Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Neuroectodermal Tumors, Primitive, Peripheral”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 703 records · Page 39Linked to original sources
PubMed2000

Apoptosis induced by doxorubicin in neurotumor cells is divorced from drug effects on ceramide accumulation and may involve cell cycle-dependent caspase activation.

Doxorubicin (0.5 microgram/ml) induced caspase-dependent apoptosis in SH-SY5Y neuroblastoma and CHP-100 neuroepithelioma cells. The apoptotic response started to be evident approximately 15 h after drug administration and, as monitored over a 48-h period, was more pronounced in CHP-100 than in SH-SY5Y cells. In both systems, apoptosis was accompanied by elevation of intracellular ceramide levels. Ceramide accumulation was blocked by the ceramide synthase inhibitor fumonisin B(1) (25 microM); this compound, however, did not prevent drug-induced apoptosis. Untreated cells from both lines expressed negligible p53 levels; on the other hand, whereas p53 and p21(Cip1/Waf1) were rapidly up-regulated in doxorubicin-treated SH-SY5Y cells, such a response was not observed in CHP-100 cells. Doxorubicin induced a G(2)/M phase block in both cell lines, but whereas the G(1) phase was markedly depleted in CHP-100 cells, it was substantially retained in SH-SY5Y cells. In the latter system, double G(1) and G(2)/M block largely preceded cell death; however, as apoptosis underwent completion, it selectively targeted late S and G(2)/M cells. Moreover, apoptosis suppression by caspase inhibition did not result in a recovery of the G(1) cell population. These results support the notion that doxorubicin-induced apoptosis and ceramide elevation are divorced events in neuroectodermal tumors and that p53 function is at least dispensable for apoptosis completion. Indeed, as G(1) cells appear to be refractory to doxorubicin-induced apoptosis, p53 up-regulation and p21(Cip1/Waf1) expression may provide an unfavorable setting for the apoptotic action of the drug.

Amino Acid Chloromethyl Ketones↗
PubMed2001

Imaging of non-central nervous system primitive neuroectodermal tumours: diagnostic features and correlation with outcome.

AIM: To document the varied radiological features before, during, and after treatment of non-Central Nervous System Primitive Neuroectodermal Tumours (PNETs), which are rare tumours of childhood. MATERIALS AND METHODS: Thirty-three children with PNETs have been treated at our institution between 1990 and 1999. Full radiological and clinical follow-up was obtained in 29 (17 females, 12 males). Imaging was retrospectively reviewed, with particular attention to Computed Tomography (CT) and Magnetic Resonance Imaging (MRI). RESULTS: Age range at diagnosis was 0-16 years old (mean 4.4 years). There were five main sites of tumour: head and neck (n = 7), scapula/axilla (n = 2), chest (n = 11), abdomen (n = 3), and spinal/paraspinal (n = 6). Overall mortality was 62%. Tumours of the scapula or paraspinal region appear to show better survival than other sites. Of 23 patients who had Tc99m-methylene diphosphonate (MDP) bone scans at diagnosis, four patients showed widespread distant metastases, seven showed focal increased uptake in an adjacent bone only, and 12 had normal examinations. CT was performed in 25 patients and MRI in 20, both at diagnosis and follow-up. Average size of tumours at presentation was 4.5 cm in the paraspinal, head and neck and scapular regions and 7.5 cm in the chest and abdomen. Tumours were typically of soft tissue density on CT with the larger (>5 cm) masses tending to be more heterogeneous in character. The lesions were slightly higher signal than muscle on T1-weighted (T1W) MRI and all masses were heterogeneous on T2W sequences. Calcification was uncommon (n = 6) and generally sparse. Tumours tended to displace adjacent soft tissue structures such as vessels and bronchi rather than invade or encase them. Tumours rarely crossed the midline. Local or bony invasion was seen in 12 patients at diagnosis. Metastases were identified in the lung (n = 5), pleura (n = 2), brain (n = 4), bone (n = 4), lymph nodes (n = 2), liver (n = 2), subcutaneous tissues (n = 2), kidney (n = 1) and peritoneum (n = 1). CONCLUSIONS: Imaging characteristics of non-CNS PNETs are described. Tumours tend to displace rather than encase adjacent structures; local invasion occurred in 43%. Tumour calcification is uncommon. Poor prognostic features included the presence of distant metastases at diagnosis (all four patients with distant metastases at diagnosis died), but even patients without metastatic disease have a relatively poor prognosis.

Abdominal Neoplasms↗
PubMed2003

Ectopic neural and neuroendocrine neoplasms.

Although neural and neuroendocrine tissues are distributed virtually ubiquitously throughout the body, the occurrence of selected neoplasms related to those lineages is extremely uncommon in some topographic sites. This review considers the clinicopathologic characteristics of heterotopic pituitary adenomas; neuroendocrine carcinomas in non-organ-based locations; ectopic (extraneuraxial) meningiomas and gliomas; visceral neuroblastic neoplasms and primitive neuroectodermal tumors; and paragangliomas arising outside the sympathoadrenal neural network. Practical approaches to differential diagnosis are emphasized.

Biomarkers, Tumor↗
PubMed2003

Primitive neuroectodermal tumor of the thumb metacarpal bone: a case report and literature review.

A 27-year-old otherwise healthy patient was diagnosed with a primitive neuroectodermal tumor of the thumb metacarpal bone of the left hand. Based on a common chromosomal translocation this tumor shows a close relationship to Ewing's sarcoma. Its occurrence in the extremities is uncommon and involvement of the hand is extremely rare. The treatment consisted of neo- and adjuvant chemotherapy and marginal resection of the affected thumb metacarpal bone including periosseous soft tissue and reconstruction of the thumb by an intercalated segmental index pollicization.

Adult↗
PubMed2000

Ewing's sarcoma/primitive neuroectodermal tumor of the ureter: a case report and review of the literature.

Extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is a rare soft tissue tumor of childhood usually found in the extremities. The authors present the case of a 17-year-old girl who presented with right flank pain and hematuria and during operation was found to have a right ureteral mass. The histopathologic, immunohistochemical, ultrastructural, and cytogenetic characteristics of the excised mass were consistent with extraosseous ES/PNET. This is the first known reported case of extraosseous ES/ PNET of the ureter. The pathologic features and clinical management of this case, as well as a review of the literature, are presented.

Adolescent↗
PubMed1975

[Clinical and morphological considerations concerning "medulloepithelioma" (author's transl)].

The clinical course and autopsy findings in a 20 months old child with a so-called medulloepithelioma of the brain stem are reported. The histological features of this tumour are described. Ten previous cases in the literature are reviewed and the controversial problems of this entity are discussed. It seems reasonable to place this malignant tumour in the group of ependymomas; in detail, the definition "embryonic ependymoma"; proposed by Fowler, appears to be the most suitable, because it comprises all morphological and biological features of this rare malignant tumour.

Brain Neoplasms↗
PubMed1992

[Midfacial degloving: an alternative approach to the frontobasal area, the nasal cavity and the paranasal sinuses].

Midfacial degloving can be characterized as an alternative surgical approach for exposing the bony structures of the midface. In combination with transient partial osteotomies the nasal cavities, the paranasal sinuses, the pterygopalatine fossa and the posterior parts of the anterior skull base are easily accessible. Using an intercartilaginous, a transseptal and a circumvestibular incision in the nose and a vestibular incision in the oral cavity the soft tissues of the upper face are mobilized and transposed cranially up to the infraorbital rim, the nasion and the lacrimal sac. Thus one can avoid scar formations in the face. In comparison with the common visible incisions in the face a bilateral exposure of midline structures is possible. The resected bone can be easily replaced and fixed with titanium miniplates for osteosynthesis. The soft tissue glove is replaced. A correct suture technique for readaptation especially in the nasal cavity is most important to avoid a circular stenosis of the nasal aperture. Between 1986 and 1991, 40 patients with various tumors (juvenile angiofibroma, inverted papilloma, esthesioneuroblastoma, squamous cell carcinoma of the maxillary sinus, benign tumors of the pterygopalatine fossa, clivus chordoma) underwent this procedure. Neoplasms and fractures of the anterior frontal skull base, the frontal sinus, the orbital cavity and the zygoma were less accessible due to the unsatisfactory exposure of these regions. Complications and side effects were rare. In five cases, a transient paresthesia of the infraorbital nerve and a facial edema were observed. In one case, a circular stenosis of the nasal aperture required a second plastic procedure.(ABSTRACT TRUNCATED AT 250 WORDS)

Adenoma↗
PubMed1995

[Neuropathologic findings in chronic epilepsy].

The surgical treatment of chronic epilepsies is increasing rapidly. A review of 279 surgical specimens of patients with chronic pharmaco-resistant epilepsies from the University of Bonn Medical Centre revealed specific histopathological findings in 89% of all well-preserved specimens. The most frequent focal lesions were low-grade brain tumours such as gangliogliomas, pilocytic astrocytomas and dysembryoplastic neuroepithelial tumours. Other common findings were glioneuronal and vascular malformations and Ammon's horn sclerosis. This suggests that chronic intractable epilepsies are frequently caused by structural alterations.

Amygdala↗
PubMed1993

[Esthesioneuroblastoma: diagnostic conformation by immunohistology and electron microscopy].

The aesthesioneuroblastoma (olfactory neuroma) is a rare neuroepithelial tumour of the nasal cavity, the clinical symptoms of which become manifest very late in most patients. In general, with the light microscope used routinely (fixation of the specimens with formaldehyde, staining with haematoxylin-eosine) a malignant round cell neoplasia can be recognised without further differentiation. To ensure the diagnosis of an aesthesioneuroblastoma, immunohistological techniques (vimentin, S-100 protein, neurofilaments, neuron-specific enolase) are undoubtedly necessary. In some cases of unclear findings the electron microscope might be used to prove an aesthesioneuroblastoma. The immunohistological and electron microscopic features of aesthesioneuroblastoma are demonstrated and problems of histological differential diagnosis are discussed.

Adult↗
PubMed1992

[Esthesioneuroblastoma with orbital invasion].

The authors report on an 83-years-old woman with esthesioneuroblastoma (ENB). The first clinical manifestation was an invasion of the suborbital and orbital region. ENB are local destructive tumours which seldom metastasize. Complete surgical excision, supplemented by irradiation therapy offers the highest cure rate. Relapses should be treated with individualized afterloading radiotherapy.

Aged↗
PubMed1986

[Computed tomographic diagnosis and differential diagnosis of malignant paranasal sinus tumors].

The CT appearances of malignant tumours of the paranasal sinuses are illustrated on the basis of 15 patients, and the differential diagnosis discussed. Malignant soft tissue tumours in the paranasal sinuses are characterised on CT by their non-homogeneous structure; they may destroy the bony margins of the sinus and infiltrate neighbouring regions in certain preferred directions, and they may enhance following the administration of contrast. Precise definition of the malignant tumour by CT permits their exact staging, may help to determine therapy and is valuable for serial observation. It remains to be seen, however, whether the improved radiological diagnosis results in improved prognosis of malignant tumours of the paranasal sinuses.

Adenocarcinoma↗
PubMed1985

[Malignant teratoid medulloepithelioma of the ciliary body and glial fibrillary acidic protein. Clinical, histochemical and immunohistochemical findings].

In a four-year-old boy a malignant teratoid medulloepithelioma of the ciliary body was removed by means of an 11.0 X 11.0 mm "block excision". Two years later the patients visual acuity was 0.6. The lesion originates in the nonpigmented ciliary epithelium, extending along the lens equator to the pupillary zone and through the iris stroma into the anterior chamber. The tumor shows tubular structures with hyaluronic acid, "retinal rosettes", and areas with ganglion and GFAP-positive glial cells. The immunohistochemical demonstration of the glial fibrillary acidic protein is also of diagnostic value for the classification of tumors of the nonpigmented ciliary epithelium.

Child, Preschool↗
PubMed1990

Streamlined approach to creating yeast artificial chromosome libraries from specialized cell sources.

The study of tumor-specific chromosomal abnormalities has been severely impeded by an inability to link cytogenetic to molecular data. Restriction fragment length polymorphism mapping of any particular chromosomal rearrangement to the resolution limit of genetic methodology generates sets of probes that frequently are still too widely spaced to render the rearrangement breakpoints accessible to molecular isolation. The stable propagation of genomic fragments of up to one million base pairs in size as yeast artificial chromosomes (YACs) represents an important development in this regard. However, existing YAC libraries have been made from karyotypically normal sources making the localization and cloning of specific rearrangement breakpoints much more difficult. As a solution to this problem, we present an improved method for creating YAC libraries that can utilize specialized tumor-derived materials and that can be executed effectively in a small laboratory setting. Procedures that enabled more consistent DNA insert size selection and enhanced yeast transformation frequency were employed to generate a human YAC library from a neuroepithelioma cell line containing a characteristic t(11;22) chromosomal translocation. Approximately 40,000 colonies with an average insert size of 330 kilobase pairs were created. This library was screened with two single-copy probes that bracket the translocation breakpoint. YAC clones ranging from 370 to 550 kilobase pairs that were specific for each single-copy probe were identified. Specialized YAC libraries will make many more tumor-specific chromosomal abnormalities accessible to molecular isolation.

Cell Line↗
PubMed2001

Primary pulmonary primitive neuroectodermal tumor (PNET). A case report.

We describe a rare case of a primary primitive neuroectodermal tumor (PNET) in the lung of a 17-year-old girl. Grossly, the tumor, located in the right lower lobe, was relatively well-circumscribed and whitish to yellowish in color with scattered hemorrhagic necrosis. Microscopically, the tumor was composed of ovoid to polygonal cells with a high nuclear to cytoplasmic ratio and relatively scant cytoplasm, arranged in solid sheets with intervening fine fibrovascular stroma. Immunohistochemically, the tumor was positive for the MIC2 gene product, whereas AE1/AE3, CAM5.2, and a variety of neuroendocrine markers such as chromogranin A, synaptophysin, and ProGRP, were negative. Three months after the lobectomy, recurrent tumors were noted in the mediastinum and right thoracic wall, and she died despite combined chemotherapy and radiation therapy. In this case cytogenetic analysis showed a hypertriploid karyotype with multiple numerical and structural chromosomal aberrations, but failed to disclose distinct evidence of translocation between chromosome 11 and 22. However, the reverse transcriptase-polymerase chain reaction (RT-PCR) demonstrated EWS/FLI-1 fusion transcripts, confirming the histopathologic diagnosis of PNET. This case indicates that the primary pulmonary PNET is a highly aggressive neoplasm occurring at a young age, and should prompt combined systemic chemotherapy, even though it is organ-confined.

Adolescent↗