Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Neuroectodermal Tumors”

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 415 records · Page 23Linked to original sources
PubMed2005

High-dose chemotherapy with autologous stem cell rescue for children with high risk and recurrent medulloblastoma and supratentorial primitive neuroectodermal tumors.

Current treatment for high risk and recurrent medulloblastoma (MB) and supratentorial primitive neuroectodermal tumors (stPNET) has a very poor prognosis in children. High dose chemotherapy (HDCT) and autologous stem cell rescue have improved survival rates. We present 19 patients (thirteen classified in the high risk group and six patients with recurrent disease) that received HDCT and autologous stem cell rescue. In the high risk group [Med Pediatr Oncol 38 (2002) 83], all patients underwent neurosurgical debulking. Standard chemotherapy was prescribed in 10 patients. Radiotherapy was given to 4 patients (all older than 4 years old). In the recurrence disease group [Childs Nerv Syst 15 (1999) 498], five patients underwent surgery. Radiotherapy was given to those who were not previously irradiated. The HDCT in twelve patients consisted of busulfan 4 mg/kg/day, orally over 4 days in 6-hourly divided doses and melphalan at a dose of 140 mg/m2/day by intravenous infusion over 5 min on day -1. Three patients additionally received thiotepa 250 mg/m2/day intravenously over 2 days and four patients additionally received topotecan 2 mg/m2/day over 5 days by intravenous infusion over 30 min. The other seven patients received busulfan and thiotepa at the same doses.Patient's 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 re-infused 48 h after completion of chemotherapy. With a median follow-up of 34 months (range 5-93) eight complete responses and one partial response were observed. Three patients died of treatment-related toxicities (15%). The 2 year event-free survival was 37.67+/-14% in all patients and 57+/-15% for the high risk group. Therefore we conclude that HDCT may improve survival rates in patients with high risk/recurrent MB and stPNET despite treatment toxicity.

Adolescent↗
PubMed2004

[Supratentorial primitive neuroectodermal tumor: report of a surgical case].

We reported a rare case of supratentorial primitive neuroectodermal tumors (sPNET) and reviewed the literature. A 15-year-old girl presented with a one-month history of headache and vomiting. CT scans showed a huge, solid and cystic tumor with calcification, occupying the left anterior cranial fossa. The solid portion of the tumor was hypointense on T1-weighted images, slightly hyperintense on T2-weighted images, hyperintense on diffusion- weighted images, isointense on fluid-attenuated inversion recovery (FLAIR) images, and strongly enhanced after administration of contrast medium. The expansile tumor had a broad attachment to the dura matter of the anterior cranial fossa. The patient underwent an uneventful extirpation of the tumor. Microscopically, the solid tumor contained small, round poorly-differentiated cells with pleomorphic nuclei and brisk mitotic activity. The tumor cells were immunoreactive for synaptophysin and GFAP, whereas lack of MIC2 gene product expression was confirmed using the monoclonal antibody 12E7. The MIB-1-positive rate was 43%. The histological diagnosis was sPNET. Postoperatively, a dose of 30.6Gy was administrated to the whole brain and a boost of 19.6Gy to the T2-hyperintensity lesion. The patient successively received a dose of 30.4Gy to the whole spine and Linac stereotactic radiotherapy with a marginal dose of 16.8Gy at the tumor bed. Three months after radiotherapy the patient received chemotherapy using carboplatin and etoposide. Follow-up MR images showed no evidence of recurrent tumor 5 months after the radiochemotherapy. On the basis of MR findings on both diffusion-weighted and FLAIR images, preoperative diagnosis of sPNET may be important for choosing appropriate therapeutic strategies for this tumor.

Adolescent↗
PubMed1997

Three-dimensional reconstruction of melanotic neuroectodermal tumor of infancy involving the occipital bone and dura.

A rare case of melanotic neuroectodermal tumor of infancy involving the right occipital bone in an 11-month-old infant is described. The bone tumor and its dural extension were surgically removed. Three-dimensional reconstruction of the tumor and brain from neuroimaging data added worthwhile information for preoperative planning. Microscopic examination revealed melanotic and neuroblastic tissue in a fibrillary matrix involving the expanded bone and superficial dura. A focal osteoblastic cranial mass in an infant should alert the clinician to consider this tumor.

Brain↗
PubMed1998

Exophytic primitive neuroectodermal tumor of the spinal cord.

We present a case of an exophytic spinal primitive neuroectodermal tumor that, radiologically, simulated an extramedullary nerve sheath tumor, meningioma, or metastatic tumor deposit. MR imaging provided discrete anatomic localization of the tumor, enabling exclusion of multicentricity in the brain and spinal cord.

Adult↗
PubMedDate not supplied

Rectal primitive neuroectodermal tumor.

We report the clinical, histologic, immunohistochemical, and molecular findings of a Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) localized to the rectum in a 17-year-old boy. Notably the 4.5 x 4 x 4-cm sessile mass was spontaneously eliminated through the anus, producing an episode of hemorrhagic shock. Histologically the tumor presented as a proliferation of membrane CD99-positive, small round cells. EWS/FLI1 chimeric mRNA was demonstrated by nested reverse-transcription polymerase chain reaction (RT-PCR) analysis in the tumor tissue. The patient is alive and well 2 years after initial symptoms. We also review reported cases of ES/PNET of the digestive system.

12E7 Antigen↗
PubMed2001

Lipomatous primitive neuroectodermal tumor with a glioblastoma component: a case report.

A case of extracerebellar lipomatous primitive neuroectodermal tumor (PNET) with glioblastoma multiforme (GBM) areas is reported. A 44-year-old woman who had been on antipsychotic agents for schizophrenia complained of hemiparesis and drowsiness. She deteriorated progressively and died 3 months later. The autopsy revealed a huge, ill-defined tumor located from right basal ganglia to brain stem. Microscopically, the tumor consisted of three distinct components: clusters of small primitive cells consistent with PNET, mature lipoma-like islands, and a GBM-like component. Neuronal differentiation in PNET areas was confirmed by the presence of Homer Wright rosette, synaptophysin-positive fibrillary background, and ultrastructural demonstration of neuritic processes. Lipoma-like areas composed of lipidized cells containing large lipid droplets were intimately intermingled and closely related with PNET areas. Furthermore, GBM areas were, although predominantly located in the brain stem, often blended with the previous two components. This component was characterized by glial fibrillary acid protein immunoreactivity of atypical tumor cells and the presence of necrosis and endothelial proliferation. PNET areas with lipomatous differentiation in the present tumor may suggest the morphological and histogenetic similarity to liponeurocytoma, although the neuronal element in the former was anaplastic. The association with a GBM component makes the present tumor a unique, and, to our knowledge, previously unrecognized lesion.

Adult↗
PubMed2004

[A case of peripheral-type primitive neuroectodermal tumor arising in the dura mater at the frontal base].

A 7-year-old boy was admitted to our hospital because of headache and frequent vomiting. The patient was noted to have papilloedema and mild palsy of the right abducent nerve. Magnetic resonance image (MRI) revealed a large tumor in the frontal base with tumoral hemorrhage. Angiography showed the tumor was fed by anterior meningeal arteries. At surgery, the tumor was arising in the dura mater at the frontal base, and was removed totally. Histological examination showed the tumor to be composed of small cells with uniform round nuclei and minimal cytoplasm. Immunohistochemical studies were positive for MIC-2, NSE, C-KIT, vimentin, Class III-beta tublin and glycogen, but negative for NFP, synaptophysin, chromogranin A and GFAP. MIB-1 labeling index was 40-50%. The tumor was histologically confirmed to be peripheral-type primitive neuroectodermal tumor(pPNET). Following surgery, he underwent whole brain, whole spine and local radiation therapy(30 Gy in total respectively) and received two 5-day cycles of chemotherapy, consisting of intravenous administration of cisplatin 20 mg/m2/day, etoposide 60 mg/m2/day and IFOS 900 mg/m2/day. After these therapies, follow-up radiological examination showed there was no recurrence of the tumor for 24 months. Intracranial pPNET is rare. Ewing sarcoma and pPNET(ES/pPNET) is the designation given to a family of small round cell tumor arising in bone or soft tissues. Intracranial PNETs are devided into central nervous system PNET(cPNET) and pPNET. It is necessary that intracranial PNETs are divided into two types of PNETs because of different prognosis between these tumors. MIC-2 is a specific marker for pPNET/ES family and is useful in the differential diagnosis of these two types of tumors.

12E7 Antigen↗
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↗
PubMed2002

Primary Ewing's sarcoma/primitive neuroectodermal tumor of the kidney: a clinicopathologic and immunohistochemical analysis of 11 cases.

Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) is an extraordinarily rare primary tumor in the kidney and can be mistaken for a variety of other round cell tumors, including blastema-predominant Wilms' tumor (WT). Approximately 90% of ES/PNET have a specific t(11;22), which results in a chimeric EWS-FLI-1 protein. Immunohistochemistry for the carboxy-terminus of FLI-1 is sensitive and highly specific for the diagnosis of ES/PNET. WT-1, the WT-associated tumor suppressor gene, is overexpressed in WT but not in ES/PNET. No study has examined FLI-1 or WT-1 expression in renal ES/PNET. The clinicopathologic features of 11 renal ES/PNET were studied along with immunohistochemistry for cytokeratin, desmin, CD99, FLI-1, and WT-1. WT were also immunostained for CD99 (5 cases), FLI-1 (10 cases), and WT-1 (9 cases). The patients (6 men, 5 women) ranged from 18 to 49 years of age (mean, 34 yr). The mean tumor size was 11.8 +/- 3.8 cm (mean +/- standard deviation). Presenting symptoms included abdominal/flank pain and/or hematuria. Grossly, all tumors showed necrosis and hemorrhage, and 4 had cystic change. Microscopically, all tumors showed vaguely lobular growth, primitive round cells, and variable rosette formation. Epithelial, myogenous, or cartilaginous differentiation was not seen. Immunohistochemical results on the renal ES/PNET were cytokeratin (2/8 focal), desmin (0/9), CD99 (8/8), FLI-1 (5/8), and WT-1 (0/8). In comparison, the WT only rarely expressed CD99 (1/5) and did not express FLI-1 (0/10), but were usually WT-1-positive (7/9). Follow-up on 8 cases (mean, 28 mo; range, 6-64 mo) showed 4 lung and pleural metastases, 1 bone metastasis, liver metastasis, 2 local recurrences, and 5 deaths from disease (median time to death, 16.8 mo). No case had distant metastatic disease at presentation. Adjuvant therapy included chemotherapy (8 cases), radiation (3 cases), and bone marrow transplantation (1 case). Our study affirms a unique proclivity of renal ES/PNET for young adults and that it is a highly aggressive neoplasm, with rapid death in many cases, usually after the development of treatment-resistant lung metastases. These tumors must be distinguished from blastema-predominant WT and other primitive renal tumors that require different therapy. FLI-1 and WT-1 immunohistochemistry may be valuable in this differential diagnosis, given the known immunophenotypic overlap between ES/PNET and blastema-predominant WT with regard to CD99, cytokeratin, and desmin. The accurate distinction between these two entities has clear prognostic and therapeutic implications.

12E7 Antigen↗
PubMed2002

Primitive neuroectodermal tumors of the pancreas: a report of seven cases of a rare neoplasm.

Primitive neuroectodermal tumors (PNETs) have rarely been described in solid organs. We report a series of seven PNETs of the pancreas. The clinical, gross, microscopic, and immunohistochemical features of these seven PNETs of the pancreas are described, as are the genetic analyses in five cases. The patients ranged in age from 6 to 25 years (mean 18 years). Four of the patients were male. All of the patients presented with jaundice and/or abdominal pain. All of the tumors were located in the head of the pancreas, and they ranged in size from 3.5 to 9.0 cm. Light microscopy revealed the typical morphologic features of PNETs. By immunohistochemistry the neoplastic cells in all seven cases expressed O13 (CD99, p30/32MIC2). In five of six tested cases, the neoplastic cells also expressed cytokeratin. All of the tumors expressed neural-neuroendocrine markers. Two of the three cases examined ultrastructurally showed prominent epithelial features. There was cytogenetic or molecular genetic evidence of the t(11;22)(q24;q12) in four of five cases examined. Clinical follow-up was available in five cases. Two of the patients were alive with no evidence of disease at 33 and 43 months. One patient was alive with disease at 27 months. One patient died of postoperative complications. Another patient died of disease 4 years after diagnosis. PNET can sometimes arise as a primary neoplasm of the pancreas. Like PNETs arising in more conventional sites, pancreatic PNETs occur in the pediatric and adolescent population, show the characteristic staining with O13, and typically harbor the t(11;22)(q24;q12) chromosomal translocation. PNETs should be included in the differential diagnosis of poorly differentiated small round cell tumors of the pancreas. Moreover, they should not be confused with pancreatic endocrine tumors, which also demonstrate dual epithelial and neuroendocrine differentiation by immunohistochemistry and express O13 in 30% of cases.

12E7 Antigen↗
PubMed1998

[Primitive neuroectodermal tumor of bone and soft tissue].

OBJECTIVE: To study the pathological characteristics of primitive neuroectodermal tumor (PNET) of bone and soft tissue. METHOD: Five patients with bone and soft tissue of PNET (2 men and 3 women) were studied by light microscope and immunohistochemistry. The age of the patients was 18, 18, 43, 14, 32 years and the primary sites of involvement were the ilium (n = 2), axillary fossa (n = 1), condyle of femur (n = 1), and diploe (n = 1). X-ray films showed bone osteolytic destruction, and no periosteal reaction or calcification. Surgery was performed in the patients. RESULT: All patients received combined chemotherapy and 3 received radiotherapy. Three patients died of metastatic tumor of lung 20, 13, and 5 months after invasion. 1 patient metastatic tumor of lung 20 months after invasion, and 1 patient bone extensive metastasis 1.5 months after surgery. CONCLUSION: The treatment of PNET of bone and soft tissue awaits further study.

Adolescent↗
PubMed1999

[Intracranial primitive neuroectodermal tumor in an infant: a case report].

A 2-month-old girl with a supratentorial primitive neuroectodermal tumor (PNET), which extended into the skull, is herein presented. The patient underwent total removal of the tumor and also received a course of postoperative chemotherapy. After a follow-up period of 12 months, the infant is alive without recurrence. Histologically, the tumor was composed of poorly differentiated neuroectodermal cells, and these neoplastic cells showed a mild immunohistochemical reaction for GFAP and synaptophysin, and a moderate reactivity for neuron specific enolase and vimentin. In addition, a moderate level of immunoreactivity for HBA71 antigen (p30/32M1C2), which is the product of the M1C2 gene and is found in peripheral PNETs but not in central PNETs, was noted in many neoplastic cells. Although this tumor was located intracranially, it may be classified as a peripheral PNET.

Antineoplastic Combined Chemotherapy Protocols↗
PubMedDate not supplied

Coexistence of cerebellar primitive neuroectodermal tumor and cerebellar dysplasia: case report.

It has been speculated that the cerebellar primitive neuroectodermal tumor (PNET) in part recapitulates stages in the maturation of normal human neuroblasts. One theory suggests that these tumors may arise from "primitive cells" or "remnants" of the external granular layer of the cerebellum, which forms a subpial, proliferative zone that gives rise to neurons of the internal granular layer and stellate and basket cells. In the present report, the coexistence of marked cerebellar cortical disorganization and cerebellar PNET is described in a 1-year-old boy. The abnormal dysplastic cortex was in close proximity as well as contiguous to the tumor. Although minor degrees of cerebellar dysplasia may be found incidentally, the coexistence of a severe malformative process contiguous to cerebellar PNET merits the consideration of a possible pathogenetic association between aberrant neuronal migration or maturation and the development of PNET in this patient.

Cerebellar Ataxia↗
PubMed1996

Malignant peripheral neuroectodermal tumor in an infant with neurofibromatosis type 1.

A case of multifocal malignant peripheral neuroectodermal tumor (PNET) arising from a plexiform neurofibroma in a 4-month-old Chinese boy with neurofibromatosis type 1 (NF-1) is described. Cytogenetic culture demonstrated hypotriploid karyotype with an abnormal clone characterized by 59-60, XY, +2, +3, +6, +8, +8, +12, +i(13)(q10), +der(14)t(1;14)(q21;q32), +16, +19, +20, +mar[cp3] with no apparent abnormality of chromosome 17. The child was treated with combination chemotherapy comprising ifosphamide, vincristine and doxorubicin. Despite initial partial response the child finally died of tumor progression and pulmonary metastases 8 months after diagnosis. We believe this is the first reported case of PNET in a child with NF-1 and may support an association between these two disorders of neural crest origin.

Fatal Outcome↗
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↗
PubMed2004

Primary Ewing's sarcoma-primitive neuroectodermal tumor of the vagina.

BACKGROUND: Only three cases of Ewing's sarcoma-primitive neuroectodermal tumor (ES-PNET) occurring in the vagina have been previously reported. CASE: A 30-year-old Chinese woman presented with a vaginal mass measuring 5 cm in greatest dimension. The tumor was composed of solid sheets of undifferentiated small round cells with numerous Homer-Wright rosettes. Immunohistochemically, it was positive for CD99, FlI-1 protein, synaptophysin, neuron-specific enolase, vimentin, and S-100 protein. The patient underwent wide local excision of the tumor and subsequent total abdominal hysterectomy with postoperative chemotherapy and radiotherapy. She is disease-free 36 months after the initial surgery. CONCLUSION: This is a rare case of ES-PNET occurring in the vagina and the first showing many distinctive Homer-Wright rosettes. A review of literature indicates that vaginal or vulvar ES-PNET affects younger women, and it may have a relatively favorable outcome.

Adult↗
PubMed2003

Primary intraspinal primitive neuroectodermal tumor: report of two cases and review of the literature.

BACKGROUND: Primary intraspinal primitive neuroectodermal tumor (PNET) is a very rare tumor entity. The optimal therapeutic approach is not known yet. We report on two women with primary intraspinal PNETs and review the literature. We describe the typical course of the disease, compare our patients to the other 17 cases reported until today, and discuss therapeutic options. PATIENTS AND METHOD: Case A: In a 49-year-old woman with an intraspinal PNET at L2, laminectomy and a gross tumor removal was accomplished. Postoperative radiation was performed from T12 to L3 to a dose of 50.4 Gy. Subsequently she was treated with chemotherapy containing vincristine, cisplatinum and lomustine. Case B: A 29-year-old woman presented with intramedullary PNET lesions at T1-3 and T10-11. Due to the multifocal location, she received a primary craniospinal axis irradiation to a dose of 35.2 Gy plus a boost to the tumor region to a total dose of 53.2 Gy. RESULTS: Both patients developed multilocular intraspinal relapses with meningeosis neoplastica 17 and 6 months from radiation therapy and underwent palliative chemotherapy. Case A died 23 months, case B 17 months after primary diagnosis. CONCLUSION: Despite modern treatment with microsurgery, irradiation and chemotherapy in primary intraspinal PNETs, local relapse or dissemination in most cases lead to death within a few months. An improvement of treatment outcome can only be achieved by intensification through multidisciplinary treatment.

Adult↗
PubMed1991

Preferential expression of alpha B-crystallin in astrocytic elements of neuroectodermal tumors.

Recently the authors have identified a major component of Rosenthal fibers as alpha B-crystallin, a major lens protein. In the current study the authors investigated the expression of alpha B-crystallin in four cultured glioma cell lines and in 115 human neuroectodermal tumors. alpha B-crystallin was expressed differentially by those glioma cell lines, but not by neuroblastoma cell lines. Northern blot analysis revealed two distinct messages for alpha B-crystallin in C-6, whereas only a single message in U-373MG and G26-24. In human surgical specimens positive immunostaining was frequently observed in the following brain tumors: pilocytic astrocytoma of the juvenile type, anaplastic astrocytoma, glioblastoma multiforme, and subependymal giant cell astrocytoma. The astrocytic elements of mixed oligoastrocytomas, glioblastomas with sarcomatous components, and gangliogliomas were likewise strongly stained. In contrast, little immunoreactivity was observed in ependymal and choroid plexus tumors. Thus, alpha B-crystallin is mainly expressed by astrocytic tumors among neuroectodermal neoplasms, without regard to the presence of Rosenthal fibers.

Blotting, Northern↗