Cervical Primary Ewing's Sarcoma:Report of One Case / 中国医学科学院学报

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.

Askin tumour: A rare thoracopulmonary tumour in adults.

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.

Cytokeratin-positive primitive neuroectodermal tumor of the prostate: Case report and review of literature.

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.

Tumor neuroectodérmico de seios paranasais: diagnóstico e tratamento / Primitive neuroectodermal tumor of paranasal sinuses: diagnosis and treatment

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...

Tumor neuroectodérmico primitivo periférico primário da órbita: relato de caso / Primary peripheral primitive neuroectodermal tumour of the orbit: case report

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.

Metastatic primitive neuroectodermal tumor involving testis.

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.

Tumor neuroectodérmico primitivo (PNET) de la mama: reporte de un caso / Primitive neuroectodermic tumor (PNET) of the breast: proposal of a case

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

[Small round cell tumours with unknown origin in pathology specimens of iman reza hospital from 1362-1382]

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

Meduloepitelioma teratóide maligno do globo ocular: relato de caso e revisão da literatura / Intraocular malignant teratoid medulloepithelioma: case report and review of the literature

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

Peripheral primitive neuroectodermal tumor with neuronal and glial differentiation: report of a case arising in suprarenal region.

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.

Tumor neuroectodérmico primitivo: neuroepitelioma periférico / Primitive neuroectodermal tumor of the chest wall

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

Peripheral neuroepithelioma of the kidney

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.

Meduloepitelioma justa-papilar: apresentaçäo de um caso / Medulloepithelioma: report of a case

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