Male with myelofibrosis and ulceronecrotic lesions.

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.

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.

Sarcoma mieloide em cavidade oral: relato de caso e revisão de 84 casos da literatura / Myeloid sarcoma in oral cavity: case report and review of 84 cases of literature

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)

Granulocytic sarcoma of the right humerus in a non-leukaemia patient.

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.

Cutaneous granulocytic sarcoma in myelodysplastic syndrome.

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.

[Central nervous system granulocytic sarcoma after bone marrow transplant: case report]. / Sarcoma granulocítico de sistema nervoso central pós transplante de medula óssea: relato de caso.

Granulocytic sarcoma is a solid tumor, composed by granulocytic precursor cells at various levels of differentiation, located at an extra-medullary site. It is associated with acute myeloid leukemia, and its presence reveals a bad prognostic factor. The treatment usually consists of radiotherapy and chemotherapy. A case of an intracranial granulocytic sarcoma occurring six months after a bone marrow transplant in a patient with acute myeloid leukemia is reported. The patient presented with headache and left hemiplegia caused by a large fronto-parietal lesion with significant mass effect. After a complete surgical resection there was a full recovery of the deficit. The patient completed radiotherapy and chemotherapy with no evidence of disease after three months of follow-up. Surgery is indicated in the presence of progressive neurological deficit. Surgical decompression may provide rapid improvement and therefore, affect quality of survival.