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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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PubMed1990

Ultrastructural, immunohistochemical, and cytogenetic study of a malignant peripheral neuroectodermal tumor in a patient seropositive for human immunodeficiency virus.

A case of malignant peripheral neuroectodermal tumor occurring during the course of a human immunodeficiency virus (HIV) infection is reported. The patient was a male homosexual who presented with a rapidly enlarging tumor of the posterior lower thoracic wall. By light microscopic examination the tumor was a small cell tumor showing occasional structures suggestive of Homer-Wright rosettes. The strong positivity for neuron-specific enolase and the neurosecretory granules indicated the neural differentiation of the tumor. Its precise nature was shown cytogenetically by the presence of the t(11;22) translocation, which distinguished it from the classical neuroblastoma.

Adult↗

PubMed1993

dbl oncogene expression in childhood tumors and tumor cell lines.

We studied the expression of the dbl oncogene in the total RNA obtained from a wide spectrum of childhood tumors, including Ewing's sarcomas, peripheral neuroectodermal tumors (PNET), esthesioneuroblastomas, neuroblastomas, retinoblastomas, rhabdomyosarcomas, osteosarcomas, and synovial sarcomas. Material was obtained from primary tumors, nude mice xenografts, and tumor cell lines. Following the Northern blot technique, a single band of 2.8 kb was found in each analyzed case. Induction of neural differentiation in Ewing's sarcoma, peripheral PNET, and neuroblastoma cell lines with dibutyryl cyclic AMP did not change the expression of the dbl oncogene. We conclude that the wide expression of the dbl oncogene in these childhood tumors reduces its value as a molecular marker for their differential diagnosis; on the other hand, the dbl oncogene does not appear to be an essential molecular factor in the process of neuroectodermal differentiation of small round cell tumors of childhood.

Animals↗

PubMed1992

A novel POU homeodomain gene specifically expressed in cells of the developing mammalian nervous system.

We report the isolation of a novel human POU domain encoding gene named RDC-1. The POU domain of the RDC-1 encoded protein is highly related to the POU domain potentially encoded by the rat brain-3 sequence and to that of the Drosophila I-POU protein; outside of the POU region, RDC-1 is unrelated to any previously characterized protein. The RDC-1 gene is expressed almost exclusively in normal tissues and transformed cells of neural origin. In the developing mouse and human fetus, RDC-1 is expressed in a spatially and temporally restricted pattern that suggests a critical role in the differentiation of neuronal tissues. In addition, RDC-1 is expressed in a unique subset of tumors of the peripheral nervous system including neuroepitheliomas and Ewing's sarcomas but not neuroblastomas. Based on its unique structural characteristics and expression pattern, we discuss potential functions for the RDC-1 protein.

Amino Acid Sequence↗

PubMed1992

Unicolor and bicolor in situ hybridization in the diagnosis of peripheral neuroepithelioma and related tumors.

The chromosome 22 breakpoint of the t(11;22) translocation of peripheral neuroepithelioma has been located, by fluorescence in situ hybridization, in the proximity of the interface between 22q12.1 and 22q12.2. Use of single cosmids or pools of cosmids of the flanking regions enables the monitoring of the translocation in interphase and in metaphase chromosomes. This method can now be routinely applied for the diagnosis of this translocation in mixed round cell tumors.

Bone Neoplasms↗

PubMed1995

Is esthesioneuroblastoma a peripheral neuroectodermal tumor?

Esthesioneuroblastoma (ENB; olfactory neuroblastoma) is a rare, locally aggressive neoplasm of the sinonasal area and anterior cranial fossa. The histogenesis of the lesion is not clearly delineated, and the broad histological spectrum of ENB has confounded the issue. The location, histological features (neuropil, Homer Wright, and olfactory rosettes), and reported immunocytochemical reactions (neuron-specific enolase (NSE) and chromogranin (CHR) positivity) suggest that ENB may be a neural or neuroendocrine neoplasm derived from the olfactory membrane. Recent demonstration in two of three metastatic putative ENB cell lines of the 11;22 chromosomal translocation, seen in Ewing's sarcoma (ES) of bone and peripheral neuroectodermal tumors (PNET) of bone and soft tissue, has led to the conclusion that ENB may be closely related histogenetically to PNET. The overwhelming majority of cases of ES and PNET express the protein product of MIC-2, a gene located on the pseudoautosomal region of the X and Y chromosomes. This protein can be identified immunocytochemically by antibodies 12E7, HBA71, and ON13. We studied the expression of MIC-2 using the 12E7 antibody as well as multiple neural markers in 18 ENB samples obtained from the files of The Johns Hopkins Hospital. The patients ranged in age from 19 to 90 years (mean, 47.5; median, 47) and included five men and 13 women. None of the 18 specimens reacted with antibody 12E7, but 16 were positive for NSE, nine reacted to synaptophysin (SYN), and 13 showed antibodies to chromogranin (CHR). Our studies agree with the previous suggestions that ENB is a primitive neural tumor but fail to support the hypothesis that it is a member of the PNET family.

12E7 Antigen↗

PubMed1998

Peripheral primitive neuroectodermal tumour of the lung: report of two cases.

AIMS: We report two unusual cases of intrapulmonary small round cell tumour with evidence that they are primitive neuroectodermal tumours. METHODS AND RESULTS: The patients were a 25-year-old woman and a 15-year-old man, in whom mass shadows were found by routine chest radiography. Both tumours were located in the pulmonary parenchyma, and there was no evidence of an extrapulmonary involvement by the tumour. The lesions showed morphological and immunophenotypic features consistent with peripheral primitive neuroectodermal tumour (pPNET). These features included a hypercellular diffuse growth pattern of closely packed small round cells occasionally forming abortive Homer-Wright-type rosettes and intense immuroreactivities to neurone-specific enolase and MIC2 gene product (O13). Unusual chondroid foci were present in one of the cases. This diagnosis was further supported by the cytogenetic and reverse transcriptase-polymerase chain reaction (RT-PCR) findings of the characteristic t(11;22) chromosomal translocation and EWS/FLI-1 fusion transcripts, respectively, in one of the cases. CONCLUSIONS: These morphological and cytogenetic findings substantiate pPNET as a subtype of intrapulmonary small round cell tumours.

Adolescent↗

PubMed1993

[Pathology and clinical aspects of peripheral neuroectodermal tumors].

A 36-year-old man developed progressive pain in the left thorax radiating to the shoulder, associated with weight loss as well as axillary and supraclavicular lymph-node swellings, and a left Horner's syndrome. The radiological diagnosis was Pancoast tumour. Computed tomography of the thorax revealed that the tumour had almost completely infiltrated the left lung and hilus. Sonography demonstrated metastases in the liver and retroperitoneum. Histological examination of an excised axillary lymph-node metastasis showed an undifferentiated small-cell tumour. Immunohistochemical tests revealed expression of protein S 100 and neurofilamental proteins, i.e. evidence of a peripheral malignant neuroectodermal tumour. The tumour also expressed vimentin. Treatment consisted of two chemotherapy cycles according to the EVAIA scheme (daily three times 235 mg etoposide, 2 mg vincristine, three times 0.8 mg actinomycin D, three times 3.2 g ifosfamide, three times 30 mg doxorubicin, and three times 12 mg dexamethasone), without any effect on the rapid progression of the disease. He died 17 weeks after the diagnosis had been made.

Adult↗

PubMed1999

Esthesioneuroblastoma is not a member of the primitive peripheral neuroectodermal tumour-Ewing's group.

Esthesioneuroblastoma (ENB) is a rare, site-specific, locally aggressive neuronal malignancy so far thought to belong to primitive peripheral neuroectodermal tumour-Ewing's tumour (pPNETs-ETs). Its anatomical location, in addition to morphologic, immunophenotypic and ultrastructural features, suggests its origin in the neuronal or neuroendocrine cells of the olfactory epithelium. However, the cytogenetic and molecular data currently available appear controversial on the presence of the typical translocation t(11;22)(q24;q12) and of trisomy 8, chromosomal changes that characterize the tumours belonging to the pPNETs-ETs. Herein we have analysed five ENB tumour specimens for trisomy 8 by fluorescence in situ hybridization (FISH), for the presence of EWS gene rearrangements by FISH, reverse transcription polymerase chain reaction and Southern blot analyses, as well as for the expression of the Ewing sarcoma-associated MIC2 antigen by immunohistochemistry. Neither EWS/FLI-I, EWS/ERG and EWS/FEV fusion genes nor MIC2 expression were found in any tumour, whereas trisomy 8 was found in one case only. Moreover, DNA from three cases analysed by Southern blot did not show EWS gene rearrangements. Our results support the evidence that ENB is not a member of the pPNETs-ETs.

Adult↗

PubMed1993

[The differential diagnosis of neuroepithelial tumors and meningiomas of the brain by using magnetic resonance tomography (field strength of 0.04 T)].

A study was made of the magnetic resonance imaging (MRI) data on 7 patients with primary neuroepithelial tumors located in the peripheral parts of the hemispheres and 9 patients with convex meningiomas. Tumors were verified by histological examinations in all the cases. MRI was performed on an apparatus Magnaview (Instrumentarium Company, Finland) in different impulse sequences and planes. The images were estimated quantitatively according to the tumor-to-white matter ratio and signal-to-noise ratio. It has been shown that the use of the complex of the macromorphological signs combined with the parameters under consideration enables one to differentiate between neuroepithelial tumors and meningiomas of the brain practically with-out error.

Adult↗

PubMed1997

Peripheral primitive neuroectodermal tumour with ganglioneuroma-like areas arising in the cauda equina.

Peripheral primitive neuroectodermal tumour (pPNET or peripheral neuroepithelioma) is one of the malignant small round cell tumours of peripheral nerves, soft tissues and bones, but rarely originates in the spinal canal. We report an example of pPNET arising in the cauda equina of a 14-year-old Japanese boy. At surgery, a well-demarcated tumour measuring 2 x 4 cm in diameter and involving one of the nerve roots of the cauda equina was located within the intradural space with no evidence of extradural extension. Microscopically the tumour was made up of sheets of closely packed small round cells, associated with ganglioneuroma-like islands. Immunohistochemically, the small round tumour cells were intensely positive for neuron-specific enolase (NSE), an MIC2 gene product (O13) and beta 2-microglobulin, whereas the foci with ganglion cell-like cells reacted positively to NSE, synaptophysin and beta 2-microglobulin but were negative for O13. A chimeric transcript of the EWS/FLI-1 fusion gene detected by a nested reverse transcriptase-polymerase chain reaction using formalin-fixed paraffin-embedded tissue justified the diagnosis of pPNET. Only 6 cases of PNET in the cauda equina have been described in the literature, and this is the first case of a pPNET with ganglio-neuroma-like areas. This finding suggests that the primitive tumour cells of pPNET may respond to unknown inductive effects and express a ganglion cell-like morphology.

Adolescent↗

PubMed1998

Diagnostic utility of MIC-2 immunocytochemical staining in the differential diagnosis of small blue cell tumors.

Ewing's sarcoma (ES) and peripheral neuroectodermal tumor (PNET) are considered in the differential diagnosis of small round blue cell tumors of infancy and childhood which includes neuroblastoma, rhabdomyosarcoma and malignant lymphoma. Fine-needle aspiration diagnosis of these neoplasms can be particularly difficult when the neoplasms are composed of poorly differentiated cells or fail to produce a stroma. MIC-2 is a highly sensitive and specific marker for the PNET/ES group of neoplasms and has been studied extensively in surgical pathology. Other small blue cell neoplasms including rhabdomyosarcoma, blastemal Wilm's tumor, and lymphoblastic lymphoma have also shown positivity, but the staining reactions are usually weak and focal. The utility of this marker in the differential of small blue cell neoplasms in cytologic material has not been examined. Twenty cases of small blue cell neoplasms obtained by fine-needle aspiration (FNA) were studied. MIC-2 antibody was applied retrospectively to formalin-fixed cell block material and destained alcohol-fixed and air-dried cytologic preparations. These cases include primitive neuroectodermal tumor (five cases), Ewing's sarcoma (two cases), neuroblastoma (four cases), Wilms's tumor (four cases), lymphoblastic lymphoma (two cases), and small-cell carcinoma (three cases). The cases were judged positive when the majority of the cells showed cytoplasmic staining. Diffuse cytoplasmic staining was observed in all seven cases of PNET/ES. Staining could be seen on the destained air-dried smears (three cases), fixed smears (two cases), or the cell block material (two cases). None of the other 13 small blue cell neoplasms showed positive staining. We conclude that MIC-2 is a sensitive and specific marker for the PNET/ES group of neoplasms in specimens from formalin-fixed cell block, air-dried, and alcohol-fixed cytologic material and is useful in the differential diagnosis of small blue cell tumors.

12E7 Antigen↗

PubMed1995

Acquired von Willebrand disease in malignant peripheral neuroectodermal tumor (PNET).

In week 12 of the EICESS 92 protocol a 12-year-old boy with pelvic PNET developed acquired von Willebrand disease: bleeding time was prolonged (> 15 min) and von Willebrand factor antigen (54%) and ristocetin cofactor activity (50) were reduced. Platelet aggregations with thrombin and collagen and the number of major platelet glycoproteins/per platelet did not differ from the controls. After Haemate P (Behring Werke, Marburg, Germany) bleeding time normalized and surgery could be performed without occurrence of bleeding episodes. Three weeks after surgery bleeding time, ristocetin cofactor activity, and von Willebrand factor antigen increased to normal paediatric values. Without occurrence of further bleeding episodes the patient received another 10 courses of polychemotherapy (EICESS 92).

Bone Neoplasms↗

PubMedDate not supplied

[Peripheral neuroepithelioma].

Neuroepithelioma of the soft tissues of the right parietotemporal region in the 25-year-old male patient is described. The tumor grew through the cranial bones into the dura mater. The patient died 1.5 months after the appearance of the first clinical signs of the disease of brain edema and invasion of the cerebellum into the great foramen occipitalis with compression of the medulla oblongata. The histological structure of the tumor and expression of neuron-specific enolase, synaptophysin, S-100 protein and glial fibrillary acidic protein by tumor cells prove its neurogenic origin.

Adult↗

PubMed1995

Peripheral primitive neuroectodermal tumour after allogeneic bone marrow transplantation.

We report a case of peripheral primitive neuroectodermal tumour (PNET) in a 36-year-old man who 10 years earlier received an allogeneic bone marrow transplantation (BMT) for a non-Hodgkin's lymphoma. Immunohistochemistry proved definitive elimination of a relapse of the original disease and confirmed the diagnosis of PNET. The role of total body irradiation pre-BMT in the genesis of this secondary tumour is discussed. The importance of registering secondary malignant solid tumours after BMT is emphasized. To our knowledge, an Ewing family tumour following BMT has not previously been reported.

Adult↗

PubMed1998

[Peripheral neuroectodermal tumors of the chest wall. Description of a case].

The authors discuss a case of malignant small round cell tumor of the thoracopulmonary region (Askin tumor) in which the initial pathological diagnosis was "sarcoma". Only a review of the histological specimens and the clinical features and evolution revealed the correct diagnosis of "Askin tumor". A 15 year old girl with fever, dyspnoea, cough underwent thoracentesis, which revealed hemothorax. At surgery, a large mass not involving the lung, with pleural thickening, was found in the left hemithorax and excised, without rib resection. No metastasis was observed at that time. Histological findings revealed features of malignant small round cell tumors, with occasional pseudo-rosettes. Chemotherapy was performed. 16 months after clinical presentation the girl died with abdomino-thoracic metastatic disease.

Adolescent↗

PubMed1999

Gangliosides and neutral glycolipids in ependymal, neuronal and primitive neuroectodermal tumors.

Neutral glycolipid and ganglioside compositions were determined on 11 ependymal tumors, 12 medulloblastomas, 6 other neuronal tumors of the brain, 4 peripheral neuroblastomas, 1 cerebral primitive neuroectodermal tumor (PNET), and 1 PNET of the thoracic wall. Within the group of tumors that can demonstrate neuronal phenotypes, there was an association between the degree of neuronal differentiation usually demonstrated by these tumors and the proportions of both GD1a and 1b-pathway gangliosides. The amount of globoside also correlated with the amount of 1b pathway gangliosides. Patients with medulloblastomas whose 1b gangliosides made up over 15% of the total gangliosides survived longer that those with lower proportions of 1b gangliosides. The only gangliosides in the choroid plexus papilloma were GM3 and GD1a, but other ependymal tumors had significant amounts of GD1b and its metabolic precursors. Ependymoma and anaplastic ependymoma had similar neutral glycolipid compositions, which were different from subependymoma, which lacked ceramide monohexoside and ceramide dihexoside. These differences in glycolipid compositions suggest that there may be fundamental biological differences between these types of ependymal tumors.

Adult↗

PubMed1995

[CT and MRI in malignant peripheral neuroectodermal tumors].

To evaluate the diagnostic accuracy of CT and MRI in the diagnosis of malignant peripheral neuroectodermal tumors (MPNT), the imaging data of nine patients with immunehistochemically proven MPNT were retrospectively analyzed. The average age was 24 years (ranging from 5 to 63 years). MRI and CT of all patients showed large tumors with direct infiltration of the surrounding structures. Intratumoral calcifications were not identified. All tumors showed equal to less high signal intensity in T1- and high signal intensity in T2-weighted images. Because of its high soft-tissue contrast, MRI is the most capable imaging modality in the diagnosis and therapy-monitoring in these tumors.

Adolescent↗

PubMed1998

Molecular features in a biphenotypic small cell sarcoma with neuroectodermal and muscle differentiation.

We report a case of a 13-year-old girl with soft tissue sarcoma of the hand, which showed muscle and neuroectodermal immunophenotypes. Molecular studies were performed on RNA collected from fine-needle aspiration (FNA) cytology and peripheral blood samples by nested reverse transcriptase-polymerase chain reaction (RT-PCR) and Southern blot analysis. This biphenotypic tumor showed simultaneous expression of EWS-FLI1 and PAX3-FKHR transcripts, specific of Ewing family tumors and alveolar rhabdomyosarcoma, respectively. Although childhood sarcomas with simultaneous muscle and neural differentiation have been described to have EWS-FLI1 transcripts, there are no reports of tumors with both transcripts. Cytological specimens are a good source of RNA for molecular studies.

Adolescent↗