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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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At least 217 records · Page 12Linked to original sources
PubMed2002

Farm exposures, parental occupation, and risk of Ewing's sarcoma in Australia: a national case-control study.

OBJECTIVE: It has been suggested that parental occupation, particularly farming, increased the risk of Ewing's sarcoma in the offspring. In a national case-control study we examined the relationship between farm and other parental occupational exposures and the risk of cancer in the offspring. METHODS: Cases were 106 persons with confirmed Ewing's sarcoma or peripheral primitive neuroectodermal tumor. Population-based controls (344) were selected randomly via telephone. Information was collected by interview (84% face-to-face). RESULTS: We found an excess of case mothers who worked on farms at conception and/or pregnancy (odds ratio (OR)=2.3. 95% confidence interval (CI) 0.5-12.0) and a slightly smaller excess of farming fathers, more case mothers usually worked as laborers, machine operators, or drivers (OR = 1.8, 95% CI 0.9-3.9). Risk doubled for those whose mothers handled pesticides and insecticides, or fathers who handled solvents and glues, and oils and greases. Further, more cases lived on farms (OR= 1.6, 95% CI 0.9-2.8). In the 0-20 years group, the risk doubled for those who ever lived on a farm (OR = 2.0, 95% CI 1.0-3.9), and more than tripled for those with farming fathers at conception and/or pregnancy (OR = 3.5, 95% CI 1.0-1 1.9). CONCLUSIONS: Our data support the general hypothesis of an association of Ewing's sarcoma family of tumors with farming, particularly at younger ages. who represent the bulk of cases, and are more likely to share etiologic factors.

Adult↗
PubMed2002

Primary peripheral PNET/Ewing's sarcoma of the dura: a clinicopathologic entity distinct from central PNET.

We describe two cases of peripheral primitive neuroectodermal tumor-Ewing's sarcoma (PNET-ES) arising intracranially in the leptomeninges. Both tumors exhibited a primitive undifferentiated round-cell morphology. Immunohistochemical stains revealed strong membrane expression of CD99 in both cases. A t(11;22)(q24;q12) could be demonstrated with reverse transcriptase-polymerase chain reaction in one case, whereas fluorescence in situ hybridization analysis performed in the second case showed a rearrangement of the EWS gene. The occurrence of PNET-ES at this site is very unusual. Immunophenotypical as well as genetic analysis play a key role in the diagnosis and the distinction from central PNET.

12E7 Antigen↗
PubMed2002

Radiotherapy for metastases to the mandible in children.

PURPOSE: We present a retrospective review of all children treated since 1979 at our institution with radiotherapy for symptomatic metastases that involve the mandible. PATIENTS AND METHODS: Nine children were treated with 1 or more courses of radiotherapy for symptomatic metastases that involve the mandible. Six children had a neuroblastoma, 1 had angiosarcoma of the liver, 1 had adenocarcinoma of the rectum, and 1 had peripheral primitive neuroectodermal tumor (Ewing's sarcoma) of the spine. In 3 children, the mandible was the first bone involved by metastases. Seven children were treated with short intensive courses of radiotherapy consisting of 1 to 3 fractions to a total dose of 400 to 1,200 cGy. One child received 2,400 cGy in 6 fractions, and another child received 3,000 cGy in 10 fractions. Three children were treated with second courses of radiotherapy at 1, 2, and 5 months, respectively, from the initial course of radiotherapy. All children had received chemotherapy. RESULTS: All children died of disseminated disease at 5 to 59 months from their initial diagnosis, 5 to 29 months from the detection of metastases to bone, and only 6 days to 17 months (median, 2 months) from the first treatment of metastases to the mandible. CONCLUSIONS: The outlook for children with metastases that involve the mandible is very poor, and we recommend short intensive courses of radiotherapy consisting of 1 to 3 treatments to total doses of 400 to 1,200 cGy for palliation of pain.

Adenocarcinoma↗
PubMedDate not supplied

EWS/FLI-1 fusion transcript detection and MIC2 immunohistochemical staining in the diagnosis of Ewing's sarcoma.

Ewing's sarcoma (ES) and other primitive peripheral neuroectodermal tumors (pPNETs) can present a significant diagnostic problem, as they may morphologically resemble other small round cell tumors (SRCTs) of childhood. However, ES/pPNET is known to carry a characteristic t(11;22)(q24;q12), the detection of which may aid diagnosis. The recent identification of the EWS and FLI-1 genes flanking the translocation break point has enabled reverse transcriptase-polymerase chain reaction (RT-PCR) to be used to detect the putative chimeric transcription factor mRNA produced by the fusion gene. We have assessed the RT-PCR method of detection by examining 40 cases of ES for the presence of EWS/FLI-1 transcripts. Twenty-six (76%) of the 34 cases with intact mRNA yielded fusion transcripts. Four different transcript sizes were detected and two tumors contained two transcripts of different size. No transcripts were detected in a control group of non-ES/pPNET SRCTs. Eight cases with intact mRNA were transcript negative. The MIC2 cell surface antigen, which is reported to be present in over 95% of ES/pPNETs, was present in 32 of 33 tumors (97%), including all 24 EWS/FLI-1 transcript-positive cases examined. Hence MIC2 is a useful screen for ES, with RT-PCR detection of t(11;22) being the optimal method for confirming the diagnosis.

12E7 Antigen↗
PubMed1992

Cytogenetic and experimental models.

The sarcomas, particularly those of soft tissue origin, often contain characteristic immunohistochemical and cytogenetic features that are of diagnostic relevance. Over the past year, novel findings have been reported that are likely to improve present sarcoma diagnostic capabilities. Findings of particular interest have been reported for Ewing's sarcoma, peripheral primitive neuroectodermal tumor, rhabdomyosarcoma, and clear cell sarcoma.

12E7 Antigen↗
PubMed1994

Cytogenetics and experimental models of sarcomas.

Many sarcomas contain characteristic chromosome translocations that result in the fusion of genes from two different chromosomes. This review focuses on the translocated genes that have been identified recently in Ewing's sarcoma, peripheral primitive neuroectodermal tumor, clear cell sarcoma, alveolar rhabdomyosarcoma, and myxoid liposarcoma. Each of these fusion genes encodes a DNA transcription factor that presumably regulates the function of other genes. Thus, a common mechanism of oncogenesis has been defined in histologically disparate varieties of sarcoma.

Adult↗
PubMedDate not supplied

Reverse transcriptase polymerase chain reaction for detecting Ewing's sarcoma in archival fine needle aspiration biopsies.

OBJECTIVE: Previous studies on pediatric soft tissue sarcomas have demonstrated a chromosomal 11;22 (q24;q12) translocation in Ewing's sarcoma (ES) and peripheral primitive neuroectodermal tumors that appears to be a unique oncogenic marker. To investigate the usefulness of reverse transcriptase polymerase chain reaction (RT-PCR) as an ancillary method for cytodiagnosis, we tested archival aspirates derived from ES patients to establish whether any beta-actin RNA expression or tumor-specific EWS/FLI-1 gene translocation had occurred. STUDY DESIGN: Sixteen skeletal specimens aspirated 10-15 years earlier from patients with cytologic and histologic diagnoses of ES were prepared for PCR. The amplification products were sequenced. RESULTS: Amplifiable RNA was detected in smears from 12 patients by beta-actin RT-PCR. Seven aspirates from beta-actin-positive patients showed ES-specific genomic translocation (58%). Sequence analysis of the resulting PCR fragments revealed EWS/FLI-1 fusion transcriptions of varying length. CONCLUSION: When RNA was retrievable, RT-PCR applied to routinely stained aspirate smears was a highly specific method in differential diagnosis of ES.

Base Sequence↗
PubMed2006

How to treat the Ewing's family of sarcomas in adult patients.

Ewing's sarcoma, peripheral primitive neuroectodermal tumor, and Askin's tumor comprise a single family of tumors, the Ewing's family of tumors, which is characterized by chromosomal translocation. Ewing's sarcoma is known as a malignancy of childhood, but with a median age of 15 years at diagnosis, it should equally be regarded as a malignancy of adolescence and young adulthood. There is much controversy regarding the role of age at diagnosis, with some studies showing older age to be associated with poorer outcome and others showing no association between age and survival. This has led to uncertainty in how best to manage nonpediatric patients with Ewing's sarcoma. This article examines whether age does affect outcome and treatment in this group of tumors.

Adolescent↗
PubMedDate not supplied

Signal transduction pathway to low-dose radiation-induced apoptosis in peripheral PNET cells.

We investigated the signal transduction pathway to low-dose radiation-induced apoptosis in vitro in the human peripheral primitive neuroectodermal tumor (pPNET) cell line with wild-type p53 established in our laboratory. Apoptosis was induced by 2Gy irradiation in an almost p53-dependent manner in this model except for a deficiency of the cleavage of caspase-9. It was detected 3 hours after irradiation by fragmentation assay. The expressions of p53, p21WAF-1 and Bax increased, in contrast to the gradually decreasing expression of Bcl-2, as observed by immunoblotting. Following this, cleavages of caspase-3 and PARP reached peak levels. There were no detectable increases in ERK expression and caspase-9 cleavage. In respect of the probability of other pathways to apoptosis, this cell line will provide a useful model both for investigating low-dose radiation-induced signal transduction pathway and for analyzing the biological characteristics of pPNET.

Adult↗
PubMed2004

Ovarian neoplasms composed of small round cells: a review.

Ovarian neoplasms composed predominantly or exclusively of small round cells with scant cytoplasm are relatively rare. However, there is a wide differential, and pathologists often struggle to make a correct diagnosis because of overlapping histologic features. Perhaps the best known of these neoplasms is ovarian small cell carcinoma of hypercalcemic type (OSCCHT), a tumor of unknown histogenesis. This may be confused with a wide range of neoplasms ranging from sex cord-stromal tumors (some of which may exhibit a small cell phenotype) to neoplasms in the family of small round blue cell tumors to various undifferentiated malignancies. A neuroendocrine small cell carcinoma, so-called small cell carcinoma of pulmonary type, may also arise within the ovary, and this may be a component of a typical ovarian surface epithelial-stromal tumor. In addition to the well-known family of small round blue cell tumors of childhood, other small cell neoplasms that may arise within the ovary or involve the ovary include intra-abdominal desmoplastic small round cell tumor, metastatic small cell carcinoma, peripheral and central primitive neuroectodermal tumor, and endometrial stromal sarcoma. Malignant melanoma, undifferentiated carcinoma, and various germ cell tumors, especially dysgerminoma and immature teratoma, also on occasion enter into the differential diagnosis of an ovarian small cell neoplasm. In this review, the morphologic features of some of these neoplasms are described, as is the value of immunohistochemistry and other ancillary techniques in establishing a diagnosis.

Abdominal Neoplasms↗
PubMedDate not supplied

Peripheral neuroepithelioma developing in the abdominal cavity.

Peripheral neuroepitheliomas are rare malignant tumors of presumed neural crest origin, arising outside the central and sympathetic nervous system. We present herein a case of a young man with diffuse peripheral neuroepithelioma of the abdominal cavity. Although we twice attempted a debulking procedure on this patient, and treated him with alternating VIP-CAV combined chemotherapy, his disease showed a relentless course without responding to any treatment. The development of peripheral neuroepithelioma in the abdominal cavity is an extremely rare occurrence. These rare malignant tumors, of presumed neural crest origin, arise outside the central and sympathetic nervous systems (1). Other names for this tumor are: extracranial primitive neuroectodermal tumor (PNET), Askin tumor, peripheral or adult neuroblastoma (2,3,4). We present a case of a young man with diffuse development of this tumor in the abdominal cavity.

Abdominal Neoplasms↗
PubMed1995

Ewing's sarcoma and extracranial peripheral neuroectodermal tumors.

Ewing's sarcoma and extracranial primitive neuroectodermal tumors of bone and soft tissue are small round-cell tumors that comprise a family of neoplasms distinguished by a cytogenetic marker that can be detected by reverse transcription and polymerase chain reaction. Based on molecular and immunohistochemical studies, it is generally acknowledged that this is a family of neuroectodermal tumors. Molecular methods allow accurate diagnosis with minimally invasive surgical options, provide a manner to detect minimal residual disease, and begin to shed light on the pathogenesis of these entities. Combined modality treatment regimens continue to improve the outlook for nonmetastatic Ewing's sarcoma and primitive neuroectodermal tumors patients. Patients who present with metastatic disease may benefit from intensive therapy as facilitated by peripheral stem cell and autologous bone marrow rescue programs.

Antineoplastic Combined Chemotherapy Protocols↗
PubMedDate not supplied

Malignant peripheral neuroectodermal tumor (MPNET) of the kidney.

BACKGROUND: Malignant peripheral neuroectodermal tumors (MPNETs) are primitive neuroblastic tumors that arise, unlike neuroblastomas, outside the autonomic nervous system. A renal origin has been described in very few cases. CASE REPORT: We report the case of a young male patient with a large MPNET of the right kidney, studied with ultrasound and computed tomography before surgical resection. The main radiologic features, the microscopic appearance and the typical immunohistochemical findings, are described and discussed.

Adult↗
PubMed2003

Schwannoma with rhabdomyoblastic differentiation: a unique variant of malignant triton tumor.

A 54-year-old woman presented with intractable perianal, bilateral buttock, and radiating thigh/calf pain. An MRI scan showed an intradural, contrast-enhancing, ovoid mass in the cauda equina region at L1-L2. At laminectomy, the ovoid mass arose from a nerve root and, intact, was gross totally resected. Histologically, the dominant pattern was that of schwannoma. One year thereafter, the symptoms recurred. An MRI scan demonstrated an irregular, heterogeneously enhancing tumor recurrence. A repeat laminectomy disclosed a large fleshy tumor involving multiple nerve roots. The lesion was subtotally resected and showed pluridirectional differentiation toward embryonal rhabdomyosarcoma, primitive neuroectodermal tumor, and rare malignant epithelial cells. Review of the original tumor disclosed only foci of embryonal rhabdomyosarcoma and primitive neuroectodermal tumor. Based upon available data regarding divergent differentiation in peripheral nerve sheath tumors, this is a unique, previously undescribed tumor demonstrating rhabdomyosarcomatous, primitive neuroectodermal tumor and scant epithelial differentiation in a schwannoma. In essence, it is a variant of malignant Triton tumor because of its origin in a tumor consisting of well-differentiated Schwann cells. It supports the contention that the Schwann cell is the source of a variety of heterologous elements in nerve sheath tumors.

Cauda Equina↗
PubMed2006

Peripheral primitive neuroectodermal tumor/Ewing's sarcoma of the craniospinal vault: case reports and review.

The peripheral primitive neuroectodermal tumor/Ewing's sarcoma family tumor (pPNET/ESFT) group includes small round cell tumors of the bone, soft tissue, and nerve with morphological attributes of the germinal neuroepithelium. Peripheral PNETs/ESFTs also occur within the craniospinal vault, a region including the central nervous system, the meninges, and the cranial and spinal nerve roots. Gene rearrangements between the EWS gene on chromosome 22q12 and members of the ETS gene family are common in and specific to pPNETs/ESFTs. Another defining characteristic of pPNETs/ESFTs is their membranous expression of the MIC2 gene product. We describe 2 cases of pPNETs within the craniospinal vault. An intradural tumor arising from the nerve roots of the cauda equina was discovered in a 32-year-old man presenting with radiculopathic back pain and lower-extremity weakness. An intracranial pPNET that mimicked a meningioma was found in a 21-year-old man presenting with headache and visual disturbances. MIC2 gene product expression and EWS/ETS gene rearrangement were detected in both case patients. The literature with regard to pPNETs/ESFTs arising within the craniospinal vault is reviewed.

12E7 Antigen↗
PubMed1998

[Differential diagnosis of distal peripheral nerve compression syndrome of the tibial nerve. Case report of primitive neuroectodermal tumor].

Tumors of the tibial nerve are not sufficiently taken into account in the diagnosis of tarsal tunnel syndromes (TTS). They may present with atrophy of the plantar flectors and with disturbances of sensory functions, but far more commonly with pain. Diagnosis is often only achieved after 1-2 years. Taking tumors into account when examining patients presenting with TTS may lead to a more rapid diagnosis and to improvement in patients' prognoses. We present the case of a 37-year-old female in whom a primitive neuroectodermal tumor (PNET) was found to be the cause of long-standing pain in the right foot.

Adult↗
PubMed1983

Primitive neuroectodermal tumors of the central nervous system.

Primitive neuroectodermal tumors are morphologically similar malignant tumors arising in intracranial and peripheral sites of the nervous system, showing varying degrees of cellular differentiation with a tendency to disseminate along cerebrospinal fluid pathways. They occur primarily in children and young adults. Under the designation primitive neuroectodermal tumors are included medulloblastomas and tumors that may differentiate in other directions, such as medulloepithelioma, neuroblastoma, polar spongioblastoma, pineoblastoma, ependymoblastoma, retinoblastoma, and olfactory neuroblastoma. From a practical, histologic point of view, these tumors are often indistinguishable from one another and are best thought of as primitive neuroectodermal tumors with or without differentiating features.

Animals↗
PubMed1995

Primitive neuroectodermal kidney tumor: 2 case reports and review of the literature.

Peripheral neuroectodermal tumors are uncommon cancers arising from outside the central nervous system. The urinary system is rarely involved. The differentiation of these tumors from other small cell cancer and neuroblastoma is based on immunohistochemical differences. We report 2 cases of such tumors arising from the renal parenchyma. Tumor behavior and treatment modalities are discussed.

Adult↗