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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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PubMed1991

Prognostic significance of nuclear DNA content in human neuroepithelial tumors.

The relationship between survival and flow cytometric DNA-ploidy and other prognostic factors such as histological subtype, anatomical tumor site, patient sex and age was investigated in 153 patients with intracranial neuroepithelial tumors who underwent surgical treatment. We found a trend toward poorer survival from anaplastic astrocytomas and glioblastomas with respect to low-grade (I and II) astrocytomas (which did not differ significantly); accordingly, patients were grouped into these 3 histologic subgroups. Thirty-seven of the 153 tumors (24.2%) were aneuploid with a median DNA-index (DI) of 1.3 (range: 1.2-2.0). DNA-ploidy correlated with histology, since anaplastic astrocytomas and glioblastomas were significantly (p = 0.041) more frequently aneuploid (around 30%) than low-grade astrocytomas (around 10%). Patients with DNA-aneuploid tumors (i.e., with DI not equal to 1.00) survived for a shorter time (31.4 weeks) than patients with DNA diploid tumors (75.1 weeks) (p less than 0.001). This difference was confirmed by Cox's multivariate analysis. Aneuploid tumors were associated with a poorer survival (p = .0002) when compared with diploid tumors, resulting in a relative risk point estimate (RR) of 2.41, 95% confidence interval (Cl) = 1.55-3.74. Histological subtype was also significantly associated with survival (p less than 0.0001), with RRs of 2.09, 95% Cl = 1.13-3.86 and 3.59, 95% Cl = 1.96-6.59 for anaplastic astrocytomas and glioblastomas, respectively, compared to low-grade astrocytomas. We therefore suggest that the flow cytometric measurement of DNA-ploidy has relevant significance in predicting survival in patients treated for intracranial neuroepithelial tumors.

Age Factors↗
PubMed1995

Neural and mesenchymal differentiations in Ewing's sarcoma cell lines. Morphological, immunophenotypic, molecular biological and cytogenetic evidence.

Three established Ewing's sarcoma (ES) cell lines (TC106, 6647, A4573), grown both in vitro and as xenograft tumors, were analyzed. In all 3 lines and tumors, the ES characteristic reciprocal translocation (11;22), as well as the presence of the ES-associated p30/32MIC2 antigen, were documented. However, these cell lines showed discrepancies in their neural and mesenchymal differentiation. The TC106 line was characterized by expression of the neuroendocrine marker secretogranin II (SgII) which was detectable by Northern blot and by radioimmunological detection (RIA) in the culture medium of secretoneurin, a proteolytic product of SgII. In contrast, TC106 cells were immunohistochemically and radioimmunologically secretoneurin-negative. Rapid cellular secretion of the peptide is probably the explanation of such a discrepancy. The 6647 and A4573 cell lines were SgII/secretoneurin-negative and the former presented morphological and immunocytochemical evidence of mesenchymal differentiation. In fact, the 6647 xenograft tumor showed osteosarcomatous-type morphological features and the neoplastic cells were immunocytochemically positive for osteonectin and osteocalcin antigens. Expression of the CgA gene, which is typical of neuroblastomas, was absent in the Ewing's sarcoma cell lines investigated. Our findings provide experimental evidence that Ewing's sarcoma is a heterogeneous tumor which may show either neuroectodermal or mesenchymal differentiation. SgII/secretoneurin analysis by hybridization and RIA procedures is a reliable approach to the identification of ES with neuroendocrine differentiation.

Animals↗
PubMedDate not supplied

Craniofacial resection for anterior skull base tumors.

We present the results of treatment of 30 patients with anterior skull base tumors operated on over an 11-year period. At the time of surgery, intracranial invasion was present in 10 patients. Histology revealed epithelial tumors in 18 patients, sarcoma in 6, esthesioneuroblastoma in 4, and 2 miscellaneous histologies. The overall median survival was 5 years and varied according to histology and grade of tumor. Currently malignancies involving the skull base can be successfully resected using a craniofacial approach, with minimum operative mortality. Limited intracranial invasion need not necessarily represent a major contraindication of this procedure if morbidity can be kept to a minimum.

Adenocarcinoma↗
PubMed1987

Recurrent olfactory neuroblastoma treated by radiation, surgery, and chemotherapy: "a case report".

Olfactory neuroblastoma is a rare tumor. The role of CT Scan, morphological details and the electron microscopic findings have been highlighted in the diagnosis of the lesion. The present patient, in which the tumor had recurred following surgical excision, was treated successfully with a combination of radiation, further surgery, and maintenance chemotherapy. We also feel that such a planned multidisciplinary approach is justified as the initial treatment for all advanced lesions in view of high recurrence rates reported following use of radiotherapy or surgery alone.

Adult↗
PubMed1979

Teratomas in children and young adults.

A review of all teratomas seen at the University of Wisconsin Hospital between 1965 and 1977 revealed that sacrococcygeal and presacral teratomas were most common. In these cases survival was best in infants less than one year old, with the exception of two cases of malignant medulloepithelioma. Testicular teratomas were predominant in the young adult male, and survival was poor. Pathology and treatment of teratomas are discussed, with an accompanying discussion of congenital anomalies associated with teratomas.

Adolescent↗
PubMed1994

Cytogenetic abnormalities of small round cell tumours.

Between 1987 and 1991, cytogenetic studies were carried out on small round cell tumours of 68 patients from the Northern Health Region of England. Clonal chromosome abnormalities were found in 30, comprising 15 neuroblastomas, 7 Ewing's tumours, 7 rhabdomyosarcomas, and 1 granular cell tumour. Characteristic rearrangements were found in five cases of Ewing's tumour [all with translocation t(11;22) (q24;q12)] and in four cases of rhabdomyosarcoma [all with evidence of translocation t(2;13) (q35-37;q14)]. In one case of Ewing's tumour and three of rhabdomyosarcoma, the cytogenetic findings were important in diagnosis. Within the neuroblastomas, examples were found of hyperdiploidy, 1p rearrangements, double minute chromosomes, and homogeneously staining regions, but too few cases were available for prognostic associations to be assessed. Our findings confirm the diagnostic importance of chromosome abnormalities in small round cell tumours and indicate that cytogenetic analysis should be an intrinsic part of the initial investigations of all patients with such tumours.

Adolescent↗
PubMed2004

Ewing sarcoma: favourable results with combined modality therapy and conservative use of radiotherapy.

BACKGROUND: At the Hospital for Sick Children (HSC), we have treated Ewing sarcoma (ES) with multi-agent chemotherapy, surgery and conservative use of radiotherapy for local control. Our objective was to describe the outcome and prognostic factors associated with this strategy. PROCEDURE: We performed a retrospective chart review of children diagnosed with ES at HSC from Feb 1984 to June 1999. RESULTS: Seventy-two evaluable children were identified. All received chemotherapy. Local control administered was surgery (n = 37), radiation (n = 23), both (n = 10) or neither (n = 2). The 7-year EFS was 66.4%. Recurrence occurred in 23 patients, 7 locally and 16 distantly. Better EFS was associated with male gender (78.5% vs. 52.1%; P = 0.007), localised disease (77.0% vs. 39.4%; P = 0.0004), extremity primary (88.2% vs. 52.8%; P = 0.005) and non-pelvic primary (75.7% vs. 18.2%; P < 0.0001). CONCLUSIONS: Favourable outcomes were seen for patients treated with multi-agent chemotherapy, surgery and conservative use of radiotherapy. Metastatic disease rather than local control was the major cause of failure.

Adolescent↗
PubMed2007

Vincristine sulfate as a possible cause of optic neuropathy.

A 6-year-old boy with skin lesions suggestive of neurofibromatosis developed a frontotemporal primitive neuroectodermal tumor and was subsequently treated with surgery, craniospinal irradiation, and chemotherapy. After the sixth cycle of treatment with vincristine sulfate, 9 months after diagnosis, the child developed a rapidly progressive bilateral deterioration in visual acuity. Retinal appearances were consistent with optic neuropathy. Gene studies for neurocutaneous syndromes were negative. Brain imaging at this time showed no tumor progression, and in the absence of other etiologies, we implicate vincristine as a probable cause. Discontinuation of this particular agent has allowed bilateral improvement in visual acuity.

Child↗
PubMed2008

Ewing sarcoma/primitive neuroectodermal tumor of the kidney in a child.

A 6-year-old female was admitted with abdominal pain and a mass in the right abdomen. Her lactose dehydrogenase level was 1,200 IU/L, and neuron specific enolase was 120 ng/ml. Computed tomography scan confirmed a large right renal mass with necrosis. A right radical nephrectomy was performed. The tumor was completely encapsulated. Based on small round cell histology, strong MIC-2 (CD99) positive tumor cells, and EWS-FLI-1 fusion transcript, Ewing sarcoma/primitive neuroectodermal tumor of the kidney was diagnosed. Induction and follow-up with seven cycles of chemotherapy were given after surgery. She has had no evidence of recurrence 90 months from diagnosis.

Child↗
PubMed2008

Brainstem primitive neuroectodermal tumors (bstPNET): results of treatment with intensive induction chemotherapy followed by consolidative chemotherapy with autologous hematopoietic cell rescue.

We have evaluated the response rate and survival utilizing intensified chemotherapy followed by myeloablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) and adjuvant radiation therapy in six young children with newly diagnosed brainstem primitive neuroectodermal tumors (bstPNET). Following maximum surgical resection of the tumor, patients received high dose induction chemotherapy including vincristine, cisplatin, cyclophosphamide, and etoposide. Eligible patients received a single cycle of myeloablative chemotherapy followed by AuHCR. Two patients survive at least 32 months with stable disease. This approach provides an alternative for young patients with bstPNET who in prior reports have had a uniformly fatal prognosis.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2007

High-dose chemotherapy and autologous stem cell rescue in children with newly diagnosed high-risk or relapsed medulloblastoma or supratentorial primitive neuroectodermal tumor.

BACKGROUND: Single or tandem double high-dose chemotherapy (HDCT) was used to treat children with newly diagnosed high-risk or relapsed medulloblastoma and supratentorial primitive neuroectodermal tumor (MB/sPNET) in order to defer or avoid radiotherapy in young children. PROCEDURE: Thirty-seven HDCTs were given to 25 children with newly diagnosed high-risk or relapsed MB/sPNET. Tandem double HDCT was used for 12 of 15 patients initially intended to receive double HDCT. RESULTS: Three-year EFS (+/-SE) in 6 newly diagnosed high-risk (>3 years old), 8 newly diagnosed (<3 years old), and 11 relapsed MB/sPNET was 83.3 +/- 15.2%, 62.5 +/- 20.5%, and 29.1 +/- 15.7%, respectively. Three-year EFS for patients in CR or PR and in less than PR at first HDCT was 67.4 +/- 11.0% and 16.7 +/- 15.2%, respectively (P = 0.001). Three-year EFS in patients initially intended to receive double HDCT and single HDCT was 66.0 +/- 12.4% and 40.0 +/- 15.5%, respectively. For 19 patients in CR or PR at first HDCT, 3-year EFS was 88.9 +/- 10.5% in tandem double HDCT group, and 44.4 +/- 16.6% in single HDCT group, respectively (P = 0.037). Although four treatment-related mortalities (TRMs) occurred during 25 first HDCTs, no TRM occurred during 12 second HDCTs. In four of eight young children, craniospinal radiotherapy was successfully withheld without subsequent relapse. CONCLUSIONS: High-dose chemotherapy may improve the survival of children with newly diagnosed high-risk MB/sPNET, and, to some extent, the survival of those with relapsed MB/sPNET. Further study is necessary to elucidate the efficacy of tandem double HDCT.

Adolescent↗
PubMed1993

Modulation of POMC expression in human neuroectodermal cells.

Neuroblastoma cell lines have been reported to contain two proopiomelanocortin (POMC) mRNA transcripts. We have now shown by immunocytochemistry and radioimmunoassay (RIA) that a number of neuroectodermally derived cell lines contain immunoreactive beta-endorphin although cell concentrations were not characteristic of any tumour type. To explore further the functional significance of beta-endorphin expression, we analysed neuroblastoma cell lines having intermediate (I), substrate adherent (S) and neuronal (N) phenotypes. No differences in cell beta-endorphin content were detected. However, the expression of POMC mRNA and of immunoreactive beta-endorphin was reduced within a few hours of treatment of these cell lines with retinoic acid. Culture of the cell lines in the presence of beta-endorphin resulted in small but significant increases in growth. Although the POMC gene is in the same chromosomal segment as N-myc, which is normally amplified in neuroblastoma, no corresponding amplification of POMC could be demonstrated. The data suggest that POMC gene products may contribute to the autocrine/paracrine growth of neuroectodermal tumours.

Cell Line↗
PubMed1998

Ceramide-induced apoptosis is mediated by caspase activation independently from retinoblastoma protein post-translational modification.

Recent evidence suggests that untimely retinoblastoma protein (RB) dephosphorylation and/or proteolytic degradation might provide key events down-stream cysteine protease (caspase) activation in apoptosis induction. We have dealt with this issue by studying apoptosis induced by N-hexanoylsphingosine (C6-Cer) in CHP-100 human neuroepithelioma cells, maintained in complete growth medium. We report that C6-Cer-induced apoptosis occurred predominantly in G1/S phases of the cycle and was associated with RB dephosphorylation, in the setting of negligible Bcl-2 expression. Apoptosis was also associated with poly(ADP-ribose) polymerase (PARP) cleavage, thus indicating activation of CPP32/Yama/apopain (caspase-3); however, while the tripeptide caspase inhibitor Z-Val-Ala-DL-Asp-fluoromethylketone was able to prevent both C6-Cer-induced PARP cleavage and apoptosis, it was ineffective in preventing RB dephosphorylation. Moreover proteolytic RB cleavage occurred only to a marginal extent after C6-Cer treatment. These results indicate that apoptosis induced by ceramide in CHP-100 cells is caspase-mediated, but RB post-translational modification does not provide a key step, downstream caspase activation, in apoptosis execution.

Apoptosis↗