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 37 records · Page 2Linked to original sources
PubMed2001

Peripheral primitive neuroectodermal tumor within the spinal epidural space.

A case of an epidural spinal peripheral primitive neuroectodermal tumor (pPNET) in a 13-year-old girl is presented. The tumor was disseminated at the moment of diagnosis, thus only diagnostic oligobiopsy of the epidural mass was performed. Histologically the tumor was composed of small round blue cells. The neoplastic cells expressed MIC2 and features of neural differentiation on immunohistochemical staining (neuron-specific enolase, synaptophysin and NCAM positivity). Fluorescent in situ hybridization (FISH) analysis was performed for the final diagnosis and the translocation t(11;22)(q24;q12) was detected. The present case emphasizes the usefulness of FISH in differential diagnosis of tumors, especially when only routinely fixed material is available.

12E7 Antigen↗
PubMed2002

Peripheral primitive neuroectodermal tumor presenting in the retroperitoneum: a case report with immunohistochemical study.

It is now believed that malignant peripheral primitive neuroectodermal tumor (PNET) form a distinct tumor entity from other malignant small round cell tumors exhibiting neuroectodermal differentiation by morphologic, immunohistochemical or electron microscopic analyses. A 17-year-old Ethiopian boy was found to have a big upper extra osseous retroperitoneal tumor mass not associated with peripheral nerve that had infiltrated the body of the pancreas. Histologic sections from excised biopsy showed neoplastic cells with a high nuclear-cytoplasmic ratio and had an indistinct cytoplasm with numerous Homer-Wright rosettes. Immunohistochemically, isolated tumor cells and the centre of rosettes disclosed strong positivity to neural markers, synaptophysin and chromogranin. To the best of our knowledge, this case report represents the first patient described in Ethiopia.

Adolescent↗
PubMed1996

Peripheral primitive neuroectodermal tumors. CT and MRI evaluation.

The clinical, radiographic, and pathologic features of 17 patients with documented peripheral primitive neuroectodermal tumor (PNET) were evaluated in a retrospective study. The age at diagnosis ranged from 9 months to 46 years (median, 15.8 years). Primary sites of involvement were the abdomen (n = 8), extremities (n = 5), chest (n = 1), temporal bone (n = 1), maxilla (n = 1), and diploe (n = 1). At the time of diagnosis, six patients had distant metastases; all of these patients died, with an average survival of 8.8 months. Radiologic workup included standard radiographs, ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and bone scintigraphy. The radiographic appearance of these tumors was not specific for differentiation of PNETs from other types of bone and soft tissue tumors. The typical appearance resembled large non-calcified, soft tissue masses with cystic or necrotic areas. Heterogeneous enhancement with intravenous contrast agents was evident on CT, as was an intermediate signal intensity on Tl-weighted images and hyperintense signal on T2-weighted and STIR sequences. After gadolinium administration, variable enhancement was seen. MRI and CT were useful in predicting resectability, in detecting distant metastases, and in the evaluation of response to treatment. Surgery was performed in all cases, either for definitive diagnosis or for therapy. All patients received combined chemotherapy and radiotherapy and five patients received autologous bone marrow transplantation. Clinical follow-up was obtained over a mean period of 3 years (range 1 day to 6 years). Prognosis was poor with a median survival of 3.4 years. Our experience in 17 patients with peripheral neuroectodermal tumors indicates that although their radiologic features are non-specific, they should be included in differential diagnosis of soft tissue tumors of aggressive behavior, especially in a young age group. CT and MRI are useful in delineating the extent and resectability of tumor and in monotoring treatment.

Adolescent↗
PubMed1998

Peripheral primitive neuroectodermal tumor presenting with diffuse cutaneous involvement and 7;22 translocation.

We report an unusual case of peripheral primitive neuroectodermal tumor (pPNET) in an infant presenting with congenital cutaneous nodules and a t(7;22)(p21;q11.2). The biologic behavior of the tumor diverged over time from a slowly growing tumor with multiple cutaneous nodules to a more aggressive neoplasm characterized by pulmonary metastases and a soft tissue mass showing additional cytogenetic alterations.

Chromosomes, Human, Pair 22↗
PubMed2000

Peripheral primitive neuroectodermal tumor of the parotid gland region: report of a case with fine-needle aspiration findings.

A case of peripheral primitive neuroectodermal tumor of the parotid gland region in a 38-yr-old woman is reported. She had a 1-yr history of a large, firm, and slightly tender left parotid-region mass. CT scan showed an invasive tumor involving the parotid gland, mandible, infratemporal fossa, and parapharyngeal space. Fine-needle aspiration cytology of the mass showed a highly cellular, poorly cohesive smear pattern exhibiting small cuboidal cells, with fibrillary cytoplasm forming occasional rosette-like structures. Numerous intact single cells with fragile cytoplasm, finely granular chromatin, and inconspicuous nucleoli were present together with free-lying nuclei in the background. Histologic, immunohistochemical, and ultrastructural findings confirmed the diagnosis. Diagn. Cytopathol. 2000;22:161-166. Published 2000 Wiley-Liss, Inc.

Adult↗
PubMed1996

Peripheral neuroepithelioma (peripheral primitive neuroectodermal tumor) of the uterine cervix.

We report here a case of peripheral neuroepithelioma arising in the uterine cervix. A 44-year-old female had complained of irregular genital bleeding for several months and was diagnosed as extraosseous Ewing's sarcoma (EOE) from biopsy specimens initially. The tumor cells, which were intensely stained by periodic acid-Schiff (PAS) and digested with diastase, were uniformly small and round, had hyperchromatic nuclei and scant, indistinct cytoplasm. The surgically-removed tumor cells were immunohistochemically stained with antisera against neuro-specific enolase (NSE), tyrosine hydroxylase. In ultrastructural study, neurosecretory granules were observed. Finally we diagnosed this case as peripheral neuroepithelioma (peripheral primitive neuroectodermal tumor, PNET).

Adult↗
PubMedDate not supplied

Peripheral primitive neuroectodermal tumor (PPNET) of pelvic origin: report of a case arising from an unusual location.

We report a rare case of a peripheral primitive neuroectodermal tumor (PPNET) originating from the left ileopsoas muscle in an adult patient with neoplastic thrombosis of the left external iliac vein, the common femoral vein and the left popliteal vein. We performed a median laparotomy with an oblique left inguinal incision to remove the neoplasm, which consisted of a large mass surrounding the iliac-psoas muscles, extending from the transverse apophysis of the spinal column to Scarpa's triangle, and passing through the lacuna musculorum. Histopathological examination revealed a primitive neuroectodermal tumor (PNET) with focal areas of necrosis, hemorrhage and vascular invasion. Immunophenotyping was positive for CD99, NSE, and focally for CK. Ultrastructural examination of the neoplastic cells showed often multiple nuclei with dense chromatin and very large nucleoli. The patient was discharged ten days after the operation. Adjuvant treatment consisted of radiotherapy at a dose of 2000 cGy in five fractions followed by six cycles of chemotherapy. The venous thrombosis was treated by anticoagulant therapy and recanalization of the occluded veins was obtained after two months of therapy. An MRI scan, carried out 12 months later, showed a local relapse, which was treated with chemotherapy and arterial chemoembolization.

Aged↗
PubMed1998

[Clinico-pathologic study of Ewing's sarcomas of bone and soft tissue and peripheral primitive neuroectodermal tumors].

OBJECTIVE: To study the diagnosis and differentiation of EW and PNET. METHODS: Fourteen cases of Ewing's sarcomas (EW) and peripheral primitive neuroectodermal tumors (PNET) were studied by light microscopy, immunohistochemistry and electron microscopy (EM). Twelve cases were followed up. Schmidt criterion was used for the differential diagnosis of EW and PNET. RESULTS: There were 6 cases of EW and 8 cases of PNET. Six of 8 PNET cases had Homer-Wright (H-W) rosettes. In this series, 12/14 cases were positive for O13 (HBA71) staining. NSE was positive in 3 cases of EW. All PNET cases were positive for neural markers, and 5 of them were positive for more than two of these markers. Electronmicroscopically, there were neurosecretory granules (4/4 cases), nerve-like protrusions and microfilaments (1/4 case) in PNET. In 3 of 6 EW and 1 of 6 PNET, PAS staining was positive. During the follow-up period from 2 months to 5 years, 3 cases with intraosseous EW remain alive and free of tumor. The remaining patients are dead or having their tumors metastasized. CONCLUSION: EW is more primitive in cell differentiation, while PNET has more neural differentiation. The presence of H-W rosettes is an important morphologic feature of PNET. To differentiate EW from PNET is of clinical significance O13 is a useful marker for the diagnosis of EW/PNET.

Adolescent↗
PubMed1994

Peripheral primitive neuroectodermal tumor of the vulva.

A 10-year-old Hispanic female presented with a 3-month history of a vulvar mass. The histologic diagnosis was a peripheral primitive neuroectodermal tumor (PNET). To our knowledge, this is the second reported case of vulvar peripheral PNET and the first such case reported in the English language.

Biopsy↗
PubMed1994

Immunohistochemical detection of P-glycoprotein in Ewing's sarcoma and peripheral primitive neuroectodermal tumors before and after chemotherapy.

The authors evaluated P-glycoprotein expression in a total of 35 cases of Ewing's sarcoma and peripheral primitive neuroectodermal tumors (PNET). Fifteen cases had matched tumors before and after treatment. The 20 unmatched tumors included 14 pretreatment PNETs and Ewing's sarcomas and 6 posttreatment Ewing's sarcomas. Two antibodies, C219 and JSB-1, were used. Immunoreactivity was almost exclusively membranous. Variability in the number of positive cells and in staining intensity was noted within individual tumors. Among the 15 matched tumors, 7 were positive for P-glycoprotein before treatment; 6 of these remained positive after treatment. Four of the 8 that were negative for P-glycoprotein before treatment became positive after treatment. Of the unmatched tumors, 9 of 14 pretreatment and 3 of 6 posttreatment tumors were positive. When relapse-free survival time, based on the presence or absence of P-glycoprotein positivity in pretreatment tumor samples, was evaluated in this group, no significant difference was found (P2 = .92); however, the numbers are too small to draw definitive conclusions. The high incidence of positive primary tumors suggests that P-glycoprotein expression is probably intrinsic in Ewing's sarcoma and PNET and not necessarily induced by therapy.

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed2001

Peripheral primitive neuroectodermal tumor with neuronal and glial differentiation: report of a case arising in suprarenal region.

It is well known that embryonal neuroectodermal tumors of the central nervous system (CNS) not infrequently display varying amount of neoplastic cells acquiring glial differentiation. In contrast, glial differentiation rarely occurs in primitive neuroectodermal tumors outside the CNS being documented in less than ten cases. The author presents herein a case of peripheral primitive neuroectodermal tumor with prominent glial differentiation identified by the presence of glial fibrillary acidic protein (GFAP) arising in the right suprarenal region of a 32-year-old man, histologically indistinguishable from an ordinary neuroblastoma.

Adult↗
PubMed2003

Ewing's sarcoma and peripheral primitive neuroectodermal tumor cells produce large quantities of bioactive tumor necrosis factor-alpha (TNF-alpha) after radiation exposure.

PURPOSE: In the present study, we examined human Ewing's sarcoma (ES) and peripheral primitive neuroectodermal tumor (pPNET) cell lines that are able to produce TNF-alpha as part of the response to irradiation. Radiation-induced tumor cell production of TNF-alpha may enhance irradiation efficacy and improve the effect of local tumor irradiation. On the other hand, radiation-induced tumor cell production of TNF-alpha may adversely affect the normal tissue. METHODS AND MATERIALS: Twelve different ES/pPNET cell lines were investigated in vitro and (after establishment as tumor xenografts in athymic nude mice) in vivo for their TNF-alpha mRNA expression (real-time quantitative reverse transcriptase polymerase chain reaction) and TNF-alpha protein production (in vitro: enhanced amplified sensitivity immunoassay; in vivo: immunohistochemistry) after exposure to different irradiation doses (2, 5, 10, 20, 30, or 40 Gy) and after different time intervals (1, 3, 6, 12, 24, 48, or 72 h after irradiation). The bioactivity of the TNF-alpha protein was evaluated in chromogenic cytotoxicity and neutralization assays. RESULTS: Nine out of 12 ES/pPNET cell lines express constitutively significant quantities of bioactive TNF-alpha in vitro. ES/pPNET cells originating from primary tumors secreted higher TNF-alpha levels than cells derived from metastatic lesions. In 5 of the 9 TNF-alpha-producing cell lines, TNF-alpha mRNA and protein levels were upregulated after irradiation exposure in a time- and dose-dependent manner. After establishment of the ES/pPNET cell lines in athymic nude mice, the radiation-induced TNF-alpha release could be demonstrated also in the xenograft tumors in vivo (analogous to the in vitro experiments). Using the same methods for quantitative analysis, it was determined that the TNF-alpha expression of the radiation-responsive tumor cells was up to 2000-fold higher compared to the maximal radiation-induced TNF-alpha release in normal lung tissue measured during the pneumonic phase. CONCLUSION: Certain ES/pPNET cell lines produce extremely large quantities of bioactive TNF-alpha after radiation exposure in a time- and dose-dependent manner. Radiation-induced TNF-alpha production of tumor cells may be of paramount importance in respect to not only tumor behavior, but also to potential damage to normal tissue and the clinical status of the host.

Animals↗
PubMed1997

Detection of circulating tumor cells in patients with Ewing's sarcoma and peripheral primitive neuroectodermal tumor.

PURPOSE: To determine the feasibility of detecting Ewing's sarcoma (ES) or peripheral primitive neuroectodermal tumor (PNET) through a reverse-transcriptase polymerase chain reaction (RT-PCR) of the t(11;22)(q24;q12) fusion transcript in blood and bone marrow samples from patients with these neoplasms. PATIENTS AND METHODS: Peripheral-blood (PB) and/or bone marrow aspirate (BM) samples were obtained from 28 patients with ES or PNET at initial presentation or at relapse. Patients were divided into two groups: newly diagnosed patients with nonmetastatic disease and those with metastatic/relapsed disease. RNA was extracted from fractionated BM and PB samples, and RT-PCR was performed for the EWS/HumFLI1 fusion mRNA was transcribed across the t(11;22) breakpoint. RESULTS: Among the 16 patients with nonmetastatic disease, three of 16 were RT-PCR positive for EWS/HumFLI1 RNA in BM and three of 10 were positive in PB. The total number of nonmetastatic patients who were positive in either PB or BM was four of 16 (25%). Among patients with metastatic/relapsed disease, two of six were positive in BM and five of 10 were positive in PB. The total fraction of patients with metastatic/relapsed disease that was positive in either BM or PB was six of 12 (50%). CONCLUSION: In this study, we show that it is possible to amplify the EWS/HumFLI1 RNA by RT-PCR from the BM and PB of a subset of patients with both nonmetastatic and metastatic ES or PNET, which implies that occult tumor cells are present at these sites. The true biologic and clinical meaning of this information is unknown. However, it does suggest a possible application of RT-PCR for the monitoring of residual disease in patients who are undergoing therapy for ES or PNET. This approach may permit early identification of patients who may benefit from alternative therapy or who may be spared possible overtreatment.

Chromosomes, Human, Pair 11↗
PubMed1996

Is the EWS/FLI-1 fusion transcript specific for Ewing sarcoma and peripheral primitive neuroectodermal tumor? A report of four cases showing this transcript in a wider range of tumor types.

The presence of t(11;22)(q24;q12) is often considered diagnostic of Ewing sarcoma and peripheral primitive neuroectodermal tumor. We report four cases, all of which possessed this translocation as detected by reverse transcriptase polymerase chain reaction and confirmed by sequencing with or without fluorescent in situ hybridization, but none of which were Ewing sarcoma or peripheral primitive neuroectodermal tumor by histological criteria. Two were polyphenotypic tumors and two were mixed embryonal and alveolar rhabdomyosarcomas. Only one case was positive for MIC2 by immunohistochemistry and only in a rare cell. Two cases (one polyphenotypic tumor and one rhabdomyosarcoma) had double minute chromosomes with > 100 copies of the MDM2 gene. The presence of the t(11;22)(q24;ql2) translocation should probably not be considered diagnostic of Ewing sarcoma and peripheral primitive neuroectodermal tumor in the absence of supporting histological evidence. The presence of this translocation in Ewing sarcoma and peripheral primitive neuroectodermal tumor has been taken as evidence that these two tumors are related. Extending this relationship to include some polyphenotypic tumors and some rhabdomyosarcomas may not be justified unless additional evidence is gathered. Pathologists and oncologists will need to decide whether treatment regimens for tumors are better based on phenotype rather than genotype when these two profiles are seemingly in conflict.

Abdominal Neoplasms↗
PubMed1992

Peripheral primitive neuroectodermal tumors. Diagnosis, classification, and prognosis.

In conclusion, the group of peripheral primitive neuroectodermal tumors has been redefined in recent years on the basis of cytogenetic, molecular genetic and more precisely defined histopathologic characteristics. Although in the past, many tumors has been called Ewing's sarcoma, currently this diagnosis is limited to tumors which cannot be more specifically classified on the basis of their ultrastructural and immunophenotypic characteristics. Most small round cell tumors previously classified as Ewing's sarcoma are now classified as peripheral PNET. The consistent cytogenetic abnormality in Ewing's sarcoma and peripheral PNET and patterns of neurotransmitter enzymes have supported a common neuroectodermal origin. The precise characterization of soft tissue Ewing's sarcoma is further complicated by the several primitive rhabdomyosarcomas that may exhibit a similar light microscopic appearance. The importance of histopathologic distinction among these various round cell tumors of childhood is well recognized. Furthermore, primitive tumors with overlapping neural and mesenchymal features, known as malignant ectomesenchymoma, are now identified more often than previously. Finally, molecular biologic and cytogenetic differences between peripheral PNET and neuroblastoma have confirmed their clinical and biologic differences, in spite of their morphologic similarities. Molecular genetic and flow cytometric evaluation have contributed to the distinction of groups with prognostic significance and offer possibilities for new clinical trials.

Adolescent↗
PubMed2003

Malignant peripheral primitive neuroectodermal tumor-peripheral neuroepithelioma of the head and neck: a clinicopathologic study of five cases and review of the literature.

BACKGROUND: The term primitive neuroectodermal tumor (PNET) encompasses a number of neoplasms of common neuroectodermal origin, but of variable clinical, histopathologic, ultrastructural, and molecular characteristics. Here, we focus on one particular member of the PNET family, the malignant peripheral PNET (pPNET) or peripheral neuroepithelioma of head and neck. METHODS: Five patients diagnosed with malignant pPNET-peripheral neuroepithelioma of head and neck were included in the study. All existing literature was reviewed. RESULTS: The diagnosis of malignant pPNET-peripheral neuroepithelioma of the head and neck was confirmed in all five cases. All patients are alive after treatment with a combination of therapeutic modalities; only one patient had metastatic disease develop. A literature review revealed another 38 reported cases. CONCLUSIONS: Malignant pPNET-peripheral neuroepithelioma should be included in the differential diagnosis of small, round, blue cell tumors of head and neck. A combination of multiple diagnostic modalities is essential for correct diagnosis, and multimodality treatment offers the best outcome.

12E7 Antigen↗
PubMed2003

Peripheral primitive neuroectodermal tumor after radiotherapy.

A 41-year-old man had a peripheral neuroectodermal tumor develop at the distal third of the fibula 4 years after radiotherapy for relapsed villonodular synovitis. This type of sarcoma usually is classified into the heterogeneic group of small round-cell bone tumors as a subdivision of Ewing's sarcomas. The immuno-staining positivity of the neoplastic cells for the neuron-specific enolase allowed the authors to make the diagnosis of a tumor with neuroectodermal origin. When the histologic study confirmed the diagnosis, the patient was treated with chemotherapy, surgical excision of the tumor, and adjuvant radiotherapy. Radiotherapy is thought to be involved in the genesis of osteogenic sarcomas as it has been shown in several reports, but there is no evidence in the literature of a peripheral neuroectodermal tumor developing after radiotherapy.

Adult↗
PubMed1992

Peripheral primitive neuroectodermal tumors. A flow cytometric analysis with immunohistochemical and ultrastructural observations.

Flow cytometry of classical neuroblastoma has provided provocative evidence that cell cycle and ploidy analysis generate prognostically useful information. To determine whether such analyses of peripheral primitive neuroectodermal tumors might yield similar results, formalin-fixed, paraffin-embedded tissue specimens from 19 peripheral primitive neuroectodermal tumors, each previously characterized by immunohistochemical or ultrastructural study, were assessed. An acceptable histogram was obtained in 16 cases. Of these, nine neoplasms were diploid and seven contained aneuploid DNA. Among patients with diploid lesions, four were free of disease, whereas three had persistent or recurrent disease, and two had died of tumor. Among patients with aneuploid neoplasms, four were free of disease, one had recurrence, and two had died. There was no apparent correlation between immunophenotype and proliferative activity with the clinical outcome. Among aneuploid peripheral primitive neuroectodermal tumors, DNA index did not predict survival. Hence, cell cycle and DNA ploidy analyses do not appear to contribute to the prognostic assessment of peripheral primitive neuroectodermal tumors, as they do to presumably related neoplasms of the central and peripheral nervous system.

Aneuploidy↗