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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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At least 667 records · Page 37Linked to original sources
PubMed1998

Binding of enterostatin to the human neuroepithelioma cell line SK-N-MC.

SK-N-MC cells were found to possess binding sites for enterostatin, a peptide with central effects on appetite and sympathetic activation of brown adipose tissue during high-fat feeding. Scatchard analyses of the binding indicated one high-affinity binding (Kd = 0.5-1.5 nM) and one low-affinity binding (Kd = 15-30 nM) for 3H-enterostatin (APGPR). 125I-YGGAPGPR showed similar binding parameters as for the low affinity binding of 3H-APGPR. Met-enkephalin and beta3-casomorphin1-5 were found to displace the binding of 3H-APGPR to the SK-N-MC cells. Affinity purification of solubilized cells revealed an APGPR-binding protein estimated to 53 kDa which may represent a distinct enterostatin receptor. Cross-linking of 125I-YGGAPGPR to intact cells labeled one major protein with the same molecular size. There was no binding of enterostatin to four other human neuroblastoma/neuroepithelioma cell lines, named IMR-92, LAN#5, NB-1 #14 and SH5-SY.

Chromatography, Affinity↗
PubMed2005

[Primitive neuroectodermal tumor. Ewing's sarcoma].

Primitive neuroectodermal tumor is an extraordinarily rare primary tumor in the kidney and can be mistaken for a variety of other round cell tumors. It is important to recognize each of these entities, because each carries unique therapeutic and prognostic implications. However, accurate diagnosis of these tumors is hindered by their significant morphologic overlap and complicated by their rarity. These neplasm are highly aggressive that tend to recurence and to metastatize. Standard therapy combining surgery, chemotherapy, radiation and genetic therapy. We report a case of primitive neuroectodermal tumor of the kidney in a 50 year old female patient.

Female↗
PubMed1986

Olfactory neuroblastoma with spinal metastasis--a problem in diagnosis.

The olfactory neuroblastoma or esthesioneuroblastoma is a rare neuroectodermal tumor originating from the olfactory neuroepithelium, which can metastasis via cerebrospinal fluid pathways. In the present case of an extensive nasal malignancy with cervical lymph node metastases in a 75-year old woman, its difficult histology alternatively led to a diagnosis of anaplastic carcinoma and non-Hodgkin lymphoma. The patient died from complications following spread of the tumor to the spinal cord and cauda equina. Review of the literature shows that this tumor is notorious for its chameleonic character. In view of its clearly demonstrated malignancy an aggressive therapeutic approach is advocated.

Aged↗
PubMed2002

Ordering ceramide-induced cell detachment and apoptosis in human neuroepithelioma.

We studied whether cell detachment from the matrix, observed during ceramide-induced apoptosis, is secondary to completion of the apoptotic program. CHP-100 neuroepithelioma cells exposed to N-hexanoylsphingosine (C(6)-Cer) underwent detachment from the substrate and apoptosis with slow kinetics. Apoptotic cells were fairly completely recovered in the detached fraction, that, differently from the adherent counterpart, displayed the hallmarks of caspase 3 activation, as well as poly-(ADP)ribose polymerase (PARP) cleavage and focal adhesion kinase (FAK) downregulation. A key role for caspase 3 in apoptosis execution was suggested by the evidence that its selective inhibitor N-acetyl-Asp-Glu-Val-Asp-aldehyde inhibited cell death. However, the pan-caspase inhibitor benzyloxycarbonyl-Val-Ala-Asp-fluoromethylketone (targeting not only caspase 3 but also caspases 1, 5, 7, 8 and 9) did not prevent ceramide-induced cell detachment, although apoptosis, caspase 3 processing, PARP cleavage and FAK downregulation were suppressed in floating cells. These results demonstrate that ceramide-induced cell detachment is upstream activation of effector caspases. We discuss the possibility that ceramide-induced cell detachment might be instrumental to apoptosis execution.

Amino Acid Chloromethyl Ketones↗
PubMed1984

Diagnosis of soft tissue sarcomas.

Based on a review of 752 cases coded as soft tissue sarcomas, histologic features were analyzed for a differential diagnosis, dividing the sarcomas in the first diagnostic step into purely descriptive groups, regardless of biologic behavior: spindle cell, small cell, pleomorphic and myxoid varieties. The tumor categories mainly discussed in order were fibrosarcoma, leiomyosarcoma, malignant schwannoma, synovial sarcoma, malignant fibrous histiocytoma, embryonal and alveolar rhabdomyosarcoma, malignant neuroepithelioma , extraskeletal Ewing's sarcoma, and myxoid and other types of liposarcoma. The discussion was focused on the problems we were facing often in the histologic diagnosis of these tumors, emphasizing in particular those microscopic features which were helpful in differential diagnosis. Principal clinical features, important for an accurate diagnosis, were added to the histopathologic considerations in each item, together with prognosis of all these Japanese patients, regardless of the stages. Other benign and malignant lesions confused occasionally with the soft tissue sarcomas were also listed. With the aid of electron microscopy and immunohistochemistry, sometimes each differentiating feature can be identified but these methods are not always contributory to a diagnosis. Routine light microscopy with or without standard histochemical techniques remains essential to avoid pitfalls when attempting to diagnose the tumor.

Chondrosarcoma↗
PubMed1985

Immature teratomas of childhood. Report of 21 cases.

The benign or malignant nature and the biological behaviour of immature teratomas of childhood are difficult to predict. The age of the patient at diagnosis, the anatomical site of the tumour and the degree of immaturity are considered to be important prognostic parameters. In this study the pathological-anatomical findings and the most important clinical features of 21 patients with immature teratoma (including two with supposedly malignant monodermal teratoma with immature neuroectodermal structures) were evaluated. Significant results were: Twelve tumours occurred in infancy or infants who died in the perinatal period, the other nine tumours in children between the ages of 7 and 16 years. The most frequent anatomical sites were the ovary (6 tumours), sacrococcygeal region (4), testis (4) and mediastinum (3). In contrast to most of the tumours of other localizations, immature ovarian teratomas did not occur in children under 7 years of age (in four cases in association with gliomatosis peritonei). The immature tissue components of the tumours were mostly neuroectodermal structures. Eight tumour specimens showed grade 1, four grade 2 and nine grade 3 malignancy. Grade 3 tridermal teratomas chiefly occurred in young children, whereas two grade 3 monodermal tumours developed in older children. Immunohistochemical analysis of the neuroectodermal components showed that mature astrocytes contained glial fibrillary acid protein, whereas mature nerve cells, nerve fibres and a few groups of immature cells reacted with an antibody to neuron-specific enolase. Six of the 21 patients died; two were stillborn immature infants, two were premature infants, one died postoperatively and one died of metastatic disease. One patient with metastatic disease was alive. None of the 19 children with tridermal immature teratoma showed distant metastases. Metastatic disease was observed in only two patients with presumptive monodermal malignant teratoma. In early childhood the biological behaviour of immature teratomas is evidently similar to that of mature teratomas (provided that the tumour can be totally excised). In older children malignancy must be assumed when the tumour is located in the ovary and/or grade 3 immaturity is determined.

Adolescent↗
PubMed1998

Radiotherapy in Ewing's sarcoma and PNET of the chest wall: results of the trials CESS 81, CESS 86 and EICESS 92.

PURPOSE: Treatment results and the pattern of relapse were evaluated in the multimodal treatment of Ewing's sarcomas of the chest wall. METHODS AND MATERIALS: In a retrospective analysis, 114 patients with non-metastatic Ewing's sarcoma of the chest wall were evaluated. They were treated in the CESS 81, CESS 86, or EICESS 92 studies between January 1981 and December 1993. The treatment consisted of polychemotherapy (VACA, VAIA, or EVAIA) and local therapy, either surgery alone (14 patients), radiotherapy alone (28 patients) or a combination of both (71 patients). The median follow-up was 46.6 months (range 5-170). A relapse analysis for all patients with local or combined relapses was performed. RESULTS: Overall survival was 60% after 5 years, event-free survival was 50%. Thirty-seven patients had a systemic relapse (32.4%), 11 patients had a local relapse alone (9.6%), and 3 patients had a combined local and systemic relapse (2.6%). The risk to relapse locally after 5 years was 0% after surgery alone, 19% after radiation alone, and 19% after postoperative irradiation. None of the 8 patients with preoperative irradiation have failed locally so far. With the introduction of central radiotherapy planning in CESS 86, local control of irradiated patients improved. Ten of 14 patients with local failure could be evaluated in the relapse analysis: 3 patients had an in-field relapse, 4 patients had a marginal relapse, 2 patients had a relapse outside the radiation fields, and 1 patient failed with pleural dissemination. Six treatment deviations were observed. CONCLUSION: Local control was best after surgery alone in a positively selected group of patients. Local control after radiation or combined radiation and surgery was good. With diligent performance of radiotherapy, it will be possible to further improve the results in the radiotherapy group.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1989

Clinical and histopathological studies of olfactory neuroblastoma.

Six patients of olfactory neuroblastoma were treated, and two of them were studied with electron microscopy and immunohistochemical staining. These patients, four male and two female, ranged in age from 37 to 73 years old. Five patients had nasal bleeding, and only one patient had frontal pain and optic disorder without nasal bleeding. None of the patients had metastasis. However, three patients had local recurrence and invasion into the intracranial region. The tumors of three patients were confined to the nasal cavity and one or more paranasal sinuses. The tumors of the other three patients extended beyond the nasal cavity and paranasal sinuses. Two patients, treated with radiotherapy and cryosurgery, died of the disease 7 and 21 years after treatment, respectively. Two patients, treated with radiotherapy alone or a combination of radiotherapy and chemotherapy, died of other causes without recurrence 2 and 3 years later, respectively. A patient treated with craniofacial resection alone is still alive after 4 years. However, he has local recurrence and has been treated with radiotherapy and adjuvant chemotherapy. The last patient, treated with craniofacial resection following radiotherapy, is still alive after 3 years without any symptoms of recurrence. The two most recent cases were studied with electron microscopy, stains for PAS, Grimelius and Bodian, and immunohistochemical staining for S-100 protein, neuron-specific enolase, and glial fibrillary acidic protein. The tumor cells of one case were well differentiated because they contained neurosecretory granules and neurites, and the tumor cells of the other case were poorly differentiated because they contained only neurites-like structures.

Adult↗
PubMed2003

Emergency department presentations of a rare tumor--extraosseous cervical paraspinal Ewing's sarcoma.

We report the case of a 9-year-old boy who presented to our community-based Pediatric Emergency Department with complaints of neck pain, stiffness and upper extremity weakness. The symptoms were later identified to be due to cervical extraosseous paraspinal Ewing's sarcoma. The patient had a fatally aggressive clinical course. Cervical extraosseous Ewing's sarcoma is rarely reported in pediatrics. Ewing's sarcoma should be considered in the differential diagnosis of children presenting with complaints of unremitting or recurring non-specific back or neck pain or neurological deficits. Appropriate diagnostic evaluation and treatment should be actively pursued.

Antineoplastic Combined Chemotherapy Protocols↗
PubMedDate not supplied

Different vascular patterns of medulloblastoma and supratentorial primitive neuroectodermal tumors.

Astrocytoma vasculature patterns differ according to histological grade of malignancy with glioblastoma multiforme (WHO grade IV) showing most extensive endothelial proliferation. Here, we determined whether the vascular patterns of medulloblastoma and supratentorial primitive neuroectodermal tumors (PNETs), which can be hardly distinguished histopathologically, differ. We evaluated the spatial organization of vessels in medulloblastomas and PNETs using antibodies to von Willebrand factor (vWF) and CD34. Medulloblastoma capillaries showed slight endothelial cell hyperplasia. Microvessels sprouted from the capillaries and formed glomeruloid clusters. There were areas with chains of unopposed endothelial cells (3-10 cells). Supratentorial PNETs had highly branched capillaries with extensive endothelial cell hyperplasia. Glomeruloid arrays of microvessels extended from the capillaries. Small fragments of endothelial tubes were scattered throughout the tumor. Therefore, medulloblastomas and supratentorial PNETs showed different spatial organization of tumor vessels which can be used for differentiation of each tumor entity. These vascular patterns may reflect different tumor derived angiogenic stimuli.

Antigens, CD34↗
PubMed1992

Esthesioneuroblastoma. A case report.

Esthesioneuroblastoma (ENB) is a rare neuroectodermal tumor originating from the olfactory mucosa and therefore usually arising from the nasopharynx. A case in which the diagnosis was made in connection with the extraction of a tooth is reported. Manifestation in the dento-alveolar region is unusual for this tumor. The case presented is also unusual in that the classic symptoms of ENB were not present. Diagnosis, classification, and therapy are described with a brief review of the literature.

Female↗
PubMed2002

[Primitive neuroectodermal tumor of the cauda equina. Report of a case].

Primitive neuroectodermal tumors are malignant neoplasms whose classification has been controversial. Spinal primitive neuroectodermal tumors are rare and at the cauda equina are even less common. We report the case of a 40 year-old man with a history of progressive back pain and gait difficulty. After the diagnosis of a primitive neuroectodermal tumor of the cauda equina, the patient presented seeding to the intracranial subarachnoid space followed by a poor outcome. We review the literature of primary spinal primitive neuroectodermal tumors.

Adult↗
PubMed1976

Olfactory esthesioneuroblastoma.

Esthesioneuroblastomas are malignant tumours, usually of slow, invasive growth and low metastatic rate. Skeletal destruction must be assumed to be common, but is often demonstrable only by tomographic sections. Clinically these tumours do not differ from others of the same site, so that the diagnosis has to be based upon the histological appearances. In the light microscope the presence of neurofibrils is considered a specific differential diagnostic factor against other small-cell malignant tumours in this region. There seems to be no basis for a morphological classification into previously described sub-groups, neither according to histogenetic, light, nor ultra-microscopic findings. The general degree of differentiation and the number of mitoses appear to be the main factors of prognostic significance. Combined irradiation and surgical excision is considered the best treatment.

Adult↗
PubMed1979

Olfactory neuroblastoma: long-term survival.

Two cases of olfactory neuroblastoma have been described. A review of the aetiology, histopathology and radiological features has been undertaken. A regime of current treatment policy has been described.

Adult↗
PubMed1980

Esthesioneuroblastoma: report of two cases in Nigerians.

Two cases of esthesioneuroblastoma occurring in African Nigerians are reported, with a review of the literature. Though the tumour has highly suggestive clinical features, histologic diagnosis may be difficult. The name 'esthesioneuroblastoma' is considered to be preferable to 'olfactory neuroblastoma' in order to avoid consideration of such a tumour as a secondary from the adrenal gland. Early diagnosis and treatment by surgery gives the best chance of cure.

Adult↗