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PubMed2001

Primitive neuroectodermal tumor of the kidney associated with Budd-Chiari syndrome in a 17-year-old girl.

Renal primitive neuroectodermal tumor (PNET) is a rare and highly malignant neoplasm of the kidney. We report the case of a 17-year-old girl with renal PNET that was complicated by Budd-Chiari syndrome. She was admitted due to abrupt left flank pain and gross hematuria. Abdominal sonography and computerized tomography (CT) disclosed a large hemorrhagic left renal mass and thrombus in the inferior vena cava (IVC). Left radical nephrectomy was performed and renal PNET with tumor rupture and tumor invasion into the IVC was diagnosed based on operative findings and histologic features. Tumor cells were positive for neuronspecific enolase, chromogranin-A, and vimentin but negative for cytokeratin, leukocyte common antigen, CD3, and CD20. The thrombus in the IVC extended into the right atrium and caused obstruction of the right and middle hepatic venous outflow, which was evident on follow-up CT scan 5 months later. The patient died due to hepatic failure and progressive cardiovascular compromise 6 months after surgery. This case demonstrates that renal PNET can be life threatening when the tumor thrombus extends into the IVC and causes hepatic outflow obstruction.

Adolescent↗
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↗
PubMed2005

Secondary supratentorial primitive neuroectodermal tumor following irradiation in a patient with low-grade astrocytoma.

We report a case of a supratentorial primitive neuroectodermal tumor (PNET) that occurred 12 years after cranial irradiation for a grade II astrocytoma. Neuroimaging was unable to distinguish between a recurrence of the original neoplasm and the development of a new, distinct entity. Pathologic review assisted by immunohistochemical staining, however, revealed a high-grade PNET. Although rare, PNET needs to be included in the differential diagnoses for previously irradiated patients, who develop recurrent brain tumors in the presence of uncharacteristic imaging features.

Adult↗
PubMed1997

No preferential parent of origin for the isochromosome 17q in childhood primitive neuroectodermal tumor (medulloblastoma).

We have shown that an i(17q) is the most frequent abnormality in central nervous system primitive neuroectodermal tumors (PNETs; medulloblastoma), implicating the presence of a tumor suppressor gene which maps to 17p. In the present study, we investigated whether the deletion of chromosome arm 17p that results from the formation of the i(17q) is preferentially of maternal or paternal origin. Eight cases of primary PNETs of the posterior fossa were examined at five polymorphic loci which map to 17p13. Two or three informative loci were detected in each family. Of the eight cases, four tumors evidenced loss of the paternal allele for loci on 17p13 and four tumors demonstrated maternal deletions. Although the number of cases is relatively small, these studies do not implicate the loss of an imprinted gene as a mechanism for tumorigenesis in children with central nervous system PNETs/medulloblastoma.

Adolescent↗
PubMed1996

Common alternative gene alterations in adult malignant astrocytomas, but not in childhood primitive neuroectodermal tumors: P 16ink4 homozygous deletions and CDK4 gene amplifications.

Alterations in P16ink4 or in the gene encoding one of its ligands, cyclin-dependent kinase 4 (CDK4), have been reported in human glioma cell lines and primary tumors but not in primitive neuroectodermal tumors (PNETs), the most common malignant brain tumor of childhood. In this study the authors have examined DNA from 20 primary PNETs in children and from 20 malignant astrocytomas to assess the frequency of P16ink4 and CDK4 gene alterations associated with each type of tumor. Southern hybridization analysis revealed homozygous P16ink4 deletions in one (5%) of 20 PNETs and in seven (35%) of 20 malignant astrocytomas. The CDK4 gene amplification was evident in two additional astrocytomas, but not in any of the PNETs. In total, nine astrocytomas (45%) exhibited homozygous P16ink4 deletion or CDK4 gene amplification, but only one PNET (5%) demonstrated either gene alteration. These results indicate that the incidence of P16ink4 and CDK4 gene alterations in these two groups of tumors is different and suggest distinct pathogenetic etiologies may be associated with each neoplasm.

Adolescent↗
PubMed2000

[Primitive neuroectodermal tumor of the kidney manifested as a spontaneous hematoma].

Ultrasound and CT in a young man, admitted for violent left flank pain, revelead a large heterogeneous left renal mass, with hypodense collection along the psoas. The diagnosis was spontaneous renal hematoma. A check CT 3 weeks later found a large tumor of the inferior pole. After radical nephrectomy, histopathology diagnosed a primitive neuroectodermal tumor. The patient died one year later despite neoadjuvant chemotherapy. This article reports the clinical, radiological and histopathological aspect of a primitive neuroectodermal tumor of the kidney, compared with the other cases in literature.

Adult↗
PubMed1998

Peripheral primitive neuroectodermal tumor of the ovary confirmed by CD99 immunostaining, karyotypic analysis, and RT-PCR for EWS/FLI-1 chimeric mRNA.

We report a case of peripheral primitive neuroectodermal tumor (pPNET), which belongs to the PNET/Ewing's sarcoma family, arising in the left ovary of a 29-year-old woman. Microscopically, the tumor was composed of solid nests and sheets of monotonous, primitive, small round cells with a few rosettes, making it difficult to distinguish from small cell carcinoma of the ovary. Immunohistochemically, the tumor cells showed intense cell-membranous immunoreactivity for MIC2 protein (CD99). A short-term cell culture and karyotypic analysis revealed the tumor to possess a balanced t(11;22)(q24;q12) chromosomal translocation that is highly specific for tumors of the PNET/Ewing's sarcoma family. In addition, EWS/FLI-1 chimeric mRNA that originated from the characteristic chromosomal translocation was detected by reverse transcription-polymerase chain reaction. These results confirmed the diagnostic validity of the present tumor being a pPNET, thus raising the possibility that in the past, pPNETs which have arisen in the ovary may have been mistakenly diagnosed as small cell carcinomas of the ovary.

12E7 Antigen↗
PubMed1993

Primitive neuroectodermal tumor arising in an ovarian mature cystic teratoma: immunohistochemical and electron microscopic studies.

The occurrence of a malignant neuroectodermal tumor in a mature cystic teratoma is extremely rare. Five cases of ovarian primitive neuroectodermal tumors (PNET) or neuroepitheliomas have been reported. In two, the tumor was adjacent to foci of a mature teratomatous element. We present the immunohistochemical profile and electron microscopic study of an ovarian mature cystic teratoma with PNET or malignant neuroepithelioma. The transition between the mature neural elements and the malignant PNET presents a model for monodermic neuroepithelial differentiation and explains the histogenesis of this unusual tumor.

Adult↗
PubMed2006

Primary pulmonary primitive neuroectodermal tumor: CT and skeletal scintigraphic image features with pathologic correlation (2006: 6b).

We describe a rare case of a primitive neuroectodermal tumor (PNET) originating in the lung of a 28-year-old woman. A lung mass was detected incidentally on a chest radiograph taken before a Caesarian delivery. The chest P-A view showed a well-defined, lobulated mass in the right lower lobe with a small pleural effusion on the right. A thoracic CT with contrast enhancement showed a lobulated, heterogeneous, enhanced mass of approximately 9.2 x 8.4 x 6.3 cm with inner amorphous calcifications in the right lower lobe and a small right pleural effusion. A right lower lobectomy was performed 1 month after delivery. The pathology report was a primary pulmonary PNET with focal visceral pleural involvement.

Adult↗
PubMed2005

Genomic deletions in cell lines derived from primitive neuroectodermal tumors of the central nervous system.

Extensive genomic deletions affecting a variety of chromosomes are a common finding in primitive neuroectodermal tumors of the central nervous system (CNS-PNETs), implicating the loss of multiple tumor suppressor genes in the pathogenesis of these tumors. We have used representational difference analysis, microsatellite mapping, and quantitative polymerase chain reaction to identify and verify the presence of genomic deletions on a number of chromosomes in CNS-PNET cell lines. This systematic approach has confirmed the importance of deletions at 10q, 16q, and 17p in PNET pathology and has revealed other regions of deletion not commonly described (e.g., Xq, 1p, 7p, and 13q). These data highlight the prevalence of hemizygous loss in CNS-PNET cells, suggesting that haploinsufficiency affecting multiple tumor suppressor genes may play a fundamental role in CNS-PNET pathogenesis. The identification of specific genes and signaling pathways that are compromised in CNS-PNET cells is crucial for development of more efficacious and less invasive treatments, as are urgently needed.

Cell Line, Tumor↗
PubMed2005

Supratentorial primitive neuroectodermal tumors (S-PNET) in children: A prospective experience with adjuvant intensive chemotherapy and hyperfractionated accelerated radiotherapy.

PURPOSE: Supratentorial primitive neuroectodermal tumors (S-PNET) are rare and have a grim prognosis, frequently taking an aggressive course with local relapse and metastatic spread. We report the results of a mono-institutional therapeutic trial. METHODS AND MATERIALS: We enrolled 15 consecutive patients to preradiation chemotherapy (CT) consisting of high-dose methotrexate, high-dose etoposide, high-dose cyclophosphamide, and high-dose carboplatin, craniospinal irradiation (CSI) with hyperfractionated accelerated radiotherapy (HART) plus focal boost, maintenance with vincristine/lomustine or consolidation with high-dose thiotepa followed by autologous stem-cell rescue. RESULTS: Median age was 9 years; 7 were male, 8 female. Site of disease was pineal in 3, elsewhere in 12. Six patients were had no evidence of disease after surgery (NED). Of those with evidence of disease after surgery (ED), 2 had central nervous system spread. Of the 9 ED patients, 2 had complete response (CR) and 2 partial response (PR) after CT, 4 stable disease, and 1 progressive disease. Of the 7 ED patients before radiotherapy, 1 had CR, 4 PR, and 2 minor response, thus obtaining a 44% CR + PR after CT and 71% after HART. Because of rapid progression in 2 of the first 5 patients, high-dose thiotepa was systematically adopted after HART in the subsequent 10 patients. Six of 15 patients relapsed (4 locally, 1 locally with dissemination, 1 with dissemination) a mean of 6 months after starting CT, 2 developed second tumors; 5 of 6 relapsers died at a median of 13 months. Three-year progression-free survival, event-free survival, and overall survival were 54%, 34%, and 61%, respectively. CONCLUSION: Hyperfractionated accelerated RT was the main tool in obtaining responses in S-PNET; introducing the myeloablative phase improved the prognosis (3/10 vs. 3/5 relapses), though the outcome remained unsatisfactory despite the adoption of this intensive treatment.

Adolescent↗
PubMedDate not supplied

High-dose chemotherapy with autologous stem cell rescue as first line of treatment in young children with medulloblastoma and supratentorial primitive neuroectodermal tumors.

In order to improve the dismal prognosis of patients younger than 4 years old with medulloblastoma and supratentorial primitive neuroectodermal tumors (stPNET) seven young children were treated with high-dose chemotherapy (HDCT) and autologous stem cell rescue in our center. All patients underwent surgical debulking and standard chemotherapy. None of them received irradiation. The HDCT included busulfan 16 mg/kg, orally over 4 days (from days -5 to -2) in 6 hourly divided doses, and melphalan at a dose of 140 mg/m2 given by intravenous infusion over 5 min on day -1. Three patients additionally received thiotepa 250 mg/m2 given by intravenous infusion daily over 2 days (from day -2 to -1) and two patients additionally received topotecan 2 mg/m2 given by intravenous infusion daily over 30 min for 5 days (from day -11 to -7). Patients' stem cells were mobilized with granulocyte colony-stimulating factor at a dose of 12 microg/kg twice daily subcutaneously for four consecutive days. Cryopreserved peripheral blood progenitor cells were reinfused 48 h after completion of chemotherapy. With a median follow-up of 21 months (range 5-64) five complete responses were observed; one patient had partial response and one had stable disease. There was no treatment-related mortality. The 2 year event-free survival was 71.43 +/- 17%. Therefore we conclude that HDCT as consolidation regimen may improve the cure rates in very young children with medulloblastoma/stPNET avoiding long-term sequelae of radiotherapy.

Antineoplastic Agents, Alkylating↗
PubMed1998

Basimeningeal/spinal axis primitive neuroectodermal tumor without intraaxial lesion: radiological features.

Over a 12-year period, the authors encountered 2 patients with spinal axis/basimeningeal primitive neuroectodermal tumor (PNET) involving the spinal axis extramedullary, intradural compartment and the basimeninges. In neither patient was an intraaxial focus demonstrated. A 48-year-old woman had computed tomography (CT) findings of hydrocephalus and later abnormal basimeningeal enhancement. Cervical myelography revealed a low cervical intradural block. A 10-year-old boy was evaluated initially with non-contrast-enhanced CT of the brain, which revealed hydrocephalus. Contrast-enhanced CT revealed abnormal basimeningeal enhancement implicating cisternal obstruction. Subsequent magnetic resonance examination of the entire spine with and without gadolinium enhancement revealed diffuse abnormal enhancement of the spinal axis intradural, extramedullary compartment. While intraaxial PNETs are well recognized, the patients described here represent a distinctly unusual pattern of intradural spinal meningeal and basimeningeal infiltration by PNET in the absence of a demonstrable intraaxial lesion.

Brain Neoplasms↗
PubMed2005

[Primitive neuroectodermal tumor arising 8 years after chemotherapy and radiotherapy for acute lymphoblastic leukemia: case report].

We report a case of primitive neuroectodermal tumor (PNET) arising 8 years after chemotherapy and radiotherapy for acute lymphoblastic leukemia. A 15-year-old boy with a history of acute lymphoblastic leukemia, at the age of 7, underwent chemotherapy and 14Gy of radiotherapy to the whole brain. He was admitted to our department due to the development of aphasia, right hemiparesis and generalized convulsive seizure. MRI showed an irregularly enhanced mass in the left frontal lobe. A gross total removal of the tumor was performed and histological examination showed it to be PNET. Postoperatively, the patient underwent 20Gy of radiotherapy to the whole brain and 42Gy of local radiotherapy. Follow-up MRI showed no evidence of recurrent tumor 4 months after the radiotherapy. This tumor was thought to be a secondary brain tumor arising in this survivor of childhood acute lymphoblastic leukemia and it is a rare complication of successful leukemia treatment.

Adolescent↗
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↗
PubMed1992

Chromosome 11p15 deletions in human malignant astrocytomas and primitive neuroectodermal tumors.

Chromosome 11p15 deletions occur frequently in several types of human cancer, both sporadic and familial, suggesting that a tumor suppressor gene is present within the deleted chromosome region. We carried out a restriction fragment length polymorphism analysis of chromosome 11p in two types of human brain tumors: malignant astrocytoma, the most common glial tumor in adults; and primitive neuroectodermal tumor (PNET), a malignant embryonic tumor that afflicts children. Loss of heterozygosity was found in 11/43 malignant astrocytomas (26%) and in 3/11 PNETs (27%). Deletion mapping revealed a region of loss on chromosome 11p (p15.4-pter) that was common to both tumor types. To determine whether the c-H-ras gene, located on chromosome 11p in the common region of deletion, was a candidate gene, we analyzed polymerase chain reaction products corresponding to all four c-H-ras coding exons for single-strand conformation polymorphisms. The absence of electrophoretic mobility shifts in tumor DNA compared to leukocyte DNA indicated that c-H-ras gene mutations were most likely not present. These results suggested that loss of a gene on chromosome 11p15 distinct from c-H-ras is an important step in tumorigenesis within the central nervous system in both children and adults.

Astrocytoma↗
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↗
PubMed1995

Isochromosome 17q demonstrated by interphase fluorescence in situ hybridization in primitive neuroectodermal tumors of the central nervous system.

We previously reported an i(17q) as a non-random finding in childhood primitive neuroectodermal tumors (PNETs) of the central nervous system. In the present study, we describe a two-color interphase fluorescence in situ hybridization (FISH) assay for detection of chromosome 17 abnormalities in tumors. Thirty-four PNETs were analyzed by FISH with a series of chromosome 17-specific probes which map to 17p13.3-17q25. The results from the FISH assay were then compared to the karyotypes prepared from the tumors. Ten of the 34 cases demonstrated an i(17q) by FISH and standard cytogenetics. Two PNETs were shown to have an i(17q) by FISH alone, and three additional tumors had deletions of 17p. Thus, a total of 15 of 34 (44%) of the PNETs in this series had a deletion of 17p. This study confirms and extends our previous reports that an i(17q) is the most common cytogenetic abnormality in PNETs. The interphase FISH assay which we employed will have clinical utility for diagnosis of children with malignant brain tumors, and it may be used for identification of tumors with 17p deletions for molecular studies aimed at identifying disease genes.

Adolescent↗