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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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At least 631 records · Page 35Linked to original sources
PubMed2004

Definitive irradiation in multidisciplinary management of localized Ewing sarcoma family of tumors in pediatric patients: outcome and prognostic factors.

PURPOSE: To assess the effect of radiation dose on local tumor control of the Ewing sarcoma family of tumors in 79 patients with localized disease treated at a single institution. METHODS AND MATERIALS: Thirty-seven patients received vincristine, actinomycin D, cyclophosphamide, and doxorubicin, and 42 received vincristine, actinomycin D, and cyclophosphamide, with alternating cycles of ifosfamide and etoposide; all underwent definitive radiotherapy (median dose, 37.5 Gy) with either low-dose (<40 Gy) or standard dose (> or =40 Gy) radiation delivered according to the protocol. We calculated the cumulative incidence of local treatment failure, disease recurrence, and overall survival and analyzed the effect of known prognostic factors and radiation dose. RESULTS: The cumulative incidence of local treatment failure at 10 years was 30.4% and that of disease recurrence was 40%. The overall survival rate was 64.5%. Patient age > or =14 years and tumor size > or =8 cm were adverse prognostic factors for local treatment failure; patient age > or =14 years was also associated with worse survival. Although the radiation dose alone did not predict for local treatment failure, the cumulative incidence of local failure at 10 years was 19% when tumors <8 cm were treated with <40 Gy, and no patient treated with standard doses (> or =40 Gy) developed local recurrence (p = 0.084). CONCLUSION: Tumor size and patient age predict for local tumor control in patients with Ewing sarcoma family of tumors treated with systemic therapy and definitive radiotherapy. Patients treated with reduced-dose radiotherapy experienced unacceptably high rates of local recurrence.

Adolescent↗
PubMed2006

Renal primitive neuroectodermal tumor in childhood with intracardiac extension.

Primary primitive neuroectodermal tumors of the kidney are exceptionally rare and usually affect children and young adults. We report the first pediatric case of renal primitive neuroectodermal tumor presenting with tumor extension along the inferior vena cava to the right ventricle. This case highlights that when considering a renal tumor with significant intravascular extension in the pediatric age group, although the most likely diagnosis remains Wilms tumor, other rare entities may also demonstrate similar clinical and imaging features.

Child, Preschool↗
PubMed2004

Primary primitive neuroectodermal tumor of the uterus: a report of two cases and review of the literature.

BACKGROUND: Primary primitive neuroectodermal (pPNET) tumors rarely occur in adults, and they very rarely present as primary tumors of the uterus. Only 12 reported cases of pPNET of the uterus have been published in the English literature. We report two additional cases treated at the Roswell Park Cancer Institute, Buffalo, NY, between 1999 and 2002. CASES: Two postmenopausal patients presenting with abnormal uterine bleeding underwent endometrial biopsy, and subsequently staging laparotomy. The diagnosis of pPNET in both cases was confirmed only by extensive immunohistochemical analysis of the tumors. One patient with disease confined to an endometrial polyp received no adjuvant therapy, while the second patient with extrauterine disease received adjuvant pelvic radiation followed by chemotherapy. CONCLUSIONS: The diagnosis of pPNET of the uterus may be a challenge. Features of diagnostic significance include positive staining with neuron-specific enolase, presence of neurosecretory granules, and positive staining with the MIC-2 gene. Currently, there is no uniformity in the treatment of these cases since the majority of the patients reported to date have had surgery, chemotherapy, and/or radiation therapy.

Aged↗
PubMed2004

Primary Ewing's sarcoma-primitive neuroectodermal tumor of the vagina.

BACKGROUND: Only three cases of Ewing's sarcoma-primitive neuroectodermal tumor (ES-PNET) occurring in the vagina have been previously reported. CASE: A 30-year-old Chinese woman presented with a vaginal mass measuring 5 cm in greatest dimension. The tumor was composed of solid sheets of undifferentiated small round cells with numerous Homer-Wright rosettes. Immunohistochemically, it was positive for CD99, FlI-1 protein, synaptophysin, neuron-specific enolase, vimentin, and S-100 protein. The patient underwent wide local excision of the tumor and subsequent total abdominal hysterectomy with postoperative chemotherapy and radiotherapy. She is disease-free 36 months after the initial surgery. CONCLUSION: This is a rare case of ES-PNET occurring in the vagina and the first showing many distinctive Homer-Wright rosettes. A review of literature indicates that vaginal or vulvar ES-PNET affects younger women, and it may have a relatively favorable outcome.

Adult↗
PubMed1978

A clinicopathologic study of 56 cases of intraocular medulloepitheliomas.

A clinicopathologic and follow-up study of 56 intraocular medulloepitheliomas in the Registry of Ophthalmic Pathology revealed a mean age of five years at the time of definitive diagnosis, most patients having had a delay in surgical therapy of more than one year after onset of signs or symptoms. Histologic evidence of malignancy was observed in 37 tumors, and follow-up studies revealed four patients to have died of metastatic disease. Heteroplastic elements (brain tissue, cartilage, or rhabdomyoblasts) were observed in four benign and 17 malignant tumors; these 21 were designated teratoid medulloepitheliomas. The most important prognostic feature was extraocular extension, observed in ten cases. All four of the known tumor deaths were preceded by clinically obvious orbital spread. In four additional cases with orbital involvement the patients were lost to follow-up. We concluded, therefore, that the prognosis is worse than the results of this study would indicate, especially among those cases observed to habe extraocular extension.

Adolescent↗
PubMed1978

Teratoid intraocular medulloepithelioma.

The right eye of a 1-year-old black infant with a painful blind eye was studied histologically. It contained a tumor at an advanced stage of growth. The tumor consisted of moderately large, round, and elongated undifferentiated cells and anaplastic cells with mitotic figures combined with multilayered, poorly differentiated epithelial cells that resembled the medullary epithelium of embryonic retina. Strap cells with cross striation typical of striated muscle were also present, together with islands of hyaline cartilage and areas of calcification.

Ciliary Body↗
PubMed2002

Advances in the surgical management of sarcomas in children.

The management of children with soft tissue sarcomas has changed and survival has improved markedly during the past three decades. This is, in large part, due to the multi-modal approach to pediatric malignancies; decreasing the need for radical surgical procedures, while improving survival and decreasing morbidity. The management of patients with rhabdomyosarcomas (RMS) and nonrhabdomyosarcomas soft-tissue sarcomas (NRSTS) as well as neurofibromatosis and related tumors is best accomplished in an organized and systematic fashion with the surgeon involved early in the planning, diagnostic, and treatment phases. Cooperative trials for rhabdomyosarcoma, as part of the Intergroup Rhabdomyosarcoma Study, have led to improved survival with less radical surgery. Ongoing trials of the Soft Tissue Sarcoma Group of the Children's Oncology Group are under way. Since these tumors are rare, cooperative trials are essential to answering the remaining questions regarding extent of surgery and adjuvant therapy.

Child↗
PubMed2003

Caspase inhibition shifts neuroepithelioma cell response to okadaic acid from apoptosis to an apoptotic-like form of death.

We have previously shown that the protein phosphatase inhibitor okadaic acid (OA) induces caspase-3 activation and apoptosis in CHP-100 human neuroepithelioma cells. Herein we provide a more general picture of the effects brought about by OA in this system, also investigating whether caspase activation is necessary for apoptosis induction. We report that incubation for 24 h with 10 nM OA induced a large fraction of the cell population to undergo premature chromosome condensation (PCC) or mitotic arrest, but not apoptosis. The former two effects were also observed after cell treatment with 20 nM OA; however, at this concentration, typical apoptotic cells were also detected, characterized by pycnotic and fragmented nuclei. Occurrence of the above-mentioned apoptotic figures turned extensive at 100 nM OA. The pan-caspase inhibitor benzyloxycarbonyl-Val-Ala-Asp-fluoromethylketone (Z-VAD.fmk, 100 microM) fully prevented apoptosis induced by 20 nM OA, increasing PCC incidence. Conversely, 100 nM OA induced an apoptotic-like phenotype, even in the presence of Z-VAD.fmk: in this case, however, nuclei, albeit pycnotic, displayed morphological characteristics distinct from those of typical apoptotic cells; moreover, as assessed by flow cytometry, they were largely unfragmented. The reported OA effects occurred in a setting in which neither p53 nor p21(Cip1/Waf1) was upregulated, thus ruling out a role for these proteins in apoptosis induction. On the other hand, apoptotic doses of OA induced a shift of the retinoblastoma gene product to the hypophosphorylated state and its downregulation by a caspase-dependent mechanism.

Amino Acid Chloromethyl Ketones↗
PubMed2001

Potentiation of okadaic acid-induced ceramide elevation but not apoptosis by inhibition of glucosylceramide synthase in human neuroepithelioma cells.

Caspase-dependent apoptosis induced by okadaic acid (OA) in CHP-100 neuroepithelioma cells has previously been shown to associate with a rapid and sustained elevation in intracellular ceramide concentration. We now report that treatment of CHP-100 cells with OA also evoked a rapid elevation in glucosylceramide levels that was maintained at steady state as cells underwent apoptosis; moreover, as observed for ceramide, OA-induced glucosylceramide accumulation was not blocked by fumonisin B1. Remarkably, when cell death was prevented by caspase inhibition, glucosylceramide accumulation was potentiated and ceramide elevation reduced, thus suggesting that, during apoptosis completion, accumulation of ceramide was partly driven by impairment of its glucosylation through a caspase-dependent mechanism. We studied whether ceramide glucosylation provided a mechanism for negative modulation of OA-induced apoptosis. We observed that the blocking of glucosylceramide synthesis markedly potentiated OA-induced ceramide elevation, but neither accelerated apoptosis onset nor potentiated the apoptotic response. These results indicate that modulation of ceramide glucosylation does not affect the apoptotic response to okadaic acid and suggest that caution must be exercised concerning the possibility that ceramide plays a key role in apoptosis induction.

Amino Acid Chloromethyl Ketones↗
PubMed1997

Immunohistochemical demonstration of Wilms tumour gene product WT1 in a canine "neuroepithelioma" providing evidence for its classification as an extrarenal nephroblastoma.

An intradural extramedullary tumour, surgically removed from the spinal canal of a young dog with paraplegia, had the histological appearance of a nephroblastoma. Subsequent necropsy revealed no evidence of a renal primary tumour or of any other tumour. Similar tumours of the spinal canal have been described previously under a variety of names, in particular neuroepithelioma. With an antibody to the human Wilms tumour (nephroblastoma) gene product WT1, labelling of glomeruloid bodies, similar to glomerular podocytes in human fetal kidney, was demonstrated in the tumour. This finding strengthened the suggestion that it was a nephroblastoma.

Animals↗
PubMed1997

Retinoic acid-induced regulation of neuropeptide Y receptor expression and function in the neuroepithelioma line SK-N-MC.

UNLABELLED: Neuropeptide Y (NPY) acts through specific receptors to inhibit adenyl cyclase and may have a role in neuroblastomas and neuroepitheliomas (NE) as a regulator of cell growth and differentiation. The authors have examined the status of NPY receptors in the NE cell line SK-N-MC and the effect of retinoic acid (RA), a known differentiating agent, on their expression and function. METHODS: Competitive NPY binding studies were performed on normal and RA-treated cells, followed by Scatchard analysis. NPY receptor function in the absence of or following RA treatment was determined by the ability of various concentrations of NPY to attenuate the forskolin-stimulated accumulation of intracellular cAMP. The mitogenic effect of NPY was evaluated by growing normal or RA-treated cell in the presence of various concentrations of NPY. RESULTS: Scatchard analysis showed a Kd of 2.3 nmol/L and a Bmax of 91,000 receptors per cell for untreated cells. RA treatment decreased receptor expression to 11,700 per cell without a significant change in receptor affinity (3.6 nmol/L). The effect of forskolin was inhibited by NPY in a dose-dependent fashion in both untreated and treated cells indicating functional receptors in both NPY stimulates the growth of normal SK-N-MC cells. NPY stimulated growth was significantly attenuated after RA treatment, possibly as a result of decreased NPY receptor expression. CONCLUSIONS: Treatment of SK-N-MC cells with RA, a known differentiating agent, leads to decreased expression of functional NPY receptors and a concomitant decrease in the mitogenic effect of NPY. This supports the role for NPY in the pathogenesis of NE.

Cell Division↗
PubMed2004

[Infratentorial localisation of a dysembryoplastic neuroepithelial tumor. A case report].

Neuroepithelial dysembryoplastic tumor (DNT) is usually considered as a supratentorial benign neoplasm. DNT of the posterior fossa is a very rare entity and only four previous cases were reported in the literature. We describe a case of a 26-Year-old woman presenting recurrent episodes of vertigo. Magnetic resonance imaging revealed four cystic lesions located in the cerebellum, hypointense on T1-weighted images and hyperintense on T2-weighted images, without gadolinium enhancement. After partial resection, histological examination showed small glial cells, oligodendrocytes-like, lying in an eosinophilic alveolar matrix with some floating neurons. Due to this specific glioneuronal element, the diagnosis of DNT was retained. We discuss the clinical and radiological particularities of this infratentorial location and compare our case with those previously described in the literature.

Adult↗