherent to dura, thus, mimicking meningioma. The patient died because of massive pulmonary thrombembolia. Microscopical and immunohistochemical features of spinal and intracranial tumor samples obtained at autopsy were consistent with the diagnosis of GS with focal megakaryocytic differentiation. Clinicians and pathologists should be aware of this rare tumor being so diverse in its clinical presentation, as well as in microscopical and immunohistochemical features. Careful evaluation of morphology, in conjunction with immunohistochemistry for evidence of myeloid differentiation are required to avoid frequent errors in diagnostics of GS. The suggested panel includes chloroacetate esterase, myeloperoxidase, lysozyme, CD117, CD43, CD79a and CD3. Only early correct diagnosis will enable proper treatment which may be successful despite the highly malignant potential of GS.