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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.

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CASE REPORT: We report the first case of orbital myeloid sarcoma that was successfully treated with a standard venetoclax dose of 25%. A 38-year-old man with acute myeloid leukemia (AML) post-haplo-hematopoietic stem cell transplantation (HSCT) presented with a nine-month history of progressive right proptosis and a visual acuity deficit. The patient was treated with venetoclax (100 mg orally on days 1-28), cytarabine (40 mg subcutaneously, days 1-10), and itraconazole (100 mg twice daily orally on days 1-28). MANAGEMENT AND OUTCOME: The present case report shows that using cytochrome P450 (CYP) inhibitors is a helpful strategy to reduce the cost of expensive treatments. DISCUSSION: There are limited data on the use of CYP inhibitors as a strategy to reduce the costs of expensive drugs (i.e. venetoclax). This approach has some advantages over standard dose venetoclax (400 mg/day) such as significantly reduced costs (which is relevant for patients in low-income countries). In this case, we used itraconazole-a potent CYP3A4 inhibitor-which can theoretically reduce the dose to 100 mg/day without losing serum therapeutic concentrations.

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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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BACKGROUND: The purpose of this study was to integrate the available data published on leukaemic infiltration in the oral and maxillofacial region into a comprehensive analysis of its clinical manifestations, imaginological characteristics, management and survival. MATERIALS AND METHODS: An electronic search with no publication date restriction was undertaken in October 2020 in the following databases: PubMed, Web of Science, Scopus and Embase. Overall survival was calculated by survival analysis with the Kaplan-Meier test. A critical appraisal of included articles was performed using the Joanna Briggs Institute tool. RESULTS: A total of 63 studies including 68 patients were selected for data extraction. The most common haematologic diagnosis was acute myeloid leukaemia (47%). The most affected individuals were 40 to 49 years old (20.9%). The male-to-female ratio was 1.2:1. The gingiva was the most affected site (37%). Swelling/mass/oedema (33.7%) and enlargement/hyperplasia/hypertrophy (25.5%) were the main clinical findings. Osteolytic lesions with bone destruction were the main imaginological characteristics among the reported cases. Follow-up was available for 36 patients. Overall, within the 21-month follow-up, the survival probability dropped to 14.3%. CONCLUSION: A considerable number of studies reported oral manifestations mainly in individuals with the acute form of leukaemia. Children and adults were affected, but the fifth decade of life was the most common. Dentists should be vigilant since these manifestations may be important for a diagnosis and for the monitoring of the treatment response and recurrence of haematological neoplasia.

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INTRODUCTION: We describe a 35-year-old male patient showing a myeloid sarcoma (MS) of the tongue as the first manifestation of acute promyelocytic leukemia (APL). The MS can appear in all parts of the human body, but it is extremely rare in the tongue. CLINICAL CASE: The main symptoms were a pain in the tongue, asthenia, gingivorrhagia, fever. We found a tumor in the tongue, which was irregular in size and located in the posterior region of the right lateral edge of the tongue. The diagnosis of MS was made by the anatomopathological and immunohistochemical study, while the definite diagnosis of APL was confirmed by the molecular test. The treatment of APL was based on the administration of trans-retinoic acid 45 mg/m2 daily continuously and daunorubicin 60 mg/m2 every other day for 4 doses, with a favorable therapeutic response to APL and MS. CONCLUSION: Promyelocytic myeloid cells can infiltrate many organs extramedullary, such as the tongue, and this might precede bone marrow infiltration. The early identification of myeloid sarcoma allows to carry out an early treatment of the APL.

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PURPOSE: Myeloid sarcoma (MS) is a rare malignant tumor that arises outside the bone marrow and usually occurs concomitantly or after the acute myeloid leukemia (AML). The aim of the present study is to report an extremely rare case of a patient with an MS arising in the maxillofacial region before the onset of AML to serve as a guide on how to conduct the initial assessment and diagnosis of similar cases. MATERIALS AND METHODS: A 29-year-old healthy man complained of a "swelling on the head," which was rapidly increasing. He was asymptomatic and never had a previous AML. RESULTS: The patient presented a large mass on the left temporal region. The computed tomography showed a well-delimited lesion extending from the lateral pterygoid muscle and involving the zygomatic arch and lateral orbital rim. The mass was diagnosed as an MS arising before the onset of the AML. CONCLUSION: Masses of a rapid increase in the maxillofacial region should always raise suspicion of being an MS even in asymptomatic patients. Following a protocol of initial assessment and diagnosis will decrease the period between the initial appearance of the MS and effective treatment aimed to prevent or delay the manifestation of leukemia, improving the prognosis and survival of these patients.

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RESUMEN Introducción y objetivos: El sarcoma mieloide puede ser la primera manifestación de la leucemia mieloide aguda (LMA), presentarse simultáneamente o constituir una forma de recaída. Material y métodos: Presentamos el caso de una paciente con sarcoma mieloide uterino, como forma de recaída de LMA. Resultados: El diagnóstico se basa en los hallazgos histopatológicos, la inmunohistoquímica y el inmunofenotipo. El tratamiento y el pronóstico son similares a LMA. Conclusión: La afectación uterina por leucemia mieloide extramedular es rara pero debe tenerse en cuenta en el diagnostico diferencial de una masa uterina en aquellas pacientes con antecedentes de LMA.

ABSTRACT Introduction and objectives: Myeloid Sarcoma can present as a first MLA sign, concurrently with or at relapse form. Materials and methods: We present the case of a patient with uterine myeloid sarcoma, as a form of relapse of MLA. Results: The diagnostic is based on the histopathology findings along with immunohistochemistry and immunophenotyping. Conclusion: Uterine involvement due to extramedullary myeloid leukemia is rare but it should be taken into account in the differential diagnosis of a uterine mass in those patients with a history of MLA.

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Granulocytic sarcoma (GS) is an extramedullary tumor associated with myelodysplastic syndromes or myeloproliferative diseases. Intraoral manifestations are considered uncommon, with a reasonable number of cases, and are mostly related to leukemia. The association of oral GS and myelofibrosis is very rare and only three cases have been published. In this paper, we report the fourth case of oral lesion in a patient with a diagnosis of myelofibrosis. The aim of this study was to present a review of the literature, discussing the current and previous cases of oral GS associated with myelofibrosis or other hematological disorders and the importance of accurate diagnosis through clinical, microscopic, and immunohistochemical features.

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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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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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