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 307 records · Page 17Linked to original sources
PubMed1987

Differential protooncogene expression characterizes histopathologically indistinguishable tumors of the peripheral nervous system.

We have found highly predictable patterns of protooncogene expression in cell lines and tumor tissue of neuroblastoma (NB), a tumor of the peripheral nervous system (PNS). These patterns make it possible to recognize two different genetically definable subgroups among histopathologically indistinguishable tumors. Additionally, we have identified a difference in neurotransmitter biosynthetic enzyme activity in these two subgroups of NB. The patterns of protooncogene expression and neurotransmitter biosynthetic enzymes suggests that these tumors arise in different cells of the PNS.

Cell Line↗
PubMed1996

Comparison of soft tissue Ewing's sarcoma and peripheral neuroectodermal tumor.

Thirty-four cases with either extraskeletal Ewing's sarcoma (21 patients) or malignant peripheral neuroectodermal tumor of the soft tissues (13 patients) were reviewed retrospectively. The patients were treated between 1964 and 1991. Followup periods averaged 7.2 years for patients with peripheral neuroectodermal tumors and 10.4 years for patients with Ewing's sarcoma (minimal followup of 2 years). There were no significant differences in patient's age, gender, tumor location, and stages on presentation between patients with Ewing's sarcoma and those with malignant peripheral neuroectodermal tumor of the soft tissue. All but 2 patients underwent surgery for tumor resection. Adjunctive therapy (radiation or chemotherapy or both) was administered in 95% of the patients with Ewing's sarcoma and in 85% of the patients with peripheral neuroectodermal tumors. The 5-year overall survival was 50% for Ewing's sarcoma and 44% for peripheral neuroectodermal tumor. The 5-year disease free survival was 33% for both types of tumors. Survival rates were higher for Stage III compared with Stage IV disease for both types of tumors. There was a tendency for better outcome after complete surgical tumor resection. Survivors tended to be of a younger age at the time of tumor diagnosis. The results suggest that from a clinical perspective, these 2 tumors are quite similar.

Adolescent↗
PubMed1995

Malignant neuroepithelioma of the colon.

A rare case of malignant peripheral neuroepithelioma originating from the right colon is presented. The patient underwent right hemicolectomy followed by combination chemotherapy and there has been no evidence of tumour recurrence or metastases during three years of follow up. Emphasis is given to the extremely unusual location of this tumour and the favorable clinical outcome.

Adult↗
PubMed1997

[Malignant non-epithelial tumors of the pancreas].

Nonepithelial malignant tumors of the pancreas are extremely rare neoplasms with a frequency of approximately 0.6%. They are always explored because of a suspected diagnosis of pancreatic carcinoma. Amongst the more than 600 primary pancreatic neoplasms in our pancreatic tumor archive only 5 neoplasms were of nonepithelial origin (one was a malignant peripheral nerve sheath tumor [MPNST], one a leiomyosarcoma, one a malignant mesothelioma, and two were peripheral neuroectodermal tumors [PNET]. The differential diagnosis includes secondary infiltration of the pancreas by mesenchymal tumors of the retroperitoneum, undifferentiated pancreatic carcinoma and, especially in the case of PNET, malignant lymphoma. Preoperative chemotherapy and down-staging can improve the operability and prognosis, especially in PNET.

Adolescent↗
PubMed1989

Malignant neuroepithelioma (peripheral neuroblastoma). A case report.

Malignant neuroepithelioma is a rare neoplasm arising within the peripheral nervous system. It usually occurs in the lower extremities and may involve patients in any age group. In children younger than the age of five years, the tumor must be differentiated from a metastatic neuroblastoma; in adolescents and adults the tumor must be distinguished from other malignant round-cell tumors. The poor prognosis and the need for aggressive, combined surgical and chemotherapeutic modalities in treating this tumor necessitate a prompt and accurate diagnosis.

Adult↗
PubMed1990

Peripheral neuroectodermal tumour (neuroepithelioma) of the thoracopulmonary region: light and electron microscopic cytology.

We describe a case of peripheral neuroectodermal tumour of the thoracopulmonary region diagnosed by fine needle aspiration cytology. Light microscope examination revealed numerous small tumour cells arranged in large irregular aggregates occasionally delimiting empty vascular-type spaces or forming rosette-like structures. Cytologically the tumour cells showed a marked degree of nuclear anaplasia, scanty cytoplasm and long thin cytoplasmic processes. Electron microscopy revealed cells with characteristics of neuroectodermal differentiation.

Humans↗
PubMed1991

Case report 682. Neuroepithelioma of soft tissues of the knee.

Peripheral neuroepitheliomas should be included in the differential diagnosis of a soft-tissue tumor in the extremities or chest wall, especially in young adolescents. MRI provides useful information as to the extent of the tumor and relationship with adjacent structures, although it may not differentiate neuroepitheliomas from other, more common, soft-tissue sarcomas. Together with clinical and histological findings, MRI can assist in the diagnosis and treatment planning of this tumor.

Adolescent↗
PubMed1996

[Peripheral neuroectodermal tumor of the thoracic wall. Experiences with surgical treatment].

The peripheral neuroectodermal tumor (PNET) is seen nowadays as a more specified form of Ewing's Sarcoma with a worse prognosis. We report our experiences of three children (10-14 years old) with PNET of the right thoracic wall, all treated surgically within 1 year. Complete local resection-the main aspect of PNET therapie-of the initially voluminous tumors was not difficult due to chemotherapy and radiotherapy for 5 months prior to surgery.

Adolescent↗
PubMed1998

[Similarities between pleuropulmonary blastoma and Askin tumor].

Adult pleuropulmonary blastoma is an uncommon mesenchymal tumor of the peripheral lung parenchyma composed of undifferentiated cells. The differential diagnosis with Askin-Rosaï's tumor, an extrapulmonary pleuroparietal neuroectodermal tumor, may be a difficult task. We propose a similar therapeutic approach for these two malignant conditions: surgical excision associated with chemotherapy and autologous bone marrow transplant with adjuvant radiotherapy in case of recurrence. These two entities have many epidemiological, clinical, histological and immunohistochemical similarities.

Adult↗
PubMed1993

Round cell tumours of bone.

Ewing's sarcoma is a very rare tumor which has, however, attracted much oncological interest since the dramatic improvement of its prognosis under chemotherapy. Its histogenesis has been discussed controversially for a long time, including a possible origin in immature reticulum, myogenous, endothelial and undifferentiated mesenchymal cells. Repeated reports have also suggested a possible neuroectodermal genesis. Convincing arguments, however, have only been brought forward during recent years, since it was found out that Ewing's sarcoma and malignant peripheral neuroectodermal tumor share a common chromosome translocation 11;22. In the meantime this hypothesis has been strengthened by numerous cell biological analyses. Histological differential diagnosis of Ewing's sarcoma has been improved by immunohistological methods. In most cases, they can be distinguished from lymphoma (leucocyte common antigen, B and T markers) and embryonal rhabdomyosarcoma (muscle specific actin, desmin). Apart from this, we now have an antibody specific for Ewing's sarcoma and malignant peripheral neuroectodermal tumors but not with neuroblastoma. Recent investigations regarding the prognosis under chemotherapy have shown that tumors with neural differentiation have a comparatively poor prognostic outcome. This is why in addition to the demonstration of Homer Wright pseudo-rosettes different neural markers such as neuron specific enolase, synaptophysin and chromogranin are to be analysed. Small cell osteosarcoma is a very rare sub-entity of osteosarcomas. Recent studies have shown that the tumor resembles Ewing's sarcoma with partial mesenchymal differentiation including osteoid formation.

Adolescent↗
PubMed1996

Small round blue cell tumors in bone: prognostic factors correlated to Ewing's sarcoma and neuroectodermal tumors.

Ewing's sarcoma and peripheral neuroectodermal tumors are the most common small round blue cell tumors of bone. Accurate prognostic factors are required to define guidelines to standardize the treatment modalities and to adapt these modalities to the potential evolution of the disease. The various factors that have emerged in the literature as influences on the outcome of patients with Ewing's sarcoma or peripheral neuroectodermal tumors of bone are considered in this review. The presence of metastases at the clinical onset of the disease represents the most adverse prognostic factor. For nonmetastatic patients, axial location appears to be the most unfavorable factor, despite initial tumor volume, by showing that the tumor burden could be a more appropriate indicator of patient outcome. The importance of a local control of the disease by surgery has been emphasized, and the value of the histopathologic evaluation of the response to chemotherapy has been stressed.

Age Factors↗
PubMed2005

An ovary in luteal phase mimicking common iliac lymph node metastasis from a primary cutaneous peripheral primitive neuroectodermal tumour as revealed by 18-fluoro-2-deoxyglucose positron emission tomography.

A 30-year-old female underwent surgical removal of a primary cutaneous peripheral primitive neuroectodermal tumour (PNET) of the left thigh. A subsequent 18-fluoro-2-deoxyglucose (FDG) positron emission tomography (PET) scan showed abnormal accumulation of FDG in the left upper pelvic region, consistent with metastasis to a left common iliac node. A series of follow-up imaging studies revealed that a cyst of the corpus luteum of ovary was responsible for the abnormal FDG accumulation.

Adult↗
PubMed2008

Familial Currarino syndrome presenting with peripheral primitive neuroectodermal tumour arising with a sacral teratoma.

This report illustrates a rare genetic disorder, Currarino syndrome, in association with an unusual malignant transformation to a peripheral primitive neuroectodermal tumour within a sacral teratoma. The triad of features consists of a presacral mass, partial sacral agenesis and anorectal anomalies. The most common presentation is constipation. In this case there was a history of constipation, teratomas and spinal abnormalities in many of the family members over three generations. Detailed family history taken at time of initial presentation may have prevented delay in diagnosis and averted the need for intensive treatment, which may well cause late sequelae.

Child, Preschool↗
PubMed1988

Peripheral neuroepithelioma in childhood. Immunohistochemical demonstration of epithelial differentiation.

Peripheral neuroepithelioma arising from the chest wall of a 4-year-old girl is described. She died of local recurrence 15 months after surgery. Light- and electron-microscopic as well as immunohistochemical findings confirmed the neuroectodermal nature of the tumor. A distinctive histologic feature was the presence of clustered epitheliallike cells, which immunohistochemically stained positive for both keratin and carcinoembryonic antigen. The epithelial nature of these peculiar cells is presented.

Carcinoembryonic Antigen↗
PubMed2005

Primitive neuroectodermal tumours (PNETs) located in the spinal canal; the relevance of classification as central or peripheral PNET : case report of a primary spinal PNET occurrence with a critical literature review.

Intraspinal location of central PNET (cPNET) is very rare. We present a case, critically review all publications of primary intraspinal cPNET occurrence and discuss tendencies in clinical presentation. In several previous attempts to summarise, authors often confused cPNET with peripheral PNET (pPNET). cPNET and pPNET are different entities with different immunohistochemical profiles and genetic backgrounds. Clinically, they are both aggressive tumours, but exhibit different characteristics in their local manifestation and metastatic spread. Survival rates are quite similar provided that treatment is applied according to the established protocols. Protocols in cPNET treatment differ from those for pPNET as regards the order of the treatment sub-modalities, specific chemotherapeutic regimen and intensity, radiation dose and its extent and consequently, the side effects. Therefore, failure to distinguish cPNET from pPNET leads to clinical guidance and treatment proposals based on false assumptions, which might effect outcomes. Often, distinguishing between cPNET and pPNET is easy, because they occur in different location. In the case of intraspinal tumour location, however, the differentiation is crucial because both primary cPNET and pPNET can occur intraspinally, even though this is rare. Nowadays, demonstrating the expression of MIC2 glycoprotein by immunocytochemical staining (CD99) showing the specific EWS-FLI1 chimeric gene presence in pPNET, offers an easy way of making a differential diagnosis between cPNET and pPNET.

12E7 Antigen↗
PubMed1989

Establishment and characterization of a small round cell sarcoma cell line, SCCH-196, with t(11;22)(q24;q12).

A cell line designated SCCH-196 was established from an extraskeletal small round cell sarcoma developed in a 16-year-old Japanese girl. The cells grew as a monolayer, and have been continuously propagated by serial subcultures during the past 26 months. Cells from the primary tumor and those from the SCCH-196 cell line at passage 10 both showed the same karyotype, 51,XX, +8, +20, +21, t(11;22)(q24;q12), +i(1q), +i(1q). Histologically the primary tumor was difficult to classify as either Ewing's sarcoma (ES) or peripheral neuroepithelioma (NE). Neuron-specific enolase-positive cells in the primary tumor and the occurrence in the upper extremity were in favor of NE, while positive reaction of SCCH-196 cells to an ES-specific monoclonal antibody 5C11 suggested a diagnosis of ES. The SCCH-196 cell line may be useful for basic studies on differentiation of neuroectodermal tumors, and for future cloning of still unidentified genes which may be located at the breakpoints of the 11;22 translocation.

Adolescent↗
PubMed1991

Biology of pediatric peripheral neuroectodermal tumors.

The pediatric peripheral neuroectodermal tumors which include neuroblastoma, peripheral neuroepithelioma and Ewing's sarcoma may correspond to distinct neural crest cell lineages or tumors arrested at different stages of neural crest development. Besides a brief commentary on the salient clinical features of these tumors, this review examines how cell and molecular biological studies have contributed to a re-classification of these tumors. The differentiation of these tumors is reviewed with a particular emphasis on retinoic acid induced differentiation of neuroblastoma as a model to identify genes important in controlling cell growth, suppression of tumorigenicity and induction of differentiation.

Adolescent↗
PubMed1992

Calcineurin, a calcium/calmodulin-regulated protein phosphatase, in mammalian neuroendocrine cells and neoplasms.

Calcineurin is a calcium/calmodulin-regulated protein phosphatase. By using enzyme-immunoassay and immunocytochemistry with an affinity-purified specific antibody to this protein, we have found that calcineurin is expressed in the central and peripheral neuroendocrine cells, also termed amine precursor uptake and decarboxylation cells. In addition, calcineurin immunoreactivity was found in the central neuroendocrine neoplasms such as pineocytoma, olfactory neuroblastoma and paraganglioma. The present findings indicate that the activity of phosphatase regulated by calcium and calmodulin is involved in neuroendocrine functions, and that the enzyme can be useful for the identification and characterization of neuroendocrine cell tumors.

Animals↗