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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↗
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↗
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↗
PubMed1998

Poorly differentiated synovial sarcoma: immunohistochemical distinction from primitive neuroectodermal tumors and high-grade malignant peripheral nerve sheath tumors.

Synovial sarcoma is a relatively common sarcoma in adults, which in its classic bimorphic form infrequently poses a diagnostic problem. Monomorphic spindled variants, as well as the less common poorly differentiated variants, may be confused with other soft-tissue sarcomas; the poorly differentiated variant (PDSS), in particular, may be histologically indistinguishable from other small, blue, round cell tumors, including primitive neuroectodermal tumors (PNETs). Detection of the synovial sarcoma-associated t(X;18) by either cytogenetic or molecular genetic approaches may be necessary to confirm the diagnosis of synovial sarcoma in difficult cases. We evaluated 10 cases of PDSS from eight patients using a panel of antibodies (including those to intermediate filament proteins, nerve-sheath associated markers, and neuronal and neuroectodermal associated markers) in order to better establish the immunophenotype of this tumor and to help distinguish it from the tumors with which it may be confused, particularly PNETs and high-grade malignant peripheral nerve sheath tumors (MPNSTs). Our results showed PDSS to have significant immunophenotypic overlap with both PNETs and MPNSTs. In most instances these three entities may be differentiated by a panel of antibodies that should include those to both low and high molecular weight cytokeratins, epithelial membrane antigen, type IV collagen, CD99, CD56, and S-100 protein. Our results also suggest that synovial sarcoma may be a tumor showing combined neuroectodermal and nerve sheath differentiation--perhaps because of translocation-associated expression of specific proteins--rather than a carcinosarcoma of soft tissues or a tumor of specialized arthrogenous mesenchyme.

12E7 Antigen↗
PubMed1993

Detection of the (11;22)(q24;q12) translocation of Ewing's sarcoma and peripheral neuroectodermal tumor by reverse transcription polymerase chain reaction.

Ewing's sarcoma and the related primitive neuroectodermal tumor (PNET) share a unique and specific t(11;22)(q24;q12) chromosomal translocation. The breakpoints have recently been cloned and shown to involve the EWS gene on chromosome 22 and the FLI-1 gene on chromosome 11. Translocation results in the fusion of these genes on the der(22) chromosome, resulting in the production of a novel chimeric EWS/FLI-1 message. Using oligonucleotide primers derived from EWS and FLI-1 complementary DNAs, we were able to amplify a specific fusion transcript from 18 of 18 cases containing t(11;22) and 10 of 14 cases of Ewing's sarcoma/PNET that had unsuccessful cytogenetics. No EWS/FLI-1 fusion transcripts were detected in five cell lines derived from cases of pediatric sarcomas having a histological diagnosis other than Ewing's sarcoma/PNET. The sensitivity and specificity of this PCR analysis demonstrates the usefulness of this approach for the primary diagnosis of t(11;22)-containing Ewing's sarcoma/PNET and for the detection of metastatic or residual disease.

Amino Acid Sequence↗
PubMed1989

N-myc amplification and neuronal differentiation in human primitive neuroectodermal tumors of the central nervous system.

Despite the phenotypic similarities between primitive neuroectodermal tumors of the central nervous system, childhood neuroblastoma, and peripheral neuroepithelioma, a histogenetic relationship among these neoplasms has not been shown. High levels of N-myc expression occur selectively in developing brain and in some embryonic tumors of neural origin. N-myc amplification and high levels of N-myc expression in childhood neuroblastoma have been correlated with disease stage and prognosis. To determine whether the copy number of the N-myc gene in primitive neuroectodermal tumors of the central nervous system is altered, we examined 20 primitive neuroectodermal tumors by Southern and/or slot blot hybridization to a 1-kilobase N-myc genomic DNA sequence and a 492-base pair N-myc-specific subclone as well as to a 1.1-kilobase albumin complementary DNA sequence as a control for gene copy number. Amplification of the N-myc gene was detected in two cerebellar tumors both of which exhibited neuronal differentiation by light microscopy. These tumors had not been treated previously. Of the remaining 18 tumors, eight were undifferentiated, three showed early neuroblastic, and seven focal glial differentiation. These findings suggest a possible relationship between N-myc amplification and neuronal differentiation.

Brain Neoplasms↗
PubMed2000

Immunohistochemistry of small round-cell tumors.

The term "small round-cell tumor" describes a group of highly aggressive malignant tumors composed of relatively small and monotonous undifferentiated cells with high nuclear to cytoplasmic ratios. This group includes Ewing's sarcoma (ES), peripheral neuroepithelioma (aka, primitive neuroectodermal tumor or extraskeletal ES), peripheral neuroblastoma ("classic-type"), rhabdomyosarcoma, desmoplastic small round-cell tumor, lymphoma, leukemia, small-cell osteosarcoma, small-cell carcinoma (either undifferentiated or neuroendocrine), olfactory neuroblastoma, cutaneous neuroendocrine carcinoma (aka, Merkel-cell carcinoma), small-cell melanoma, and mesenchymal chondrosarcoma. Their clinical presentations often overlap, thus making a definitive diagnosis problematic in some cases. Yet, a clear understanding of their clinicopathologic features usually allows for a confident diagnosis, especially if immunohistochemistry is used. The following is a review of the immunohistochemistry of this small round-cell tumor group.

Biomarkers, Tumor↗
PubMedDate not supplied

Fine needle aspiration of peripheral neuroepithelioma of soft tissues.

Peripheral neuroepithelioma of soft tissue is a malignant primitive neuroectodermal tumor that appears both in children and adults and usually has a poor outcome. Fine needle aspiration on two patients with tumors in the lower limbs showed small round cells with unipolar processes and a tendency to form Homer-Wright rosettes. The cells had a round to oval nucleus with fine chromatin, up to four small, conspicuous nucleoli and vacuolated, periodic acid-Schiff-positive cytoplasm. The diagnosis was supported by electron microscopic study of the aspirate, which showed features of neuroblastic differentiation (i.e., neurosecretory granules), and by histologic, immunohistochemical and cytogenetic study of the resected tumors.

Adolescent↗
PubMed1994

Comparison of Ewing's sarcoma of bone and peripheral neuroepithelioma. An immunocytochemical and ultrastructural analysis of two primitive neuroectodermal neoplasms.

Ewing's sarcoma of bone (ESB) and peripheral neuroepithelioma (PN) are frequently considered to be different tumors. Some researchers have suggested that PN is morphologically a neuroectodermal Ewing's sarcoma. We sought to determine the extent of neuroectodermal features in conventional ESB on direct patient material (25 cases) and to compare these tumors with a similar group of readily diagnosed PNs (10 cases). Light microscopic, ultrastructural, and immunophenotypic parameters were assessed and compared for both groups. The avidin-biotin complex method was used. All tumors were antigenically intact since all stained for vimentin or at least one marker. Neuroectodermal antigens (neuron-specific enolase, Leu-7 [HNK-1], neurofilament 200 kd, and S100) were found in nine of 10 cases of PN and in 17 of 25 cases of ESB. In ESB, an atypical light microscopic appearance correlated with the presence of neuroectodermal features in most cases, but neuroectodermal phenotype was more frequent (68%) than morphological evidence of neuroectodermal differentiation (36%). These data support the concept that ESB and PN are both peripheral primitive neuroectodermal neoplasms, differing only in extent of neuroectodermal phenotype and morphological differentiation.

Adolescent↗
PubMed1994

Cloning of the entire FLI1 gene, disrupted by the Ewing's sarcoma translocation breakpoint on 11q24, in a yeast artificial chromosome.

FLI1 (Friend leukemia virus integration 1), a member of the Ets gene family, is disrupted on 11q24 by the Ewing's Sarcoma (ES) and Peripheral Neuroepithelioma (PNE) t(11;22)(q24;q12) translocation. ES and PNE are Primitive Neuroectodermal Tumors (PNETs) and the consistent translocation t(11;22)(q24;q12) can be used for differential diagnosis. In PNETs the 3' part of human FLI1 is translocated from 11q24 to 22q12, where it is juxtaposed to the 5' end of the Ewing's Sarcoma gene (EWS). A fusion transcript, resulting in a chimeric protein, is generated. Here, we present the isolation and detailed characterization of a 250-kb colinear YAC, B45C11, which encompasses the ES and PNE breakpoint on 11q24, as shown by FISSH on ES and PNE chromosomes and interphase nuclei. This YAC represents a new reagent for potential use in rapid differential diagnosis by FISSH on tumor biopsies and on paraffin embedded samples, particularly when DNA and/or RNA are not available for molecular analysis. YAC B45C11, which spans 250 kb of contiguous DNA around the ES and PNE breakpoint, contains the entire FLI1 gene. Three potential HpaII-tiny-fragment (HTF) islands are revealed within the YAC. One of these islands appears to be associated with the 5' end of FLI1, which extends over approximately 120 kb of DNA on 11q24. In addition, we demonstrate that YAC B45C11 contains other transcribed sequences in addition to FLI1, by "cross-species" Northern blot hybridizations, which suggests the presence of additional genes in the immediate vicinity of the ES breakpoint on 11q24.

Base Sequence↗