RESUMO
Ewing's sarcoma in the cervix is characterized by extremely rare occurrence,high degree of malignancy,and rapid progression.The diagnosis of this disease is based on pathology and immunohistochemistry. The main image of the case reported in this paper showed the cervical cyst with solid mass,large volume,and uneven density and signal,and the solid part can be strengthened in enhanced scanning.Because of the rapid growth,the lesion is prone to liquefaction necrosis and bleeding.Since the metastasis occurs early,timely diagnosis is essential.
Assuntos
Feminino , Humanos , Colo do Útero/patologia , Imuno-Histoquímica , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Sarcoma de Ewing/patologia , Neoplasias do Colo do ÚteroRESUMO
Askin tumour, a primitive neuroectodermal tumour of the thoracopulmonary region, is a rare tumour presenting in childhood. Its presentation in adults is rare. We report a case of an Askin tumour in an adult patient who presented to us with worsening breathlessness and vague chest pain. Investigations including immunohistochemistry confirmed the diagnosis of Askin tumour.
Assuntos
Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Biópsia , Neoplasias Ósseas/tratamento farmacológico , Neoplasias Ósseas/patologia , Neoplasias Ósseas/fisiopatologia , Progressão da Doença , Humanos , Pulmão/patologia , Neoplasias Pulmonares/patologia , Metástase Linfática , Masculino , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , /secundário , Pleura/patologia , Neoplasias Pleurais/patologia , Neoplasias Pleurais/secundário , Sarcoma de Ewing/tratamento farmacológico , Sarcoma de Ewing/patologia , Sarcoma de Ewing/fisiopatologia , Parede Torácica/patologia , Tomografia Computadorizada por Raios X/métodos , Resultado do TratamentoRESUMO
Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) of the prostate is extremely rare. Here, we report a case of ES/PNET of prostate in a 24-year-old man presenting with dysuria and pelvic discomfort. Computed tomography scan revealed a heterogeneous mass involving the prostate without evidence of distant metastases. Histologically, the tumor was composed of small round blue cells strongly and diffusely positive for CD99 and epithelial markers. Fluorescence in situ hybridization confirmed rearrangement of the Ewing's sarcoma region on chromosome 22.
Assuntos
Humanos , Queratinas/metabolismo , Masculino , Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico , Tumores Neuroectodérmicos Primitivos Periféricos/epidemiologia , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Próstata , Literatura de Revisão como Assunto , Sarcoma de Ewing/diagnóstico , Sarcoma de Ewing/epidemiologia , Sarcoma de Ewing/patologia , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
Tumores neuroectodérmicos primitivos (PNET) são neoplasias raras e extremamente agressivas encontradasprincipalmente em crianças e adultos jovens. São classificados em periféricos ou centrais, de acordo com sua origem. O diagnóstico baseia-se na história clínica, sendo essenciais exames de imagem, como tomografia computadorizada e ressonância magnética, e, para a confirmação, estudo anatomopatológico. É importante distingui-los de outros tumores de células redondas pequenas,como linfoma, sarcoma de Ewing extraósseo e rabdomiossarcoma,exigindo diferenciação imunoistoquímica através de marcadores específicos. O tratamento envolve cirurgia, quimioterapia e radioterapia, sendo o prognóstico pobre e a sobrevida bastante reservada. O objetivo deste artigo é discutir as características clínicas, radiográficas ehistológicas dos tumores neuroectodérmicos primitivos e seu tratamento...
Primitive neuroectodermal tumours (PNET) are rare and highly aggressive neoplasms found mainly in children and young adults. They are classified in peripheral or central according to their origin. The diagnosis is based on clinical history, computed tomography and magnetic resonance imaging, but the pathological study is the only way to confirm it. It´s very important to distinguish the PNET from other small cell round cell tumors - such as lymphoma, Ewing´s sarcoma and rhabdomyosarcoma through immunohistochemical specific markers. The treatment includes surgical resection, chemotherapy and irradiation. The prognosis is poor and the survival rate is variable. This article aims to discuss the clinical, imagingand histological features of the primitive neuroectodermal tumors and their treatment...
Assuntos
Humanos , Sarcoma de Ewing , Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Tumores Neuroectodérmicos Primitivos Periféricos , Seios ParanasaisRESUMO
Os autores relatam o primeiro caso de tumor neuroectodérmico primitivo periférico primário da órbita apresentado na literatura nacional. Características clínicas, radiológicas e histopatológicas são discutidas. O diagnóstico foi confirmado por meio de análise imuno-histoquímica, etapa essencial no diagnóstico dos tumores de pequenas células redondas.
The authors present a case of primary peripheral primitive neuroectodermal tumor of the orbit in a 10-month-old girl, which is the first case in the Brazilian literature. Clinical, radiologic and histopathologic features are discussed. The diagnosis was confirmed by immunohistochemical analysis which is essential to the diagnosis of small round cell tumors in the orbit.
Assuntos
Feminino , Humanos , Lactente , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Neoplasias Orbitárias/patologia , Tumores Neuroectodérmicos Primitivos Periféricos/terapia , Neoplasias Orbitárias/terapia , Biomarcadores Tumorais/análiseRESUMO
A 40-year-old male patient presented with enlarging mass over left leg region. Incisional biopsy report revealed an undifferentiated malignant round cell sarcoma, for which above-knee amputation was done. One year later, the same patient presented with left testicular swelling which appeared to be malignant. A left high orchiedectomy was done. Biopsy report showed metastatic round cell tumor, which was confirmed by immunohistochemistry as primitive neuroectodermal tumor. Metastasis to testis is very uncommon.
Assuntos
Adulto , Humanos , Perna (Membro) , Masculino , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Neoplasias de Tecidos Moles/patologia , Neoplasias Testiculares/patologiaRESUMO
El tumor neuroectodérmico primitivo, se incluye entre las neoplasias de células redondas y azules, forma parte de la familia de tumores de Ewing. El tumor neuroectodérmico primitivo es uno de los tumores que pertenecen a la familia de neoplasias formadas por células redondas, pequeñas, indiferenciadas que presentan una expresión fenotípica neuroectodérmica. Aparece con mayor frecuencia en la segunda década de la vida. Tiene varias formas de presentación, la más frecuente es en hueso, seguida de partes blandas, cavidad abdominal, área torácico-pulmonar, columna vertebral y sistema nervioso central. Excepcionalmente, se han descrito casos en miocardio, vulva y meninges. El caso que presentamos a continuación, es el segundo caso reportado en la literatura mundial diagnósticado en la glándula mamaria, así como, la revisión de la literatura en relación con esta patología
Assuntos
Humanos , Feminino , Adolescente , Sarcoma , Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Venezuela , OncologiaRESUMO
The term" small round cell tumour"describes a group of highly aggressive tumours composed of relatively small and monotonous undifferentiated cells with high nuclear to cytoplasmic ratio. This group includes: Ewing sarcoma [EWS], Peripheral neuroepithelioma [PN], Primitive neuroectodermal tumour [PNET], Neuroblastoma, Rhabdomyosarcoma [RMS], Desmoplastic small round cell tumour [DSRCT], Lymphoma, Leukemia, Small cell osteosarcoma Small cell carcinoma, Olfactory neuroblastoma, Merkel cell carcinoma, Small cell melanoma, and Mesenchymal chondrosarcoma. Their clinical presentation often overlap, thus making a definitive diagnosis problematic in some cases. Yet, a clear undrestanding of their clinicopathologic features usually allows for a confident diagnosis, especially if immunohistochemistry is used. This is an immunohistochemistry study of small round cell tumours with unknown origin that were diagnosed in pathology service of Imam Reza Hospital from 1362-1382. In this period, we found 24 cases which were reported SRCT without definitive diagnosis the immunohistochemistry kits are from DAKO Company and applied as directed by manufacturers. The SRCT is most frequent in the first decade of life [45%], 13 cases were male and 11 cases were female. IHC influence on diagnosis in 11 cases [46%] confirm the first ordered diagnosis and diagnosis made in another 9 [33%] of SRCTs,and in 2cases[8%]the first diagnosis was changed. Overally in review, 35 cases from 48 SRCTs with unknown origin [%75] were diagnosed by routine stainings by light microscopy without immunohistochemistry
Assuntos
Humanos , Masculino , Feminino , Carcinoma de Células Pequenas/diagnóstico , Células-Tronco , Sarcoma de Ewing/patologia , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Neuroblastoma/patologia , Rabdomiossarcoma/patologia , Linfoma/patologia , Leucemia/patologia , Sarcoma de Células Pequenas/patologia , Imuno-HistoquímicaRESUMO
Os autores relatam um caso de meduloepitelioma teratóide maligno intra-ocular acometendo uma menina de 7 anos com história de glaucoma congênito. O padrão histológico demonstrou ilhas de epitélio primitivo, com freqüentes estruturas tubulares e focos de cartilagem. Após procedimento cirúrgico, a paciente encontra-se bem, sem evidência de recorrência tumoral
Assuntos
Humanos , Feminino , Criança , Corpo Ciliar , Exoftalmia , Enucleação Ocular , Glaucoma , Esclera , Tumores Neuroectodérmicos Primitivos Periféricos/cirurgia , Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Neoplasias Uveais , Acuidade Visual , TomografiaRESUMO
It is well known that embryonal neuroectodermal tumors of the central nervous system (CNS) not infrequently display varying amount of neoplastic cells acquiring glial differentiation. In contrast, glial differentiation rarely occurs in primitive neuroectodermal tumors outside the CNS being documented in less than ten cases. The author presents herein a case of peripheral primitive neuroectodermal tumor with prominent glial differentiation identified by the presence of glial fibrillary acidic protein (GFAP) arising in the right suprarenal region of a 32-year-old man, histologically indistinguishable from an ordinary neuroblastoma.
Assuntos
Adulto , Proteína Glial Fibrilar Ácida/análise , Humanos , Imuno-Histoquímica , Masculino , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Neoplasias Torácicas/patologiaRESUMO
Presentamos un paciente de sexo masculino, de 29 años de edad, que consultó por tumoración dolorosa de pared toracoabdominal derecha. Los estudios por imágenes sugieren como diagnóstico diferencial: tumor de askin (neuroepitelioma), teniendo en cuenta sus características morfológicas y topográficas. Mediante el estudio anatomopatológico y con inmunohistoquímica se confirmó el diagnóstico de neuroepitelioma primitivo
Assuntos
Humanos , Masculino , Adulto , Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico , Tórax/patologia , Músculos Abdominais/patologia , Recidiva Local de Neoplasia , Recidiva Local de Neoplasia , Neoplasias Neuroepiteliomatosas , Neoplasias Neuroepiteliomatosas/secundário , Tumores Neuroectodérmicos Primitivos Periféricos , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Tomografia Computadorizada por Raios X , TóraxRESUMO
Peripheral neuroepithelioma is a rare tumor, comprising less than 1% of all soft tissue malignancies arising from the peripheral nonautonomic nervous system. Most peripheral neuroepitheliomas reported were located in the extremities, thoraco-pulmonary region, and pelvic areas, and as many as 30% of cases were associated with peripheral nerve. We report one case of peripheral neuroepithelioma arising in the kidney, mimicking renal cell carcinoma on the CT scan.
Assuntos
Adulto , Feminino , Humanos , Neoplasias Renais/patologia , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Doenças do Sistema Nervoso Periférico/patologiaRESUMO
Os autores apresentam um caso de meduloepitelioma justa-papilar, forma muito rara de tumor intra-ocular. Apresentam ainda, suas principais características clínicas, ecográficas e anatomopatológicas