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Results for “Neuroectodermal Tumors, Primitive”

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PubMed1995

Growth-inhibiting effect of intratumoral recombinant human tumor necrosis factor on an experimental model of primitive neuroectodermal tumor.

The effect of intratumoral administration of recombinant human tumor necrosis factor on an experimental model of primitive neuroectodermal neoplasia was studied. A clear inhibition of tumor growth was achieved by immunotherapy that consisted in intralesion injections of 100 micrograms of tumor necrosis factor daily, the first three days of each week, for a period of four weeks. At this time, tumor size was 2.21 +/- 0.66 cm2 (mean +/- standard deviation) in the treated group, versus 7.62 +/- 0.43 cm2 in the control group. These data support previous studies on the influence of tumor necrosis factor on the development of ethyl-nitrosourea-induced tumors, and suggest the potential usefulness of this cytokine in human primitive neuroectodermal neoplasms.

Animals↗
PubMed1994

Experimental production of primitive neuroectodermal tumors and its relevance to human neuro-oncology.

The origin and nature of central nervous system tumors primarily composed of primitive neuroepithelial cells have intrigued neuropathologists since 1925, when the prototype tumor in this group, the medulloblastoma, was defined as a specific type by Bailey and Cushing. Theories regarding this group of tumors have been widely divergent, ranging from classification as sarcomas, neuroblastomas, spongioblastomas, ie, poorly differentiated gliomas (including astrocytic, oligodendroglial, and ependymal), to primitive tumors with a capacity for multidirectional differentiation. Studies of both human and experimental primitive neuroectodermal tumors have yielded considerable information about biological features, although controversies remain regarding interpretation of the findings. Moreover, many investigators remain fixed upon the notion that discovery of the "cell of origin" of these tumors will somehow enhance understanding of their nature. Rationale for relevance of this view is examined against the current state of knowledge regarding carcinogenesis as a whole and studies of human and experimental primitive neuroectodermal tumors in particular, and it is concluded that the time has come to discard the archaic concept of cell of origin and devote resources to investigations of the phenotypic character of the tumor and its relationship to prognosis and treatment.

Animals↗
PubMed1998

[Primitive neuroectodermal tumors: difficult tumors versus modern oncology].

INTRODUCTION: Primitive peripheral neuroectodermal tumours (PNET) are rare masses and form part of the group of round small cell tumours which include a wide range of highly aggressive neoplasias such as Ewing's sarcoma, neuroblastoma, lymphoma and rhabdomyosarcoma. PNET present the same cell line as the tumours presented by F. Askin in 1979, both located in the thoracic-pulmonary region. MATERIAL AND METHODS: Of the last 26 thoracic neuroblastomas and 11 mediastinal-thoracic sarcomas treated at our centre, we observed 5 PNET in children with a mean age of 12 years (range: 9-14 years). These patients presented a thoracic mass infiltrating sternum, clavicle, supraspinal muscle or, in two cases, a left lateral or paravertebral intrathoracic mass. The time elapsed between clinical observation and diagnosis was 6 weeks. Diagnosis was established by chest X-Ray, CT, bone scintigraphy, immunocytochemistry and cytology. Aggressive local treatment associated with stage IV SIOP chemotherapy for rhabdomyosarcoma was applied in all cases to prevent metastasis. RESULTS: Of the five PNET treated, one 16-year-old patient died (4 y 5 m post-diagnosis) from bone marrow infiltration which had evolved badly from the beginning. The remaining patients are disease-free. One patient who did not undergo surgery relapsed 1 year and the half after completing chemotherapy. He then underwent resection of the cranial portion of the sternum and substitution with iliac graft from the tissue bank. CONCLUSION: PNET manifest clearly some of the characteristics of current paediatric oncology. These tumours are easily misdiagnosed and at present may be differentiated by new diagnostic methods (immunohistochemistry, cytogenetics, hybridomas, molecular genetics), with the aim of selecting the most adequate treatment and consequently improving the prognosis of these aggressive embryonary tumours.

Adolescent↗
PubMed1996

Ewing's sarcoma and peripheral primitive neuroectodermal tumor: an interim report.

The primitive neuroectoderm and its progeny seemingly give rise to an ever-increasing number of clinicopathologic entities. Ewing's sarcoma and peripheral primitive neuroectodermal tumor have recently been united by relatively unique antigens expressed by the MIC-2 gene, commonly coexpressed neural markers, and cytogenetic and molecular genetic abnormalities. Because of these factors, the current thinking is that Ewing's sarcoma and the peripheral primitive neuroectodermal tumor are parts of a phenotypic spectrum. We present an "interim report" on this group of neoplasms, emphasizing their presentation in the head and neck.

Female↗
PubMed1998

Primitive neuroectodermal tumor in the spinal epidural space--case report.

A 4-year-old boy presented with a rare case of an epidural tumor causing compression of the thoracic spinal cord manifesting as rapid worsening of gait disturbance. The tumor was grossly totally resected, and radiotherapy and chemotherapy were subsequently administered. The histological diagnosis was primitive neuroectodermal tumor. He has been free of recurrence for more than 6 years. Early tumor resection followed by chemotherapy and irradiation is recommended for patients with primitive neuroectodermal tumor and unstable symptoms.

Chemotherapy, Adjuvant↗
PubMed1992

Ewing's sarcoma and extracranial primitive neuroectodermal tumors.

Ewing's sarcoma and primitive neuroectodermal tumor of bone and soft tissue are rare tumors, considered with the small round blue cell tumors of childhood. Recent advances link Ewing's sarcoma and primitive neuroectodermal tumor, and support the concept that these tumors represent stages of differentiation in a neoplasm of neuroectodermal origin. Advances in chemotherapy have resulted in the survival of the majority of patients presenting with these tumors. This review briefly outlines the year's literature regarding the histogenesis, biology, and treatment of this tumor.

Adolescent↗
PubMed1994

Immunolocalization of granulocyte-colony-stimulating factor in human glial and primitive neuroectodermal tumors.

Granulocyte-colony-stimulating factor (G-CSF) is a hematopoietic cytokine that regulates the differentiation of myeloid progenitors and the function of mature neutrophils. It is produced in vitro by monocytes/macrophages, mesothelial cells, fibroblasts and endothelial cells after appropriate induction by inflammatory mediators like IL-1 and TNF. Normal as well as tumorous glial cells can also be induced to produce CSFs in vitro. However, little is yet known about the in vivo expression of G-CSF as a mediator in inflammation and malignancy within the human central nervous system. The aim of the present study was to investigate by immunostaining the expression of the G-CSF protein within non-tumorous and tumorous glial tissues, and primitive neuroectodermal tumors. Using the murine monoclonal anti-G-CSF TM 82/60 antibody, we found high G-CSF expression in astrocytoma WHO grades I and II and reactive brain tissue, low expression in astrocytoma WHO grade III, and none in glioblastoma, oligodendroglioma WHO grades II and III, and medulloblastoma. In consecutive sections of the tissue samples, G-CSF protein was localized in GFAP-positive glial cells, but not in macrophages/microglial cells, which expressed HLA-DR, detected by the antibody CR3/43. Computer-assisted microdensitometric evaluation of the intensity of immunostaining for G-CSF and statistic analysis of the data revealed significant differences between the diagnostic entities studied (p < 0.0001). We conclude that in vivo expression of G-CSF is a characteristic of reactive as well as tumorous astrocytes, with the latter losing this feature at higher degrees of dedifferentiation.

Adolescent↗
PubMedDate not supplied

Primitive neuroectodermal tumor of the pancreas. An extremely rare tumor. Case report and review of the literature.

Peripheral primitive neuroectodermal tumor (PPNET) is a malignant neoplasm of the peripheral nervous system and soft tissues. Representing the fourth case published we herein report a PPNET arising in the pancreas of a six year old girl. She presented with severe anemia due to ulcerative tumor growth and hemorrhage into the duodenum. From the first biopsy pancreatoblastoma was considered as histological diagnosis. Therefore pancreato-duodenectomy was successfully performed. Immunohistochemically, the tumor cells were positive for cytokeratines and several neuronal markers. Due to focal membranous staining for MIC-2 gene product and rosettes in one lymph node metastasis the diagnosis had to be altered into PPNET. This was confirmed by cytogenetic analysis. We conclude that the interpretation of histologic sample excisions from pediatric pancreatic neoplasms may be difficult and that PPNET should be included in the differential diagnosis.

12E7 Antigen↗
PubMed1989

Molecular markers of primitive neuroectodermal tumors and other pediatric central nervous system tumors. Monoclonal antibodies to neuronal and glial antigens distinguish subsets of primitive neuroectodermal tumors.

Seventy-one tumors of the central nervous system in children were studied immunohistologically. Thirty-seven were classified histologically as PNETs, of which 35 were located in the cerebellum (medulloblastomas), one in the cerebrum, and one in the spinal cord. The 34 non-PNETs included five ependymomas, seven gangliogliomas, 15 astrocytomas, and seven tumors of other histology. We used monoclonal antibodies specific for neurofilament (NF) triplet proteins, for microtubule associated protein 2 and tau protein and for glial fibrillary acidic protein (GFAP) and myelin basic protein. In addition, a monoclonal antibody to epithelial membrane antigen was applied. The presence or absence of these antigens defined four major groups of PNETs: 1) PNETs not otherwise specified (10 cases), 2) PNETs with neuronal differentiation (eight cases), 3) PNETs with astrocytic differentiation (six cases), and 4) PNETs with both neuronal and astrocytic differentiation (12 cases). One case showed ependymal differentiation. The pattern of expression of NF isoforms in PNETs was reminiscent of that seen during normal mammalian development, such that phosphorylated NF-H was only present in combination with NF-M and NF-L. Among the other central nervous system tumors, all astrocytomas and gangliogliomas were positive for GFAP, and the gangliogliomas also expressed all NF isoforms. Three atypical teratoid tumors and two rhabdoid tumors showed strong positivity for epithelial membrane antigen and also for GFAP. We conclude that the differentiation antigens described here serve to distinguish PNETs from other pediatric central nervous system tumors and to identify subsets of PNETs. Accordingly, PNETs represent a heterogeneous group of pediatric brain tumors capable of neuronal and glial differentiation.

Adolescent↗
PubMed1995

The multiple tumor suppressor 1/cyclin-dependent kinase inhibitor 2 gene in human central nervous system primitive neuroectodermal tumor.

The recently described multiple tumor suppressor 1/cyclin-dependent kinase inhibitor 2 (MTS1/CDKN2) gene, encoding the cyclin-dependent kinase 4 inhibitor p16, is mutated in a wide variety of tumor cell lines, including gliomas. To investigate the possible role of this gene in the genesis of the central nervous system primitive neuroectodermal tumor (PNET), four established PNET cell lines and 18 PNET surgical specimens were studied for deletions and mutations of the MTS1/CDKN2 gene. One of the four cell lines had homozygous deletion of the gene. No mutation in any of the three MTS1/CDKN2 exons was detected in the other three cell lines by single strand conformational polymorphism analysis. Eighteen surgical PNET specimens were studied for allelic and homozygous deletion at chromosome 9p21, the location of the MTS1/CDKN2 gene. No loss of heterozygosity was noted in 11 of the tumors, and no homozygous loss was noted in any tumor. Single strand conformational polymorphism analysis of the entire coding region of the MTS1/CDKN2 gene revealed no mutation within MTS1/CDKN2 in any tumor. Although deletion of MTS1/CDKN2 may occur in some PNET cell lines, neither deletion nor mutation of the gene is found in tumors before culture. The genesis of the human central nervous system PNET does not involve deletion or mutation of the MTS1/CDKN2 gene.

Base Sequence↗
PubMed1992

Establishment and characterization of a cell line of congenital primitive neuroectodermal tumor of soft tissue.

A new human cell line, termed Muraoka, has been established from the recurrent tumor of a case of congenital primitive neuroectodermal tumor (PNET) arising at the temporofacial region of a male infant. The microscopic findings of this cell line were epithelioid, and the xenografted tumor in a nude mouse consisted of the malignant epithelioid cells. Immunohistochemically, the cells were positive for neuron-specific enolase, S-100 protein, carcinoembryonic antigen, cytokeratin, epithelial membrane antigen, and glial fibrillary acidic protein. These findings were quite similar to those of the epithelioid cells in the original tumor and of the xenografted tumor cells. Neither chromosomal abnormalities nor N-myc amplification were observed. Morphological differentiation after treatment with N6-2'-O-dibutyryladenosine 3':5'-cyclic monophosphate (Bt2-cAMP), all-trans-retinoic acid (RA), prostaglandin E1 (PGE1), and 5-bromo-2'-deoxyuridine (BrdU) showed two different results. Bt2-cAMP and PGE1 induced neuronal differentiation with the extension of neurites, whereas RA and BrdU predominantly induced Schwannian differentiation (flat cells). In these respects, the cell line Muraoka seems to be useful for studying characteristics of PNET as well as for developing the new treatments against such tumors.

Animals↗
PubMed1991

Primitive neuroectodermal tumor of the leptomeninges.

Primitive neuroectodermal tumor (PNET) without an obvious intraparenchymal mass lesion and with diffuse spread in the subarachnoid space is rare. We report such a rare case and discuss the computed tomography (CT) and magnetic resonance (MR) features.

Adolescent↗
PubMed1990

[Induction of primitive neuroectodermal tumors by oncogene complementation].

Primitive neuroectodermal tumors (PNET) represent a family of undifferentiated neural neoplasms which predominantly occur in children. PNETs are likely to originate from precursor cells and exhibit a marked potential for neuronal, glial and ependymal differentiation. A prominent example is the medulloblastoma of the cerebellum. In a model system using a novel transgenic CNS transplantation model, we have introduced a combination of ras and myc oncogenes into cell suspensions from fetal forebrain (E14) and postnatal cerebellum (P2) of the rat. Oncogene transfer into fetal forebrain grafts resulted in a high incidence of anaplastic neural tumors predominantly derived from glial precursors. Cell lines established from these neoplasms expressed high levels of both oncogenes. In a second experiment, the ras/myc vector was introduced into cell suspensions from neonatal cerebellum. The transformed cells were cultured for 3 weeks. Following stereotaxic transplantation, tumors of a similar morphology were observed. However, one animal developed a neoplasm with features of a cerebellar medulloblastoma. A cell line which exhibits a marked capacity for neurite extension and synaptogenesis was established from this tumor. Since these cells neither express ras nor myc, an insertion mutagenesis event appears to be responsible. Experiments to characterize this mutation are in progress. Our results indicate a potent transforming effect of ras and myc on neural precursor cells in vivo.

Animals↗
PubMed1992

Sinonasal primitive neuroectodermal tumor arising in a long-term survivor of heritable unilateral retinoblastoma.

BACKGROUND: Patients who survive retinoblastoma (RB) are at risk for having second nonocular tumors, usually osteosarcomas, which often are fatal. Such patients almost always have bilateral RB. METHODS: This article reports a woman who, at the age of 1 year had been cured of a unilateral RB by radiation therapy and enucleation. Eighteen years later, she had a sinonasal small cell tumor that rapidly recurred and proved fatal 2 months after surgical debulking. The tumor was studied by immunohistochemistry and electron microscopic (EM) examination. RESULTS: It showed diffuse neuron-specific enolase staining, focal weak staining for chromogranin, synaptophysin, and Leu-7 monoclonal antibodies in paraffin-embedded, B5-fixed tissue (Great Lakes Diagnostics, Troy, MI). EM study showed an undifferentiated primitive neuroectodermal tumor with many polyribosomes, simple cell junctions, few microtubules, and rare dense core granules. CONCLUSIONS: The combined immunohistochemical, ultrastructural, and clinical features of the tumor were interpreted as a sinonasal primitive neuroectodermal tumor with early neuronal differentiation. The tumor was pathologically indistinguishable from poorly differentiated olfactory neuroblastoma (ONB) and Ewing sarcoma.

Chromogranins↗
PubMed1999

Bone morphogenetic proteins-2 and -4 attenuate apoptosis in a cerebellar primitive neuroectodermal tumor cell line.

Similarities between primitive neuroectodermal tumors and central nervous system (CNS) progenitor cells have evoked interest in the response of these tumors to endogenous growth factors. The bone morphogenetic proteins (BMPs) have recently been found to regulate survival and differentiation of CNS progenitor cell populations. In this study, we investigated the effects of BMP-2, BMP-4, and BMP-6 on the undifferentiated cerebellar primitive neuroectodermal tumor or medulloblastoma cell line DAOY. Analysis by reverse transcriptase-polymerase chain reaction showed that mRNAs for type IA and type II BMP receptors were present in control cultures. In cultures treated with BMP-2, mRNAs for BMP receptor type IB and the activin R-I receptor became evident. Cultures were analyzed for total cell counts, proliferating cell nuclear antigen (PCNA), and apoptotic DNA fragmentation. There was a significant increase in total cell number in the BMP-2 and BMP-4 treatment groups, without any change in PCNA reactivity, and a dramatic decrease in the proportion of apoptotic nuclei at concentrations of BMP-2 and BMP-4 above 5 ng/ml (P<0.001). These effects were not observed with BMP-6, TGF-beta1 or GDNF. These results suggest that the increase in total cell number is due to the attenuation of apoptosis by BMP-2 and BMP-4. The anti-apoptotic effect of BMP-2 and BMP-4 on this neuroectodermal cell line has potential clinical implications for neuroectodermal tumors.

Apoptosis↗
PubMed1999

[A case of primitive neuroectodermal tumor of the kidney].

A primitive neuroectodermal tumor (PNET) is a solid tumor originating from the neural crest. This tumor is known to occur in the central nervous system and soft tissue, but recently determined to also invade the kidney. Although primary renal PNET is very rare, we encountered a case of primary renal PNET with multiple lung metastasis. This case was a 35-year-old man with a chief complaint of macroscopic hematuria, which he noticed in January 1996. He was hospitalized because of right renal tumor detected with ultrasonography. On CT scan, a low-density solid tumor with a maximum diameter of 8 cm was visualized in the right kidney. Low-signal and high-signal tumorous lesions were demonstrated in the same region on T 1-weighted and T 2-weighted MR images, respectively. Then right radical nephrectomy was performed on Feb. 5, 1996. On histopathological observation, the tumor was composed of small tumor cells with solid growth. Immunohistochemical staining revealed that the tumor was positive for CD 99. Thus our final diagnosis was primary right renal PNET. Although 2 metastatic lesions with a diameter of about 1 cm were observed in the right lower lung before the time of surgery, pulmonary metastatic lesions markedly increased in number 3 months after surgery. Thus we initiated CAP chemotherapy with cyclophosphamide, doxorubicin, and CDDP. After 3 cycles, pulmonary metastatic lesions disappeared at CT scan, indicating complete remission. However, a tumor with a maximum diameter of about 10 cm recurred in the retroperitoneum 7 months after complete remission. Although 2 cycles of CAV/PE therapy with cyclophosphamide, doxorubicin, vincristine, CDDP, and VP-16 were performed following resection of the retroperitoneal tumor, he showed no response and decreased 24 months after surgery.

Adult↗