RESUMO
Myeloid sarcoma (MS) is an uncommon localized extramedullary tumor composed of immature myeloid precursor cells that can affect any organ. Promyelocytic sarcoma (PS), an extremely rare subtype of MS, is characterized by immature myeloid cells with features of acute promyelocytic leukemia (APL). We describe a case of pediatric PS that presented as a solitary sacral mass without any evidence of systemic or bone marrow involvement. The cytopathologic evaluation using touch imprint demonstrated numerous blasts with bilobed nuclei, cytoplasmic hyper-granularity, and aggregates of Auer rods, which are typical cytomorphologic features of APL. Herein, we report an extremely rare case of isolated PS in a child, emphasizing the importance of cytomorphologic evaluation, which is complemented by the findings from a comprehensive work-up.
Assuntos
Sarcoma Mieloide , Humanos , Sarcoma Mieloide/patologia , Sarcoma Mieloide/diagnóstico , Sacro/patologia , Masculino , Criança , Leucemia Promielocítica Aguda/patologia , Leucemia Promielocítica Aguda/diagnósticoRESUMO
Promyelocytic sarcoma is an uncommon solid tumor made up of myeloblasts. It is characterized, like acute promyelocytic leukemia (APL), by a chromosomal translocation t(15;17) involving the retinoic acid receptor alpha (RARalpha) and the promyelocytic gene (PML). The diagnosis and monitoring of promyelocytic sarcoma is a challenge due to the rarity and severity of the disease. We describe a case with several initial sites and without APL. The patient was monitored with regular 18F-FDG PET/CT from diagnosis to complete response. The evolution of PET/CT imageries was compared to the quantification of PML-RARα fusion gene by RQ-PCR. In promyelocytic sarcoma medical care, 18F-FDG PET/CT appears to be an attractive tool for finding targets for biopsy, for the primary staging, for assessing therapeutic response and for detecting early relapse.
Assuntos
Leucemia Promielocítica Aguda , Sarcoma , Fluordesoxiglucose F18 , Células Precursoras de Granulócitos , Humanos , Leucemia Promielocítica Aguda/diagnóstico por imagem , Leucemia Promielocítica Aguda/genética , Proteínas de Fusão Oncogênica/genética , Tomografia por Emissão de Pósitrons combinada à Tomografia ComputadorizadaRESUMO
Promyelocytic leukemia is a known medical emergency and requires rapid diagnosis and expedient therapy with differentiating agents. We present an unusual case in which the diagnosis is based on a fine needle aspirate of a humeral mass. Despite lack of systemic involvement, the sarcoma responded to traditional differentiation agents.
RESUMO
This study reports a case of promyelocytic sarcoma that developed as a solitary sternal mass without any clinical evidence of acute promyelocytic leukemia. The case presented the diagnostic difficulties common to all aleukemic granulocytic sarcomas, and diagnosis was made possible by local identification of the PML/RARα fusion gene by fluorescent in situ hybridization. The patient was treated by surgery followed by chemotherapy plus all-trans retinoic acid therapy and local radiotherapy.
RESUMO
This study reports a case of promyelocytic sarcoma that developed as a solitary sternal mass without any clinical evidence of acute promyelocytic leukemia. The case presented the diagnostic difficulties common to all aleukemic granulocytic sarcomas, and diagnosis was made possible by local identification of the PML/RARalpha fusion gene by fluorescent in situ hybridization. The patient was treated by surgery followed by chemotherapy plus all-trans retinoic acid therapy and local radiotherapy.