Askin Rosai tumor--a type of primitive neuroectodermal tumor.
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Humans↗
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Primary primitive neuroectodermal tumor of the kidney.
Primitive neuroectodermal tumor (PNET) is a small round cell sarcoma that mainly develops in the central nervous system and soft tissues of childhood; however recently, primary occurrence of this tumor in the kidney has been reported. We experienced one case of PNET primarily arose in the kidney without metastasis. The patient was a 28-year-old man whose chief complaint was abdominal pain, especially on exercise. On computed tomography scan and magnetic resonance imaging, a solid lesion was found in the left kidney, and a left nephrectomy was performed based on the diagnosis of a tumor in the left kidney. The tumor was within the parenchyma of lower end of left kidney protruding into the abdominal cavity. Histologically, diffuse proliferation of primitive small round cells with rosette formation was found. Immunohistochemically, MIC2 gene product, neuron-specific enolase and S-100 protein were positive. No metastasis to the regional lymph nodes was found. From these observations, the tumor was diagnosed as PNET primarily arising in the left kidney. Although chromosome analysis was not performed, EWS-FLI1 chimera gene was identified by reverse transcriptase-polymerase chain reaction on the freshly frozen specimen and fluorescence in situ hybridization on paraffin sections.
12E7 Antigen↗
A unique occurrence of a cerebral atypical teratoid/rhabdoid tumor in an infant and a spinal canal primitive neuroectodermal tumor in her father.
This report describes the clinical, pathological, immunohistochemical and genetic data of two rare malignant neoplasms of the central nervous system (CNS)--a cerebral atypical teratoid/rhabdoid tumor (AT/RT) in a 5-month-old girl and a spinal canal primitive neuroectodermal tumor (PNET) in her father. Despite aggressive treatment, both tumors were fatal, displaying extensive local recurrence and diffuse neoplastic dissemination. The paraffin-embedded tumor tissue samples were analyzed using a dual-color FISH with a locus specific LSI22q (BCR) probe. In the AT/RT tissue, a loss of BCR locus was observed in a significant proportion of the cells in contrast to the PNET specimen where the majority of nuclei did not reveal any loss of the BCR region. No mutations in exon 5 and no changes in SNP of intron 5 of hSNF/INI1 gene were found. In addition, analysis of loss of heterozygosity (LOH) was performed using a panel of 15 microsatellite markers of chromosome 22. No LOH were found in both tumor tissues. In both cases no constitutional mutations of gene TP53 were found. Analysis of the TP53 mutations in the tumor tissues revealed that the PNET, not the AT/RT tumor, was homozygous for a missense mutation at codon 175 (CGC ==> CAC). Thus, our findings emphasize the genetic differences between the two specimens and suggest that the occurrence of these two aggressive tumors of CNS in one family could be coincidental.
Adult↗
[Primitive neuroectodermal tumor of the kidney].
Primitive neuroectodermal Tumor (PNET) is a rare malignant tumor of young adult. Patients often present with the classical trias of renal cancer (pain, haematuria, palpable tumor). Specific radiological signs are missing, and therefore a PNET is often diagnosed postoperatively. It is characterized by the expression of MIC2, neural markers (Vimentin, S-100, Synaptophysin) and EWS/FLI1-translocation. The tumor is often diagnosed in advanced stage, and prognosis is poor despite of multimodal treatment including radical nephrectomy, polychemotherapy (vincristine, adriamycin, cyclophosphamid, etoposide, ifosfamide) and radiation in case of metastases or incomplete resection of primary tumor. We represent two cases of metastatic renal PNET in a 28- and 39-year-old patient. Due to the multimodal treatment a partial and a complete remission was achieved, respectively.
Adult↗
Pineal and nonpineal supratentorial primitive neuroectodermal tumors.
Pineal region supratentorial primitive neuroectodermal tumors (SPNETs; pineoblastomas) and nonpineal SPNETs are rare tumors that historically have carried a very poor prognosis. With multimodality therapy, including maximal surgical resection, craniospinal radiation therapy and chemotherapy, the survival for patients with pineal PNETs has significantly improved. Chemotherapy alone, at least in conventional doses, appears to be insufficient treatment for younger children with pineoblastomas, in whom there is almost universal rapid tumor progression and death. Survival of patients with nonpineal SPNETs remains in the order of 30-35% despite multimodality therapy. Unlike those with pineal SPNETs, a significant percentage of infants with nonpineal SPNETs who undergo gross total surgical resection followed by chemotherapy will be long-term survivors. This article gives an overview of the natural history, prognostic factors and treatment of both pineal and nonpineal SPNETs.
Child, Preschool↗
Neurotrophins and Trk receptors in primitive neuroectodermal tumor cell lines.
OBJECTIVE: Primitive neuroectodermal tumors (PNETs) are thought to be derived from early central nervous system precursors. Therefore, we hypothesized that the neurotrophins (nerve growth factor, brain-derived neurotrophic factor, and neurotrophin-3) and their receptors (TrkA, TrkB, and TrkC), which are involved in the proliferation, differentiation, and survival of neuronal cells, might be important in regulating tumor growth. METHODS: Using ribonucleic acid (RNA) blotting and reverse transcription-polymerase chain reaction analysis, we investigated the expression of these ligands and their receptors in six PNET cell lines (Daoy, PFSK, D283 Med, UW288-1, CHP707m, and D341 Med). Neurotrophin protein levels were measured using enzyme-linked immunosorbent assay procedures. Receptor function was demonstrated by autophosphorylation. Induction of c-Fos expression and effects on cell proliferation were assessed after the addition of exogenous neurotrophin. RESULTS: Three cell lines expressed messenger RNA for all neurotrophins, whereas the other three expressed two of the three neurotrophins. Neurotrophin protein levels were low. All cell lines expressed trkA messenger RNA. Five expressed the amino terminus of trkB, but three of these did not express the carboxyl terminus. All cell lines contained trkC messenger RNA, but the receptor was truncated in two cell lines. No cell line contained message for a receptor containing an insertion in the tyrosine kinase domain. The addition of neurotrophin to PNET cells resulted in phosphorylation of a protein that was immunoprecipitated with an anti-pan-Trk antibody. c-Fos expression and cell growth were increased by preincubation with neurotrophins, but only in the cell lines expressing the relevant full-length receptors. CONCLUSION: The expression of neurotrophins and neurotrophin receptors by PNET cell lines is variable. The presence of activated Trk receptors in these cell lines may be required for rapid growth, via an autocrine loop mechanism. This will require further investigation.
Brain Neoplasms↗
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↗
Somatostatin receptor subtype 2 is expressed by supratentorial primitive neuroectodermal tumors of childhood and can be targeted for somatostatin receptor imaging.
PURPOSE: Although gliomas predominate among central nervous system (CNS) neoplasms in adulthood, embryonal tumors are the most common malignant brain tumors in children. Despite novel treatment approaches, including improved radiotherapy and high-dose chemotherapy, survival rates remain unsatisfactory. The timely diagnosis of residual or recurrent embryonal CNS tumors and thus the earliest possible time point for intervention is often hampered by inaccuracies of conventional imaging techniques. Novel and refined imaging methodologies are urgently needed. EXPERIMENTAL DESIGN: We have previously demonstrated the use of somatostatin receptor imaging (SRI) in the diagnosis of recurrent and residual medulloblastomas. Here, we evaluated somatostatin receptor type 2 (sst(2)) expression using an antibody in an array of CNS tumors of childhood. Eight high-grade gliomas, 4 atypical teratoid/rhabdoid tumors, 7 supratentorial primitive neuroectodermal tumors (stPNET), 1 medulloepithelioma (ME), and 8 ependymomas were screened. Tumors positive in vitro were additionally analyzed in vivo using SRI. RESULTS: Abundant expression of somatostatin receptor type 2 in stPNET, a ME, and ependymomas warranted in vivo imaging of 7 stPNET, 1 rhabdomyosarcoma, 3 ependymomas, 1 ME, and 1 glioblastoma. Although SRI was positive in 6/7 stPNET, 1 rhabdomyosarcoma, and 1 ME, none of the ependymomas nor the glioblastoma could be imaged using SRI. In selected cases SRI was more sensitive in the detection of relapse than conventional imaging by magnetic resonance imaging and computed tomography. CONCLUSIONS: SRI should be considered in the evaluation of residual or recurrent embryonal CNS tumors, especially stPNET. The strengths of SRI lie in the differentiation of reactive tissue changes versus residual or recurrent tumor, the detection of small lesions, and possibly in the distinction of stPNET from gliomas.
Central Nervous System Neoplasms↗
Current diagnostic and therapeutic management of CNS metastasis in childhood primitive neuroectodermal tumors and ependymomas.
Metastatic disease is a major problem in the management of the most frequent childhood brain tumors, in ependymoma and primitive neuroectodermal tumors (PNET). Today, contrast enhanced craniospinal MRI and careful analysis of craniospinal fluid are a prerequisite for correct staging of both tumors and imply therapeutic consequences. So far metastatic spread of medulloblastoma and some ependymomas is prevented by conventional chemotherapy and radiotherapy. However, some forms of diffuse metastatic dissemination in medulloblastoma are resistant to conventional therapeutic regimens and require new experimental strategies.
Brain Neoplasms↗
Primitive neuroectodermal tumor of maxilla in an adult.
Primitive neuroectodermal tumor is primarily a central nervous system tumor. These tumors are generally manifest in infancy or early childhood. The following article reports a rare case of primitive neuroectodermal tumor in posterior maxilla in an adult. Treatment for primitive neuroectodermal tumor in extracranial sites in adults is not clearly defined in the literature. This case was treated by combined chemotherapy followed by radiotherapy, which failed to cause regression of the lesion.
Adult↗
Supratentorial primitive neuroectodermal tumors of infancy: clinical and radiologic findings.
One hundred ninety-eight children were entered on POG 8633, "Prolonged Postoperative Chemotherapy and Delayed Radiation for Children <3 years of age with Malignant Brain Tumors" (1986-1990). Thirteen manifested supratentorial nonpineoblastoma primitive neuroectodermal tumors, making this the second most common supratentorial tumor in the study. Symptoms and signs included seizures, nausea, vomiting, lethargy, irritability, headache, focal motor weakness, and increased head circumference. Twelve of 13 had symptoms for less than 1 month before diagnosis. The average tumor size was 5.96 cm (+/- 0.37) x 5 cm (+/- 0.28) x 5.15 cm (+/- 0.31). Eight tumors were predominantly hemispheral, and five were midline. Computed tomographic scans on nine patients revealed tumor hyperdensity (nine), midline shift (eight), hydrocephalus (seven), cysts (six), well-defined borders (five), and calcification (four). No patients exhibited peritumoral edema. Contrast enhancement, primarily heterogeneous, was present in all patients. Magnetic resonance imaging appearance of the tumor (six patients) demonstrated midline shift (four), well-defined margins (four), necrosis (two), cysts (three), and hemorrhage (two). No peritumoral edema was present. Most enhanced heterogeneously. The diagnosis of supratentorial nonpineoblastoma primitive neuroectodermal tumors should be suspected when a large, sharply marginated, hyperdense supratentorial mass is observed in a young child, particularly when no peritumoral edema is present.
Child, Preschool↗
Primitive neuroectodermal tumor of the vagina.
OBJECTIVES: Primitive neuroectodermal tumor is grouped together with Ewing sarcoma of the bone, but its presentation includes a wider age range and more central locations than its osseous counterpart. Presentation in vagina is exceedingly rare. CASE: A 34-year-old woman with personal history of kidney transplant presented with a painless vaginal mass. After surgical excision, pathology results demonstrated a primitive neuroectodermal tumor. Chemotherapy was given, followed by external beam and vaginal intracavitary brachytherapy. After 20 months postexcision, there is no clinical nor radiographic evidence of recurrence. CONCLUSIONS: This is the second case of primitive neuroectodermal tumor located in the vagina reported in the literature.
Adult↗
Peripheral primitive neuroectodermal tumors of the head and neck.
OBJECTIVE: Primitive neuroectodermal tumor is a malignant small round cell tumor of neuroectodermal origin. The occurrence of these tumors in the head and neck is rare. Our objective is to document the frequency of presentation, methods of diagnosis, and forms of treatment used to combat primitive neuroectodermal tumors in the head and neck. DESIGN: Retrospective chart review. RESULTS: The most common location for primitive neuroectodermal tumors in our series was the thoracopulmonary region (46%); the next most common location was the head and neck (42%). Metastatic disease was present in 31% of patients at the time of diagnosis. Cytogenic analysis was found to be helpful in confirming the diagnosis of primitive neuroectodermal tumor. Therapy for primitive neuroectodermal tumors included surgery, chemotherapy, and radiation therapy. Survival statistics were poor, with 65% of the patients alive at 2 years' follow-up. CONCLUSIONS: Primitive neuroectodermal tumor is an aggressive malignant small round cell tumor that may present in the head and neck. Long-term survival for patients with primitive neuroectodermal tumor is still poor; however, the use of improved chemotherapeutic agents combined with aggressive surgical control of primary disease and, in some cases, radiation therapy should lead to improved long-term survival.
Adolescent↗
PTEN and DMBT1 homozygous deletion and expression in medulloblastomas and supratentorial primitive neuroectodermal tumors.
Medulloblastoma, which accounts for 20-25% of all childhood brain tumors, is defined as a primitive neuroectodermal tumor (PNET) located in the cerebellum. Supratentorial PNET are less frequent than medulloblastoma. But their clinical outcome is worse than in medulloblastomas. Chromosome 10q contains at least 2 tumor suppressor genes that might play a role in brain tumor development: PTEN and DMBT1. The aim of this study was to compare the status of homozygous deletion and expression of PTEN and DMBT1 genes in PNET primary tumor samples and cell lines. Homozygous deletions of PTEN and DMBT1 were studied in 32 paraffin-embedded PNET samples (23 medulloblastomas and 9 supratentorial PNET) and in 7 PNET cell lines, by differential PCR and by FISH. PTEN homozygous losses were demonstrated in 7 medulloblastomas (32%) and in no supratentorial PNET, while homozygous deletions of DMBT1 appeared in 1 supratentorial PNET (20%) and in 7 medulloblastomas (33%). No homozygous deletion of PTEN or DMBT1 was detected in any of the PNET cell lines either by differential PCR or by FISH. Expression study of the 2 genes was performed in the 7 PNET cell lines by RT-PCR. One PNET cell line lacked PTEN and DMBT1 expression, while 2 medulloblastoma cell lines did not express DMBT1. Our results add some positive data to the hypothesis that supratentorial PNETs and medulloblastomas might be genetically different.
Agglutinins↗
Reduction to homozygosity and gene amplification in central nervous system primitive neuroectodermal tumors of childhood.
The loss of genetic material from specific chromosomal locations has been identified for a number of pediatric tumors. This loss has been taken as evidence for the importance of tumor suppressor genes at these loci in the genesis of these tumors. One of these pediatric tumors, the primitive neuroectodermal tumor of the central nervous system, has not been well studied. In this report, an analysis of primitive neuroectodermal tumors for allelic deletions on chromosomes 1p, 7q, 10, 11p, 13q, and 17p has been performed. One of ten tumors was found to have increased copies of c-myc. Three different patients were found to reduce to homozygosity at one of three different locations. Significantly, however, three of nine informative patients showed a reduction to homozygosity on chromosome 17p. Thus, primitive neuroectodermal tumor is one of a growing number of tumor types in which deletions in the short arm of chromosome 17 might be important in oncogenesis.
Brain Neoplasms↗
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↗
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↗
Congenital cerebellar primitive neuroectodermal tumor in a newborn.
Neonatal primitive neuroectodermal tumor is a rare clinical entity. The initial presentation in affected newborns is often subtle, nonspecific, and rarely includes neurological signs. Here we report a newborn with congenital cerebellar primitive neuroectodermal tumor. We had detected hydrocephalus and enlarged biparietal diameter at the 36th week of gestational age by fetal ultrasonography.
Cerebellar Neoplasms↗