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1/112. Intraspinal primitive neuroectodermal tumour: report of two cases and review of the literature.

    Two patients with primary intraspinal primitive neuroectodermal tumour are presented. In a 32-year-old man, the tumour evolved intradurally from a sacral nerve root. Despite repeated surgery and radiochemotherapy, the patient suffered multiple intraspinal tumour relapses and intracranial seedings, and died 29 months after the first diagnosis. In a 17-year-old male adolescent, the tumour was located in the lumbar epidural space, extending into the paraspinal muscles. Following resection and radiochemotherapy, the patient is free from disease 23 months after the initial presentation. The clinical, radiological, histopathological and cytogenetic findings of both patients are presented and the relevant literature is reviewed. Particular attention is given to the histogenetic relationship between peripheral primitive neuroectodermal tumour and Ewing's sarcoma.
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ranking = 1
keywords = peripheral, nerve
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2/112. Malignant peripheral neuroectodermal tumor (MPNET) of the kidney.

    BACKGROUND: Malignant peripheral neuroectodermal tumors (MPNETs) are primitive neuroblastic tumors that arise, unlike neuroblastomas, outside the autonomic nervous system. A renal origin has been described in very few cases. CASE REPORT: We report the case of a young male patient with a large MPNET of the right kidney, studied with ultrasound and computed tomography before surgical resection. The main radiologic features, the microscopic appearance and the typical immunohistochemical findings, are described and discussed.
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ranking = 5.0007307419412
keywords = peripheral, nervous system
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3/112. Peripheral primitive neuroectodermal tumor of the parotid gland region: report of a case with fine-needle aspiration findings.

    A case of peripheral primitive neuroectodermal tumor of the parotid gland region in a 38-yr-old woman is reported. She had a 1-yr history of a large, firm, and slightly tender left parotid-region mass. CT scan showed an invasive tumor involving the parotid gland, mandible, infratemporal fossa, and parapharyngeal space. Fine-needle aspiration cytology of the mass showed a highly cellular, poorly cohesive smear pattern exhibiting small cuboidal cells, with fibrillary cytoplasm forming occasional rosette-like structures. Numerous intact single cells with fragile cytoplasm, finely granular chromatin, and inconspicuous nucleoli were present together with free-lying nuclei in the background. Histologic, immunohistochemical, and ultrastructural findings confirmed the diagnosis. Diagn. Cytopathol. 2000;22:161-166. Published 2000 Wiley-Liss, Inc.
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ranking = 0.99984721147715
keywords = peripheral
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4/112. Peripheral primitive neuroectodermal tumor of the small bowel mesentery: a case showing perforation at onset.

    A case of peripheral primitive neuroectodermal tumor of the small bowel mesentery with an uncommon clinical onset is reported. A 40-year-old man was admitted to hospital because of acute severe abdominal pain. Chest X-ray revealed a free air sign beneath the diaphragm. At emergency surgery a mass measuring 11.0 x 8.0 cm with perforation was located in the jejunal mesenteric region. Histologically the resected lesion consisted of sheets of undifferentiated small round cells forming abortive Homer Wright rosettes. Some spindle-shaped cells showed perivascular pseudorosettes. Immunohistochemical study revealed that the tumor cells expressed positivity against CD99 (MIC2), neuron-specific enolase, synaptophysin and vimentin. To the authors' knowledge this is the first documentation of peripheral primitive neuroectodermal tumor of the small bowel mesentery with perforation at onset.
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ranking = 1.9996944229543
keywords = peripheral
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5/112. Thoracoabdominal peripheral primitive neuroectodermal tumors in childhood: radiological features.

    Peripheral primitive neuroectodermal tumors (PNET) are extremely uncommon, malignant neoplasms affecting mostly children and young adults. We retrospectively reviewed the clinical data and radiological studies of four such cases. All cases were pathologically proven. Plain films, US, and CT scans were used. The youngest child had a huge pelvic tumor and two adolescents each had a chest wall (Askin) tumor. The fourth patient had a most unusual location of the PNET in the anterior mediastinum. The CT findings are emphasized. We emphasize that the markedly abnormal CT findings are not specific for PNET.
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ranking = 3.9993888459086
keywords = peripheral
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6/112. A para-testicular primitive neuroectodermal tumor in an adult: a case report and literature review.

    OBJECTIVE: The authors describe the salient clinical, radiologic and histopathologic features of an extremely rare para-testicular primitive neuroectodermal tumor in a 25 year-old man. INTERVENTION: Excisional biopsy of the tumor en bloc was performed. Adjuvant VAdriaC-based chemotherapy (vincristine, doxorubicin, and cyclophosphamide) was given post-operatively. MAIN OUTCOME MEASURES: Histopathologic examination and immunohistochemical studies were performed on formaldehyde-fixed, paraffin-embedded tumor tissue. RESULTS: Histologic examination showed an undifferentiated small cell tumor. The tumor cells stained positively with MIC-2, a marker specific for primitive neuroectodermal tumors. The patient is 12 months post surgery and has completed adjuvant chemotherapy with no evidence of recurrent disease. CONCLUSIONS: This highly unusual, peripheral primitive neuroectodermal tumor should be considered in the differential diagnosis of undifferentiated small cell neoplasms of the genitourinary system in adults, from the kidney to the testicle. We present a patient with a PNET treated based on a Ewing's family of tumors protocol.
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ranking = 0.99984721147715
keywords = peripheral
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7/112. Cystic liver metastases from extracranial primitive neuroectodermal tumour: a case report.

    We report a rare case of cystic metastases to the liver from a peripheral primitive neuroectodermal tumour in a child. Ultrasound and CT appearances are described with a discussion on the histological findings and the differential diagnosis.
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ranking = 0.99984721147715
keywords = peripheral
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8/112. Subcutaneous primitive neuroectodermal tumour in the abdominal wall of a child: long-term survival after local excision.

    Tumours of the peripheral primitive neuroectodermal tumour/Ewing's sarcoma (PNET/ES) family can rarely occur as primary lesions within the skin and subcutis. We present a case of subcutaneous PNET within the abdominal wall of a 33-month-old child. Histologically it was a small round cell tumour that marked strongly for CD99 and displayed ultrastructual evidence of neural differentiation. The tumour was completely excised, but due to an initial misdiagnosis no adjuvant therapy was given. Despite this, the patient is well without evidence of disease 10 years and 3 months after surgery. A review of similar cases reveals not all patients are as fortunate, suggesting combination therapy, including chemotherapy, is still the best treatment for these tumours, even when small and superficial. Our report therefore highlights the importance of recognising PNET/ES in the skin and subcutis so appropriate therapy can be administered.
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ranking = 0.99984721147715
keywords = peripheral
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9/112. Intracranial Ewing sarcoma/'peripheral' primitive neuroectodermal tumor of dural origin with molecular genetic confirmation.

    Ewing sarcoma/'peripheral' primitive neuroectodermal tumor (ES/pPNET) is the designation given to a family of small cell neoplasms that typically arise in bone or soft tissue and are unified by their common expression of the MIC2 antigen and specific translocations involving a gene on chromosome 22q12 [the most common being t(11;22)(q24;q12)]. ES/pPNET of intracranial origin is extraordinary. We report the case of a 6-year-old boy with a large left frontal region mass that adhered to dura and was extracerebral at surgery. Histologic study revealed a high-grade, undifferentiated-appearing neoplasm of small cell type that was negative on immunostudy for glial fibrillary acidic protein, synaptophysin, desmin, leukocyte common antigen, smooth muscle actin and epithelial membrane antigen, but positive for vimentin and neuron-specific enolase and diffusely labeled by antibody O13 (which recognizes the MIC2 gene product). rna-based polymerase chain reaction assay confirmed the diagnosis of ES/pPNET by demonstrating fusion transcripts indicative of t(11;22) translocation. Bone scan, computerized tomography of the chest and bone marrow examination revealed no systemic tumor. The limited observations published to date suggest that primary intracranial ES/pPNET is most likely to present in childhood as a circumscribed, contrast-enhancing and dural-based extracerebral mass. It must be distinguished from a variety of small cell neoplasms, particularly PNETs of central neuroepithelial origin.
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ranking = 4.9992360573858
keywords = peripheral
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10/112. Peripheral primitive neuroectodermal tumor (PPNET) of pelvic origin: report of a case arising from an unusual location.

    A peripheral primitive neuroectodermal tumor arising from the abdominopelvic cavity is reported in a 24-year-old young male without any previous remarkable pathology. He was admitted to our hospital with complaints of urinary symptoms (acute urinary retention) and mild intestinal occlusion that had been present for three months. physical examination and CT scan revealed a pelvic mass occupying the entire pelvic cavity. The diagnostic workup included a CT-guided biopsy which defined the tumor as a sarcomatous type. Radical surgery was performed including tumor resection, pelvic exenteration (bladder and prostate gland) and urinary and fecal diversion. Adjuvant chemotherapy (VAIA) was delivered once the histology was confirmed. We reviewed the available literature focusing on the varied nomenclature of this tumor (peripheral neuroepithelioma, Askin's tumor, Ewing's extraosseous tumor, peripheral adult neuroblastoma, peripheral primitive extracranial neuroectodermal tumor (PPNET), the clinical features, the role of diagnostic imaging techniques, pathologic assessment and controversial therapeutic management. In addition, the prognosis and survival of this rare condition were analyzed.
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ranking = 3.9993888459086
keywords = peripheral
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