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BACKGROUND: Myeloid sarcoma (MS) is a rare hematologic malignancy defined as an extramedullary tumor of immature granulocytic cells. It can occur as primary or de novo and be associated with myelodysplasia or myeloproliferative neoplasms. The most frequent locations are the skin, lymph nodes and bones. The case of a patient with a diagnosis of primary granulocytic de novo gastric MS is reported. CASE SUMMARY: A 19-year-old female patient with MS, whose abdominal computed tomography showed a bulky tumor of 16.5 cm in the gastric chamber with infiltration in the retroperitoneal, pancreatic and bile duct region; the histological study showed gastric mucosa diffusely infiltrated by mononucleated cells and the immunohistochemistry expressed myeloperoxidase. After receiving induction chemotherapy based on the 3 + 7 regimen (daunorubicin/cytarabine), the patient developed severe hematological toxicity and neutropenic typhlitis which required a prolonged medical treatment. She presented a rapid disease progression. Although she received supportive treatment, the patient died. CONCLUSION: Gastric primary de novo MS is a rare and aggressive course neoplasm, fostering knowledge is very important to decide its management and to promote more approaches focused on understanding this pathology and its particularities in our population.

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Severely immunocompromised patients are at increased risk for uncommon infectious diseases with atypical presentations. Fusarium sp., has been reported in patients with hematological malignancies and prompt diagnosis is necessary due to high mortality. We report a myelodysplastic syndrome (MDS) patient who presented Fusarium solani infection associated with granulocytic sarcoma as an initial presentation of acute myeloid leukemia (AML) transformation. We performed histological examination, immunohistochemistry analysis, culture of the biopsy tissue and DNA sequencing to make a conclusive diagnosis of F. solani and granulocytic sarcoma, reinforcing the necessity of performing complete evaluation of skin lesions in immunocompromised patients.

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Sarcoma mielóide é uma massa tumoral de céluas mielóides imaturas ou células granulocíticas que afeta locais anatómicos extramedulares, incluindo a cavidade oral com pouca frequência. Uma mulher de 24 anos de idade, foi encaminhada para avaliação de um núdulo gengival doloroso de rápido crescimento com duração de 2 semanas, associada a febre, fadiga e linfadenopatia cervical. O exame intra-oral mostrou um nódulo azulado em região posterior inferior da gengiva à direita exibindo superfície necrótica. A biópsia da lesão gengival mostrou infiltração difusa de células tumorais indiferenciadas com aparência granulocítica, fortemente imuno positivo para CD99, mieloperoxidase e Ki-67 (60%), e negativo para CD20, CD3, CD34 e TdT. Os exames de sangue apresentaram uma pancitopenia grave, e análise genética confirmou o diagnóstico de leucemia promielocítica aguda. O diagnóstico final foi de sarcoma mielóide oral associado a leucemia promielocítica aguda com t (15; 17). A paciente foi submetida à quimioterapia, mas morreu da doença um mês depois. As características clinicopatológicas e imuno-histoquímico do presente caso são comparadas com os 84 casos de sarcoma mielóide oral, previamente relatados na literatura de língua Inglês. (AU)

Myeloid sarcoma is a tumor mass of immature myeloid or granulocytic cells that affects extramedullary anatomic sites, including uncommonly the oral cavity. A 24-year-old female was referred for evaluation of a fast growing painful gingival swelling lasting 2 weeks, associated with fever, fatigue, and cervical lymphadenopathy. Intraoral examination showed a bluish swelling on the right posterior lower gingiva exhibiting necrotic surface. Incisional biopsy of the gingival lesion displayed diffuse infiltration of undifferentiated tumor cells with granulocytic appearance, strongly immunopositive for CD99, myeloperoxidase and Ki-67 (60%), and negative for CD20, CD3, CD34 and TdT. Blood tests presented a severe pancytopenia, and genetic analysis confirmed the diagnosis of acute promyelocytic leukemia. The final diagnosis was of oral myeloid sarcoma associated with acute promyelocytic leukemia with t(15;17). The patient was submitted to chemotherapy but died of the disease one month later. The clinicopathologic and immunohistochemical features of the present case are compared with the 84 cases of oral myeloid sarcoma previously reported in the English-language literature. (AU)

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Neoplasmas em suínos são raros. Esse trabalho descreve os neoplasmas encontrados em suínos na rotina diagnóstica de um laboratório de patologia veterinária localizado na Região Central do Rio Grande do Sul. Durante um período de 49 anos, 2.266 casos de várias afecções em suínos foram encontrados, dos quais 37 (1,6%) eram neoplasmas. Em ordem decrescente de frequência, os seguintes neoplasmas foram encontrados: Linfoma (11/37), nefroblastoma (11/37), melanoma (8/37) e papiloma (2/37). Adicionalmente, um caso de cada um dos seguintes tumores foi observado: Adenoma hepatocelular, carcinoma hepatocelular, colangiocarcinoma, histiocitoma fibroso maligno e sarcoma granulocítico. O aspecto macroscópico e histológico desses tumores é descrito e a sua epidemiologia é comparada com os dados disponíveis na literatura para neoplasia suína.

Neoplasms in swine are rare. This paper describes neoplasms found in swine in the diagnostic routine of a veterinary pathology laboratory in the Central Region of Rio Grande do Sul, Brazil during a 49-year period, during which 2,266 cases of the various affections in swine were diagnosed. Of those 37 cases (1.6%) were neoplasms. In decreasing order of prevalence, the following neoplasms were found: lymphoma (11 out of 37 cases), nephroblastoma (11/37), melanoma (8/37), and papilloma (2/37). Adenoma hepatocelular, carcinoma hepatocelular, cholangiocarcinoma, malignant fibrous histiocytoma, granulocytic sarcoma were each found in one case out of the 37 cases. The gross and histological aspects of these tumors are described and their epidemiology is compared with the data available in the literature for neoplasia in swine.

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Neoplasmas em suínos são raros. Esse trabalho descreve os neoplasmas encontrados em suínos na rotina diagnóstica de um laboratório de patologia veterinária localizado na Região Central do Rio Grande do Sul. Durante um período de 49 anos, 2.266 casos de várias afecções em suínos foram encontrados, dos quais 37 (1,6%) eram neoplasmas. Em ordem decrescente de frequência, os seguintes neoplasmas foram encontrados: Linfoma (11/37), nefroblastoma (11/37), melanoma (8/37) e papiloma (2/37). Adicionalmente, um caso de cada um dos seguintes tumores foi observado: Adenoma hepatocelular, carcinoma hepatocelular, colangiocarcinoma, histiocitoma fibroso maligno e sarcoma granulocítico. O aspecto macroscópico e histológico desses tumores é descrito e a sua epidemiologia é comparada com os dados disponíveis na literatura para neoplasia suína.(AU)

Neoplasms in swine are rare. This paper describes neoplasms found in swine in the diagnostic routine of a veterinary pathology laboratory in the Central Region of Rio Grande do Sul, Brazil during a 49-year period, during which 2,266 cases of the various affections in swine were diagnosed. Of those 37 cases (1.6%) were neoplasms. In decreasing order of prevalence, the following neoplasms were found: lymphoma (11 out of 37 cases), nephroblastoma (11/37), melanoma (8/37), and papilloma (2/37). Adenoma hepatocelular, carcinoma hepatocelular, cholangiocarcinoma, malignant fibrous histiocytoma, granulocytic sarcoma were each found in one case out of the 37 cases. The gross and histological aspects of these tumors are described and their epidemiology is compared with the data available in the literature for neoplasia in swine.(AU)

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El sarcoma granulicítico primario ginecológico es una rara neoplasia maligna de difícil diagnóstico. En este reporte describimos el caso de una mujer de 27 años con sarcoma granulosítico en ausencia de enfermedad hematológica demostrable. Asimismo, se presenta una revisión de la literatura concerniente al diagnóstico de esta enfermedad.

Primary gynecologic granulocytic sarcoma is a rare malignancy with a complex diagnosis. In this report we describe the case of a 27- year –old woman with granulocytic sarcoma in the absence of demonstrable hematologic disease. A review of the literature is also presented, regarding the diagnosis of this diseases.

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El sarcoma granulocítico es un tumor localizado poco común, compuesto de células granulocíticas inmaduras. Generalmente se presenta en pacientes con leucemia mieloide aguda, síndromes mielodisplásicos o leucemia mieloide crónica. Puede ocurrir en cualquier localización anatómica. Reportamos el caso de una mujer de 41 años con el diagnóstico de leucemia mieloide crónica que presentó edema en brazo derecho que no remitía a pesar de tratamiento antibiótico y fasciotomía. El estudio histológico de la biopsia la lesión tomada mostró un sarcoma granulocítico. En este trabajo se revisa la citogenética, presentación clínica, diagnóstico y pronóstico de este síndrome tumoral.

Granulocytic sarcoma is an uncommon and localized extramedullary tumor composed of immature granulocytic cells. It may present in patiens with acute myeloid leukaemia, myelodysplastic syndrome or chronic myelogenous leukaemia. It may occur in any anatomical site. We report on the case of a 41 year old female diagnosed with chronic myelogenous leukaemia who presented with right arm swelling that did not resolve after antibiotic and surgical fasciotomy. The histological examination showed granulocytic sarcoma. In this report the citogenetics, clinical presentation, diagnosis and outcome of granulocytic sarcoma was reviewed.

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Introducción: el sarcoma granulocítico (SG) es una neoplasia maligna cuya incidencia es de 2,9% a3,1% en pacientes con leucemia mieloide (LM) o enfermedades mieloproliferativas. Se presentahabitualmente en hombres y en población africana, asiática y suramericana. Objetivo: describir las características imaginológicas del SG en la cara y el cráneo de cinco niños y jóvenes. Pacientes y métodos: presentamos cinco pacientes con SG en la cara y la base del cráneo. A cuatro de ellos se les hizo tomografía computarizada (TC) y a tres, resonancia magnética (RM) cerebral. Cuatro presentaron una masa de tejido blando en la órbita, dos tenían afectación ósea y otro reveló lesión en el sistema nervioso central. En cuatro se diagnosticó leucemia mieloide aguda (LMA) .Conclusión: el SG puede manifestarse con invasión orbitaria y craneofacial en niños y adultos jóvenes. Usualmente los pacientes consultan por proptosis y edema orbitario. Con este cuadro clínico el SG es la primera probabilidad diagnóstica en el contexto de la LMA o las mielodisplasias. En otras situaciones clínicas se debe hacer diagnóstico diferencial con complicaciones de sinusitis, rabdomiosarcoma, linfoma de la órbita y otras neoplasias. La imaginología demuestra invasión de tejidos blandos e infiltración ósea. Es muy característico del SG afectar en un comienzo la pared lateral o la superior de la órbita. En algunos casos simula abscesos. El diagnóstico se confirma por histopatología.

Introduction: Granulocytic sarcoma (GS) is a rare malignant neoplasia, with an incidence rate of 2.9% to 3.1% in patients with myeloid leukemia or myeloproliferative diseases. Usually it affects males, of African, Asian and South American populations. Objetive: To describe the radiological characteristics of GS in the face and skull of children and young people. Patients and methods: We report five patients with GS in the face and skull. Computerized tomography (CT) was carried out in four and orbital and brain magnetic resonance (MRI) in three. Out of the five, four had soft tissue masses in the orbit, two had bone infiltration, and in one there was a central nervous system lesion. In four patients acute myeloid leukemia was demonstrated. Conclusion: GS may affect the orbit, the face and the skull of children and young adults; it manifests with exophthalmia and orbital edema. It must be considered as a diagnostic possibility in the context of myeloid leukemia and myelodysplasias. Otherwise, differential diagnosis should be made with complications of sinusitis, orbital rhabdomyosarcoma, orbital lymphoma and other tumors. Imaging studies may help in the diagnosis by revealing soft tissue masses and areas of bone infiltration. In some cases GS may resemble abscesses. Diagnosis must be confirmed by histopathology.

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O termo sarcoma granulocítico (SG) designa um raro tumor sólido composto de agregados de precursores granulocíticos imaturos em sítios extramedulares. A lesão geralmente ocorre durante o curso natural da leucemia mieloide aguda (LMA) ou após sua remissão. O SG primário manifesta-se mais comumente na pele e linfonodos, portanto, quando se apresenta na mama, o erro diagnóstico de linfoma não Hodgkin, carcinoma lobular, sarcoma e melanoma maligno é um problema comum. A mama tem sido relatada como um local incomum de SG. Relata-se um caso raro de SG bilateral em mamas concomitante com LMA numa mulher de 47 anos. A paciente foi admitida em nosso hospital devido a manifestações neurológicas e descobrimos, durante a investigação, tumorações nas mamas. A histopatologia das lesões sugeriu linfoma não Hodgkin, sendo iniciada quimioterapia esquema CHOP. No entanto, o mielograma mostrou hiperplasia das séries granulocíticas, e a imuno-histoquímica revelou mieloperoxidase e CD68 positivos, confirmando o diagnóstico de SG primário em mamas. A citogenética não detectou anomalias. A revisão da microscopia e a análise do líquor confirmaram a presença de infiltração no parênquima mamário e no sistema nervoso central por leucemia monoblástica aguda (LMA-M5a). O protocolo de indução da remissão foi iniciado com daunorrubicina, arabinosídeo-C e quimioterapia intratecal com metotrexate, arabinosídeo-C e dexametasona (MADIT). Um mês depois, a paciente recusou a continuação do tratamento, depois de ter feito pedido de alta.

Granulocytic sarcoma (GS) is an uncommon solid tumor composed of aggregates of immature granulocytic precursors in extramedullary sites. The lesion generally occurs during the natural course of acute myelogenous leukemia or after remission has been achieved. Primary GS manifests most commonly in skin and lymph nodes, therefore when it presents in the breast, misdiagnosis of non-Hodgkin's lymphoma, lobular carcinoma, sarcoma and malignant melanoma is a common problem. The breast has been reported to be an uncommon site for GS. We report on a rare case of granulocytic sarcoma presenting as bilateral breast masses concomitant with acute myeloid leukemia in a 47-year-old woman. The patient was admitted to our hospital due to neurological manifestations, at which time we discovered lesions in the breasts. The histopathology suggested non-Hodgkin lymphoma, and chemotherapy using the CHOP regimen was performed. However, a myelogram showed hyperplasia of the granulocyte cells and immunohistochemistry tests were positive for myeloperoxidase and CD68, confirming the diagnosis of primary granulocytic sarcoma of the breasts. Cytogenetic examinations did not detect anomalies. The review of microscopy and the analysis of cerebrospinal fluid confirmed the presence of infiltration in the breast and in the central nervous system by acute monoblastic leukemia (AML M5a). AML M5a protocols had been started with daunorubicin, arabinoside-C and intrathecal chemotherapy using methotrexate, arabinoside-C and dexamethasone (MADIT). One month later, the patient refused further treatment.

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Sarcoma granulocítico (SG) é um tumor sólido extramedular, constituído por células precursoras de granulócitos. É geralmente associado a leucemia mieloide aguda ou raramente a outras desordens mieloproliferativas. O tumor geralmente ocorre precedendo uma leucemia mieloide aguda, durante o seu curso ou após a remissão ter sido alcançada. O prognóstico é pobre e tem como principais modalidades terapêuticas a quimioterapia e a radioterapia. Relata- se um caso de SG multicêntrico, de evolução rápida, com acometimento difuso de pele, mamas, gânglios linfáticos, tecido celular subcutâneo e líquor, em mulher de 45 anos, fora de tratamento para leucemia mieloide aguda e em remissão hematológica há 18 meses. A paciente apresentava dor intensa em membro inferior direito há uma semana e estava em anticoagulação oral há seis meses por trombose venosa profunda neste membro. Diagnosticado o SG, a paciente foi tratada com radioterapia e quimioterapia com boa resposta. Após três meses de seguimento, em vigência do tratamento quimioterápico, evoluiu com recidiva do SG neste membro, associado ao acometimento das mamas e posteriormente do sistema nervoso central, evoluindo para óbito em aplasia e sepses.

Granulocytic sarcoma is an extramedullary solid tumor consisting of immature granulocytic cells. It is often associated with acute myelogenous leukemia and more rarely with other myeloproliferative disorders. The tumor generally occurs before acute myeloid leukemia, during its course or after disease remission. It has a poor prognosis with the main therapeutic options being chemotherapy and radiotherapy. A multicentric accelerated case of granulocytic sarcoma of a 45- year- old woman with diffuse skin, breast, lymphatic ganglia and subcutaneous tissue presentations no longer undergoing treatment for acute myeloid leukemia and in hematologic remission for 18 months is reported. The patient presented with severe pain of right lower limb for a week and was undergoing oral anticoagulation for 6 months due to deep venous thrombosis. After diagnosis of granulocytic sarcoma she received radiotherapy and chemotherapy with good response. After a 3- month follow- up, under chemotherapy, she presented relapse of granulocytic sarcoma in the limb followed by breast and central nervous system presentations leading to death in aplasia and sepsis.

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Sarcoma granulocítico (cloroma) é um tumor de precursores mieloides em sítio extramedular. É complicação de leucemias mieloides agudas e crônicas. Apesar de poder surgir em qualquer lugar, envolvimento ovariano é raro. Relatamos um caso de tumor ovariano associado a leucemia mieloide aguda e seus achados de imagem na ressonância magnética.

Granulocytic sarcoma (chloroma) is a tumor consisting of myeloid precursors in an extramedullary site. It is complication of both acute and chronic myelogenous leukemias. Although the lesion can occur at any site, ovarian involvement is rare. We report a case ofovary tumor associated with acute myeloid leukaemia and its imaging appearance on magnetic resonance.

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Granulocytic sarcoma (GS) is a rare extramedullary tumor mass composed of immature cells derived from the hematopoietic myeloid series. It is usually associated with leukemia and other myeloproliferative disorders but can also occur without overt hematologic diseases. The breast has been reported to be an uncommon site of presentation. We report a case of acute myeloid leukemia preceded by GS of the breast. The immunohistochemistry revealed myeloperoxidase, CD68 and CD43 positivity, thus indicating a diagnosis of GS. Conventional cytogenetic analysis of peripheral blood cells showed t(8;21)(q22;22). Complete remission was achieved with Daunorubicin and Cytarabine induction therapy followed by three courses of high-dose Cytarabine consolidation. The patient remains in continuous complete remission at 27 months.

Sarcoma granulocítico (GS) é um raro tumor extramedular composto de células imaturas derivadas das linhagens hematopoiéticasmielóides. É geralmente associada à leucemia ou outras desordens mieloproliferativas, mas também pode ocorrer sem manifestação de doenças hematológicas. A mama tem sido relatada como local incomum de apresentação. Relatamos um caso de leucemia mielóide aguda precedida por GS da mama. A imunohistoquímica revelou mieloperoxidase, CD68 e CD43 positivos, indicando um diagnóstico da GS. A análise citogenética convencional de células de sangue periférico mostrou t(8; 21)(q22; 22). Remissão completa foi atingida com indução terapêutica com daunorubicina e citarabina, seguida de três ciclos de alta dose de citarabina. O paciente permanece em completa remissão há 27 meses.

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We report on a case of mediastinal granulocytic sarcoma with cardiac infiltration in a young man with no evidence of leukemia involving the bone marrow or peripheral blood. Diagnosis was accomplished by immuno-histochemistry with expressions of myeloperoxidase and CD99 antigens. The patient achieved clinical remission, but evolved with febrile neutropenia during chemotherapy and died. Although subclinical cardiac infiltrations are commonly found at autopsy in patients with acute non-lymphoblastic leukemia, only one case of involvement of the heart with granulocytic sarcoma in the absence of bone marrow disease has been published in the literature. A diagnosis of granulocytic sarcoma should not be excluded when the biopsy of the bone marrow does not show any evidence of leukemic infiltration.

Relata-se o caso de um adulto jovem com sarcoma granulocítico (SG) mediastinal com infiltração cardíaca sem evidência de leucemia envolvendo medula óssea ou sangue periférico. O diagnóstico foi revelado pela imuno-histoquímica com positividade para mieloperoxidase e CD99. O paciente apresentou remissão clínica, porém evoluiu com neutropenia febril durante a quimioterapia e foi a óbito. Embora infiltrados cardíacos subclínicos sejam comumente detectados na autópsia em pacientes com leucemia aguda nãolinfoblástica, somente um caso de SG com envolvimento cardíaco na ausência de doença na medula óssea foi descrito na literatura. Um diagnóstico de SG não deve ser excluída quando a biópsia da medula óssea não mostrar nenhuma evidência de infiltração leucêmica.

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A case of granulocytic sarcoma of skin and lymph nodes is reported in a 65-year-old man as an initial presentation of a myeloproliferative disorder, chiefly involving myelofibrosis. The symptoms, physical examination, hematological findings, imunohistochemistry and anatomopathological results and evolution of the disease are described. As this is an unusual case, stress was placed on the diagnostic confusion that may occur.

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