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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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PubMed1989

Pigmented esthesioneuroblastoma showing dual differentiation following transplantation in nude mice. An immunohistochemical, electron microscopical, and cytogenetic analysis.

Esthesioneuroblastoma (ESTH) is a neuroepithelial-cell-derived neoplasm of the olfactory mucosa composed of homogeneous small round cells which contain neurosecretory granules. Melanin has been detected in such tumours only occasionally. Here we describe a new case of ESTH with divergent differentiation. The primary neoplasm was found in a 67 year-old female, involving the left nasal and maxillary sinus; she died of cerebral metastasis ten months after diagnosis. Histologically only small round cells were seen, with S-100 and NSE positivity. Electron microscopy revealed neurosecretory granules and filaments, as well as the occasional presence of melanosomes. A nude mice xenograft line has been established, and is presently in its ninth transfer. Two cell types are present: small round-to-spindle shaped cells with neural features, and large epithelial-like ones. Both immunohistochemistry and electron microscopy confirm this dual differentiation, with the presence of membrane-bound dense-core neural secretion, as well as melanosomes of neuroectodermal origin. Additionally, an in vitro cell line has been established. Cytogenetic analysis confirmed the presence of both malignant human melanoma patterns; non-random abnormalities in chromosomes 1 and 6, extra copies of chromosome 7. Duplication of the long arm of chromosome 14, as seen in olfactory neuroblastoma, is also seen.

Aged↗
PubMed1996

A phase II study of thioTEPA in children with recurrent solid tumor malignancies: a Children's Cancer Group study.

A Phase II study of thioTEPA was performed by the Children's Cancer Group. ThioTEPA was administered intravenously every three weeks, at a dose of 65 mg/m2. Pediatric patients with recurrent sarcomas were targeted, but patients with other tumor diagnoses were also eligible. Toxicity was primarily hematopoietic, with thrombocytopenia being predominant. ThioTEPA did not demonstrate significant activity in the target tumor groups evaluated.

Adolescent↗
PubMed1995

Response of brain tumors to chemotherapy, evaluated in a clinically relevant xenograft model.

Chemotherapy for brain tumors remains unsatisfactory. Despite increasing participation in clinical trials, there is a clear need for pre-clinical models. Heterotransplantation of surgical specimens directly into the anterior chamber of the nude mouse eye has been demonstrated to produce evaluable xenografts. Drug access in this model is considered to mimic the blood-brain barrier. Five clinical specimens in 3 children with primitive neuroectodermal tumor/medulloblastoma were the sources of 293 intraocular xenografts (5 cohorts by source). Each tumor-bearing mouse received 1 of 5 drugs or normal saline, by intraperitoneal injection, weekly for 5 weeks. Response was monitored for up to 22 weeks, using a staging system which estimates the proportion of the anterior chamber filled by tumor. Results were analysed both as response rates (shrinkage in excess of 50%) at the conclusion of the treatment course and as time to tumor progression by the life table method. Comparison of response rates within cohorts by source of xenografts (exact chi-square test for overall and 2-sided Fisher's exact test for paired comparisons) indicated cyclophosphamide to be the most effective single agent. In logrank analyses cyclophosphamide achieved significantly longer delays to progression than all other drugs in one cohort and longer delays than all but diaziquone in 2 other cohorts. The intraocular xenograft model is a clinically relevant system for the study of therapeutic agents in brain tumors. The effectiveness of intensive dosage cyclophosphamide in a model dependent on access across the blood-aqueous barrier is important and consistent with recent clinical data.

Animals↗
PubMed1989

Neuro-rhinosurgical treatment of aesthesioneuroblastoma.

The results of a combined neuro-rhinosurgical procedure in eight cases of aesthesioneuroblastoma are presented. All patients were suffering from tumours in the advanced stage (stage C according to Kadish). Diagnosis was established by the clinical history, neuro-radiological imaging and by endoscopic endonasal biopsy. Contrary to most reports in the literature the authors performed a one step operative removal of the whole tumour mass by a combined transcranial-transbasal approach alone. A second transfacial operation was unnecessary in all our cases. To the best of our knowledge only Loew (see Jakumeit 1971) already in the 1960ties used a comparable one step transcranial approach for tumour removal. The long-term survival rate in our patients is 50%, a result comparable to reported series in the literature. Mortality is due to early recurrences and metastases within a few months after the initial treatment including post-operative irradiation. Four patients are living without evidence of tumour recurrence 1.5 to 5 years after treatment. The authors surgical technique is presented in detail and compared with the results of other treatment modalities.

Adolescent↗
PubMed1987

Olfactory neuroblastoma metastatic to the eye.

A case of olfactory neuroblastoma metastatic to the choroid is reported and described. Histologically, the tumor consisted of islands of small round cells, many of them surrounding blood vessels in a 'pseudorosette' pattern. Numerous mitotic figures were present. Transmission electron microscopy reveals neuritic processes containing neurosecretory granules and microtubules, features characteristic of olfactory neuroblastoma. This is the first published case of an olfactory neuroblastoma demonstrating intraocular metastasis.

Choroid Neoplasms↗
PubMed1986

Esthesioneuroblastoma: cerebral and spinal metastases without direct cranial invasion.

A patient with esthesioneuroblastoma is reported with metastatic lesions to the cervical nodes, intradural thoracic spinal canal, and the posterior fossa without evidence of local recurrence or cranial bone involvement four years after initial diagnosis and eradication. The initial lesion was a localized nasal polyp apparently treated adequately with excision and radiotherapy.

Brain Neoplasms↗
PubMed1996

Analysis of the neurofibromatosis type 2 gene in different human tumors of neuroectodermal origin.

The autosomal dominant syndrome neurofibromatosis type 2 (NF2) is characterized by the development of bilateral vestibular schwannomas, meningiomas, ependymomas and gliomas. The NF2 gene, recently isolated from chromosome 22, is mutated in both sporadic and NF2 tumors such as schwannomas, meningiomas and ependymomas. Mutations of the gene have been described not only in the neoplasms usually associated with NF2, but also in 30% of the melanomas and 41 % of the mesotheliomas analyzed. In particular, the finding of mutations in melanomas supports the hypothesis that the NF2 gene is involved in the genesis of several tumor types that arise from the embryonic neural crest. In this study we examined, by single-strand conformational polymorphism (SSCP) analysis, 41 tumors of the central nervous system (11 schwannomas and 30 gliomas), 19 melanomas and 15 Merkel cell carcinoma specimens for mutations in the coding sequence of the NF2 gene. We found three inactivating mutations of the NF2 gene in schwannomas. No alterations of the gene were detected by SSCP analysis of the other tumors. These results confirm the role of NF2 in pathogenesis of schwannomas, but do not define its significance in the genesis of the other neuroectodermal tumors studied.

Central Nervous System Neoplasms↗
PubMed1988

Treatment of esthesioneuroblastoma with chemotherapy: a report of two cases.

Esthesioneuroblastoma is an uncommon tumor arising from the olfactory epithelium within the nasopharynx. Conventional treatment consists of surgical resection and irradiation. The use of chemotherapy in limited responses has been reported using cyclophosphamide, thio-TEPA, nitrogen mustard, vincristine, doxorubicin, and chlorambucil as single drugs or in various combinations. Two cases are presented in which neurologic involvement prompted the application of intraventricular methotrexate by an implantable constant infusion drug delivery system in one patient and intra-arterial cis-platinum in combination with intravenous 5-fluorocytosine in a second patient. The tumor of the first patient responded to irradiation followed by methotrexate for four years. The second patient experienced a brief response to the combined chemotherapy following surgery for a recurrent esthesioneuroblastoma. A brief review of the literature regarding chemotherapy and the aggressive form of esthesioneuroblastoma is presented.

Adult↗
PubMed1977

Chronic oral administration of 1-nitrosopiperazine at high doses to MRC rats.

1-Nitrosopiperazine was fed to two groups of rats as drinking water solutions containing 400 mg/liter (3.5 millimolar) and 800 mg/liter (7.0 millimolar), respectively. The treatment was 20 ml per rat per day, 5 days per week for life. In both groups many animals died with olfactory tumors (mostly esthesioneuroblastomas), the first at 36 weeks in the higher dose group, the first at 64 weeks in the lower dose group. There was also a small number of liver tumors in both groups. None of these tumors was seen in the untreated controls. The similarity of this tumor distribution to that produced by 1,4-dinitrosopiperazine suggests that the observed carcinogenicity of 1-nitrosopiperazine may be entirely due to its disproportionation in the acidic medium of the rat stomach. Chemical data supporting this interpretation are presented.

Administration, Oral↗
PubMed2005

Type 1 (11;22)(q24:q12) translocation is common in Ewing's sarcoma/peripheral neuroectodermal tumour in south Indian patients.

The Ewing's sarcoma family can present diagnostic difficulties. In the past the basis of diagnosis has been a exclusion. Identification of a specific translocation especially t(11;22) (EWS-FLI 1 fusion gene), which is seen in nearly 85 percent of Ewing's sarcoma cases can help in precise diagnosis. We have carried out a study on twenty patient samples diagnosed to have Ewing's sarcoma/peripheral neuroectodermal tumour (PNET)/small round cell malignant tumour. The study involved RT-PCR analysis for the fusion transcript, followed by sequencing to identify the specific type of fusion. Ninety percent (18/20) of the samples tested were found to be t(11;22) translocations involving EWS-FLI 1 genes. Sixty-one percent (11/18) were found to be type 1 fusion and seven were type 2 (39 percentage). This is the first study in India with quantitative information about the types of EWS-FLI 1 translocations present in Ewing's family of tumours in south Indian patients.

Adolescent↗
PubMed1995

Insulin-induced differentiation and modulation of neuronal thread protein expression in primitive neuroectodermal tumor cells is linked to phosphorylation of insulin receptor substrate-1.

Neuronal thread proteins (NTPs) are a family of developmentally regulated molecules expressed in central nervous system (CNS) neurons and primitive neuroectodermal tumor (PNET) cell lines. NTP gene expression is modulated with DNA synthesis, neuritic sprouting, and neuronal differentiation. The present study explores the mechanism of insulin modulation of NTP gene expression during neuronal differentiation using PNET cell lines of CNS origin. PNET2 cells underwent neuronal differentiation with neurite outgrowth coupled with transient up-regulation of several species of NTP. In contrast, PNET1 cells failed to differentiate in response to insulin stimulation, although insulin receptors were more abundant than in PNET2 cells. Analysis of the insulin-mediated signal transduction pathway demonstrated that the lack of insulin responsiveness in PNET1 cells was primarily caused by impaired insulin-mediated tyrosyl phosphorylation of the insulin receptor substrate-1 (IRS-1). Correspondingly, the association between phosphatidyl-inositol 3 (PI3) kinase and phosphorylated IRS-1 was reduced in PNET1 compared with PNET2 cells. In contrast, the levels of IRS-1 protein were similar in PNET1 and PNET2 cells, and expression of the insulin receptor beta subunit (Ir beta) and insulin-mediated tyrosyl phosphorylation of the Ir beta were greater in PNET1 than PNET2 cells. The findings suggest that insulin effected neuronal differentiation and modulation of NTP gene expression in PNET cells utilizes a signal transduction cascade that requires tyrosyl phosphorylation of IRS-1.

Blotting, Western↗
PubMed1997

Local spread of malignant neuroepithelial tumors.

A short review of invasiveness of primary malignant neoplasms in the nervous system is given. Invasiveness implies progressive spread and destruction locally, which eventually leads to a fatal outcome in the patient. In particular, the malignant cells are able to rapidly migrate over large parts of the brain. This process includes the capacity to adhere to a substratum, usually constituted by the various components of the extracellular matrix, followed by detachment and migration. Anatomical structures and local regulatory factors in the brain influence the direction and extent of this migration. Several model systems are now available for monitoring the aggressiveness of such tumours both in vivo and in vitro, and different phenotypic properties characteristic of invasive cells have been elucidated. Although still in its infancy, and currently as an experimental approach, anti-invasive therapy may in the future be an interesting alternative to conventional chemotherapy of brain tumours.

Brain Neoplasms↗
PubMed1992

Establishment and characterization of a cell line of congenital primitive neuroectodermal tumor of soft tissue.

A new human cell line, termed Muraoka, has been established from the recurrent tumor of a case of congenital primitive neuroectodermal tumor (PNET) arising at the temporofacial region of a male infant. The microscopic findings of this cell line were epithelioid, and the xenografted tumor in a nude mouse consisted of the malignant epithelioid cells. Immunohistochemically, the cells were positive for neuron-specific enolase, S-100 protein, carcinoembryonic antigen, cytokeratin, epithelial membrane antigen, and glial fibrillary acidic protein. These findings were quite similar to those of the epithelioid cells in the original tumor and of the xenografted tumor cells. Neither chromosomal abnormalities nor N-myc amplification were observed. Morphological differentiation after treatment with N6-2'-O-dibutyryladenosine 3':5'-cyclic monophosphate (Bt2-cAMP), all-trans-retinoic acid (RA), prostaglandin E1 (PGE1), and 5-bromo-2'-deoxyuridine (BrdU) showed two different results. Bt2-cAMP and PGE1 induced neuronal differentiation with the extension of neurites, whereas RA and BrdU predominantly induced Schwannian differentiation (flat cells). In these respects, the cell line Muraoka seems to be useful for studying characteristics of PNET as well as for developing the new treatments against such tumors.

Animals↗
PubMed1998

Is the ancillary chemotherapy approach of any value in the treatment of infratentorial primitive neuroectodermal tumors with surgery and radiotherapy?

A retrospective historical analysis of patients under 18 years of age with the histopathological diagnosis of infratentorial primitive neuroectodermal tumor (PNET) is presented. The survey embraced two different groups of children. Group 1 was defined as those patients treated from 1972 to 1984 with surgical resection plus neuraxis radiotherapy alone. Group 2 was made up of children treated from 1990 to 1996 with the same approach but with the addition of adjuvant chemotherapy: cisplatin (day 1) and etoposide (days 1-3) every 3 weeks for 6 months. Group 1 embraced 42 children with an age range of 1-16 years (mean 6 years, SD 4.4 years). In group 2 there were 34 children, their ages ranging from 1 to 18 years (mean 7.2, SD 4.6 years). The prevalence of stages T2M0 and T3M0 was similar in both groups, but in group 1 there were 4 patients (9.5%) whose spinal fluid was positive for tumor cells (M1), while in group 2 there were 7 children (20.5%) with positive spinal fluid. There was an unequivocal initial response to treatment in 86% of these children in group 1 and in 79% in group 2. The event-free survival (EFS) was 30% at 252 months in group 1, while for group 2 the EFS was 67.6% at 63 months (P 0.002). Mortality from tumor activity was noted in 26 patients (70%) in group 1, while in group 2 mortality attributable to tumor progression was documented in 11 children (32%). We conclude that the use of adjuvant chemotherapy in these patients improves survival without any significant morbidity.

Adolescent↗
PubMed1999

Supratentorial primitive neuroectodermal tumor in children: clinical features, treatment outcome and prognostic factors.

To investigate clinical features, treatment outcome and prognostic factors of pediatric supratentorial primitive neuroectodermal tumors(ST-PNETs), 28 ST-PNET cases were retrospectively analyzed. The prognostic importance of age, sex, size of tumor, M stage, extent of surgical resection, histological features, immunohistochemical labelling indices (Ki-67, p53), and apoptotic index were assessed. The mean age at diagnosis was 6.8 years, and the male-to-female ratio was 18:10. The presenting symptoms in 22 cases were increased intracranial pressure and focal neurological deficits. Gross total resection was achieved in 17 cases, near-total (>90%) resection in 3, and subtotal in 7; biopsy was performed in 1 case. The mean duration of follow-up was 37 months. For 25 patients who completed planned adjuvant therapy, the 3-year survival rate was 73%. Univariate analysis showed that the presence of tumor necrosis (P=0.002) and extent of resection (P=0.04) correlated with survival. Patients with a high Ki-67 labelling index (>10%) tended to have shorter survival (P=0.095). In multivariate analysis, tumor necrosis showed statistical significance(P=0.03).

Adolescent↗
PubMed2002

Epidemiology of primary intracranial tumours in NW Italy, a population based study: stable incidence in the last two decades.

Data about the epidemiology of primary intracranial tumours (PIT) are still heterogeneous depending on different methodological approach in collecting data. In Valle d' Aosta, north west side of Italy, we have carried out a prospective consecutive population based study to calculate the incidence of PIT in the last decade (1992-1999) and to compare these rates with the previous period (1986-1991), data reported in a previous paper. The mean annual PIT incidence rate (ri) per 100,000 inhabitants was 25.48. The mean annual incidence rates in the two period of comparison were adjusted to the 1991 Italian population by the direct method. The standardised ratio was 26.43 in the previous period and 23.24 in the second decade. There is no statistically significant difference. The mean annual PIT incidence rates by tumour types were meningiomas 13.27/100,000 (men 9.77; women 16.7), neuroepithelial group 9.3 (men 10.62; women 8.1), adenomas 1.26, neurinomas 0.7. Mean annual incidence rates by tumour class were also stable. The stable incidence rate in the two periods and the similar incidence in England (21.04/100,000 person year), strengthen the evidence for a stable incidence rate of PIT in the last decade. These three papers used similar methodology. The homogeneous methodology allows comparison and further evaluation.

Adenoma↗