Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Neuroectodermal Tumors, Primitive”

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 559 records · Page 31Linked to original sources
PubMed2008

Brainstem primitive neuroectodermal tumors (bstPNET): results of treatment with intensive induction chemotherapy followed by consolidative chemotherapy with autologous hematopoietic cell rescue.

We have evaluated the response rate and survival utilizing intensified chemotherapy followed by myeloablative chemotherapy with autologous hematopoietic cell rescue (AuHCR) and adjuvant radiation therapy in six young children with newly diagnosed brainstem primitive neuroectodermal tumors (bstPNET). Following maximum surgical resection of the tumor, patients received high dose induction chemotherapy including vincristine, cisplatin, cyclophosphamide, and etoposide. Eligible patients received a single cycle of myeloablative chemotherapy followed by AuHCR. Two patients survive at least 32 months with stable disease. This approach provides an alternative for young patients with bstPNET who in prior reports have had a uniformly fatal prognosis.

Antineoplastic Combined Chemotherapy Protocols↗
PubMedDate not supplied

Role of MAP kinase pathways in primitive neuroectodermal tumors.

Mitogen-activated protein kinase and Phosphatidylinositol-3 kinase/Akt-mediated signaling pathways play a major role in controlling cell proliferation, differentiation and cell death. Phosphorylation and dephosphorylation of their specific Thr/Tyr residues is critical in determining their activity. We determined the expression pattern and activity of MAP kinases and Akt in Primitive Neuroectodermal Tumors (PNETs). The kinase activity of extracellular signal-regulated kinase (ERK) was higher in both primary tumors and cell lines, as evident from the increased phosphorylation of ERK1 and ERK2. We did not observe the activation of C-jun N-terminal kinase (JNK) or p38 MAPK The expression of Raf-1, a kinase acting upstream of ERK, was significantly increased in primary tumors compared to normal brain. The PI-3 kinase-activated phosphorylation of Akt was also higher in primary tumors. These results suggest that activation of the Raf-1/ERK module of the MAP kinase pathway play an important role in PNETs.

Brain Neoplasms↗
PubMed1995

Transplantation of congenital primitive neuroectodermal tumor of fetus to the uterus of mother: application of biotin-labeled chromosome-specific probes.

We report a 38-year-old gravida 3, para 3, white female with an unremarkable history and normal amniocentesis, who delivered a male infant with a large pedunculated and ulcerated tumor by vaginal delivery. A portion of the tumor was sheared and expelled with the normal placenta at the time of delivery. The biopsy of the infant mass and the expelled tissue showed histologic features of a small round cell tumor with immunohistochemical features of a primitive neuroectodermal tumor (PNET). The infant eventually died at the age of 4 weeks as a result of extensive dissemination of tumor. The mother had postpartum bleeding, and fractional dilation and curettage procedures at 6 weeks and 4 months after delivery revealed tumor similar to that of the infant. The mother underwent radical hysterectomy and bilateral salpingo-oophorectomy, which revealed a neoplasm at the junction of the lower uterine segment and cervix. Fluorescent in situ hybridization (FISH) study of both the infant's tumor and the mother's uterine tumor showed positivity for the Y chromosome by using a classical alpha-satellite Y chromosome-specific probe. These findings support the hypothesis that the tumor was transferred from the fetus to the mother.

Journal Article↗
PubMed1989

Monoclonal antibody MB2: a potential marker for Ewing's sarcoma and primitive neuroectodermal tumor.

The small round-cell tumors of childhood present difficulties in diagnosis when differentiation is not apparent. Immunohistochemistry is helpful; however, the only antigen consistently detected in Ewing's sarcoma is vimentin, which may also be detected in the other types of small-cell neoplasms. The monoclonal antibody (MAb) MB2 is marketed as a B-lymphocyte marker that can be used on paraffin-embedded tissue. To determine its specificity, we performed immunohistochemical staining on pediatric tumors with MB2. These included 55 cases of small round-cell tumors (lymphomas, Ewing's sarcoma, peripheral primitive neuroectodermal tumors [PNET], neuroblastomas, rhabdomyosarcomas, and nephroblastomas). MB2 positivity was detected in all B-cell lymphomas and in seven of nine cases of Ewing's sarcoma and three PNET. In neuroblastomas only differentiating ganglion cells were positive. In rhabdomyosarcomas only large rhabdomyoblasts were positive. Blastema of nephroblastomas was negative. Thus, in cases of poorly differentiated small round-cell tumors, MB2 was positive in all B-cell lymphomas, most Ewing's sarcomas and all cases of PNET. Lymphomas were distinguished by staining for leukocyte-common antigen and PNET by neuron-specific enolase. Therefore, the addition of MB2 to a discrete panel of antibodies may prove useful in the diagnosis of Ewing's sarcoma and PNET.

Adolescent↗
PubMed2004

Second malignant neoplasms in childhood acute lymphoblastic leukemia: primitive neuroectodermal tumor of the chest wall with germline p53 mutation as a second malignant neoplasm.

About 80% of children treated for acute lymphoblastic leukemia (ALL) will be long-term survivors. Second malignant neoplasm (SMNs) are a devastating sequelae observed on these children, with an estimated cumulative risk of 2-3.3% fifteen years after diagnosis. Primitive neuroectodermal tumor of bone (PNET) is rarely observed as a SMN following treatment of childhood ALL. The authors described the occurrence of a chest wall PNET of the bone at the site of a central line placement associated with both germ-line and tumor cell p53 mutation in a 8-year-old boy 1 year after completing therapy for standard risk ALL. A review of the literature of 25,051 children treated for ALL discovered 230 SMNs (0.99%), and only one case of PNET of the bone was noted among this group. The occurrence of a SMN in children treated for ALL is a rare event. Such an occurrence, in particular the development of an unusual SMN, should be evaluated for a germline p53 mutation.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2001

Primary Ewing's sarcoma/peripheral primitive neuroectodermal tumor at the vertex of the skull with elevated serum carcinoembryonic antigen: case report.

A primary Ewing's sarcoma arising in the skull is relatively rare. Although a small number of case reports noted elevated carcinoembryonic antigen (CEA) in patients with primary central nervous system (CNS) neoplasms, there is no report of Ewing's sarcoma/peripheral primitive neuroectodermal tumor (PNET) with elevated serum levels of CEA. A 7-year-old boy who had episodes of headache and vomiting had noticed a solid mass in the vertex of the head. Imaging studies revealed a large intra- and extracranial tumor at the vertex of the skull. Hematological examination demonstrated high serum levels of CEA: 91.09 ng/ml. The patient initially underwent an embolization of the bilateral middle meningeal arteries with Gelfoam particles. One week later, the patient was operated on and a subtotal resection of the tumor was performed. On histopathological and molecular genetic examination, the tumor was diagnosed as a Ewing's sarcoma/peripheral PNET. Immunohistochemical study showed strongly positive staining for CEA in the tumor cells. The serum level of CEA was normalized at 0.83 ng/ml after the tumor was removed and the boy underwent radiotherapy and 3 courses of chemotherapy. This is the first reported case of a primary Ewing's sarcoma/peripheral PNET at the vertex of the skull with elevated serum CEA.

Carcinoembryonic Antigen↗
PubMed1997

[Primitive neuroectodermal tumors of the minor pelvis in women--case report].

A primitive neuroectodermal tumour (PNET) of the minor pelvis is a rare malignant small-cell tumour developing from the neural groove. It metastatizes into the lungs, bones, liver and brain. Treatment involves radical surgical extirpation followed by chemotherapy and actinotherapy. The author presents the case-history of PNET of the pelvis minor in a 33-year-old woman.

Adult↗
PubMed2001

Feasibility of four consecutive high-dose chemotherapy cycles with stem-cell rescue for patients with newly diagnosed medulloblastoma or supratentorial primitive neuroectodermal tumor after craniospinal radiotherapy: results of a collaborative study.

PURPOSE: This study was designed to determine the feasibility and safety of delivering four consecutive cycles of high-dose cyclophosphamide, cisplatin, and vincristine, each followed by stem-cell rescue, every 4 weeks, after completion of risk-adapted craniospinal irradiation to children with newly diagnosed medulloblastoma or supratentorial primitive neuroectodermal tumor (PNET). PATIENTS AND METHODS: Fifty-three patients, 19 with high-risk disease and 34 with average-risk disease, were enrolled onto this study. After surgical resection, high-risk patients were treated with topotecan in a 6-week phase II window followed by craniospinal radiation therapy and four cycles of high-dose cyclophosphamide (4,000 mg/m2 per cycle), with cisplatin (75 mg/m2 per cycle), and vincristine (two 1.5-mg/m2 doses per cycle). Support with peripheral blood stem cells or bone marrow and with granulocyte colony-stimulating factor was administered after each cycle of high-dose chemotherapy. Treatment of average-risk patients consisted of surgical resection and craniospinal irradiation, followed by the same chemotherapy given to patients with high-risk disease. The expected duration of the chemotherapy was 16 weeks, with a cumulative cyclophosphamide dose of 16,000 mg/m2 and a planned dose-intensity of 1,000 mg/m2/wk. RESULTS: Fifty of the 53 patients commenced high-dose chemotherapy, and 49 patients completed all four cycles. The median length of chemotherapy cycles one through four was 28, 27, 29, and 28 days, respectively. Engraftment occurred at a median of 14 to 15 days after infusion of stem cells or autologous bone marrow. The intended dose-intensity of cyclophosphamide was 1,000 mg/m2/wk; the median delivered dose-intensity was 1,014, 1,023, 974, and 991 mg/m2/wk for cycles 1 through 4, respectively; associated median relative dose-intensity was 101%, 102%, 97%, and 99%. No deaths were attributable to the toxic effects of high-dose chemotherapy. Early outcome analysis indicates a 2-year progression-free survival of 93.6% +/- 4.7% for the average-risk patients. For the high-risk patients, the 2-year progression-free survival is 73.7% +/- 10.5% from the start of therapy and 84.2% +/- 8.6% from the start of radiation therapy. CONCLUSION: Administering four consecutive cycles of high-dose chemotherapy with stem-cell support after surgical resection and craniospinal irradiation is feasible in newly diagnosed patients with medulloblastoma/supratentorial PNET with aggressive supportive care. The early outcome results of this approach are very encouraging.

Adolescent↗
PubMedDate not supplied

Ultrastructural study of nuclear evagination in neoplastic cells of a cerebral primitive neuroectodermal tumor.

Evaginations of the nuclear envelope containing nuclear material were observed in neoplastic cells of a cerebral primitive neuroectodermal tumor. The possible sequential development of nuclear evaginations included: 1) formation of a segmental naked inner membrane of the nuclear envelope due to loss of the underlying marginal heterochromatin, 2) elevation of the overlying outer nuclear membrane with focal expansion of the perinuclear cistern, 3) outpouching of the inner nuclear membrane forming a small bleb, and 4) protrusion of electron-lucent nuclear material into the bleb forming a nuclear evagination. Some nuclear evaginations were isolated in the cytoplasm and some protruded into the intercellular space. The inner nuclear membrane of the nuclear evagination was devoid of the marginal heterochromatin. Nuclear pores and rupture of membranes with release of the contents of nuclear evaginations into the cytoplasm were not observed. Instead, the nuclear evaginations isolated from the cytoplasm often exhibited degenerative changes with disintegration of their contents and inner nuclear membrane. It is suggested that the nuclear evaginations of the neoplastic cells in the present study may represent a means to eliminate excess nuclear material.

Brain Neoplasms↗
PubMed2004

Primary subcutaneous primitive neuroectodermal tumor with aggressive behavior and an unusual karyotype: case report.

Primitive neuroectodermal tumor/Ewing sarcoma (PNET/ES) rarely occurs in the skin and subcutaneous tissues. We present a case of a 16-year-old girl with primary cutaneous and subcutaneous PNET/ES of the abdominal wall. Despite wide local excision and chemotherapy, she rapidly developed cranial bone and brain metastases, followed by lung and skeletal metastases, and died shortly thereafter. The recurrent tumor exhibited light microscopic features of a small, round, blue cell tumor with intracytoplasmic glycogen. Immunohistochemical analysis showed positivity for CD99, CD56, S100, and glial fibrillary acid protein, and ultrastructural features included cytoplasmic glycogen and focal complex interdigitating synaptic junction-like cytoplasmic folds. Cytogenetic analysis of the relapsed tumor showed a complex karyotype: 47,XX,i(1)(q10), der(4)t(4;19) (q33 approximately q35;q13.1), + 8,t(15;17)(q24;p11.2 approximately p12),der(19)t (19;20)(q13.1;p11.2),der(22)t(20;22)(q13;q13). Cytogenetic, interphase fluorescence in situ hybridization, and molecular genetic analyses failed to show t(11:22) (q24;q12) or abnormalities of chromosome region 22q12. The clinical behavior and atypical and complex cytogenetic abnormalities exhibited by the tumor in this patient are unusual and represent the most aggressive end of the clinical spectrum of cutaneous and subcutaneous PNET/ES.

Adolescent↗
PubMed1986

[Clinicopathological study of supratentorial tumors with multipotential differentiation in childhood. Primitive neuroectodermal tumor].

Three cases of supratentorial tumor in childhood were studied clinico-pathologically in an attempt to clarify its histological character. Case 1: A 3-year-old boy. Carotid angiogram revealed avascular lesion in the left parietal lobe. Twice operations and radiotherapy were performed. Ten months after the second operation, he died. Surgical specimen at the first operation was composed mainly of round tumor cells. The tumor tissue contained many collagen fibers. At the periphery of this tissue, medulloblastomatous areas consisting of closely aggregated hyperchromatic small round cells were found. There were perivascular rosettes and Homer Wright rosettes. In part, tubular and papillary arrangement of cells was also present. Astrocytomatous and oligodendrogliomatous structures were also present. Surgical specimens at the second operation showed the predominance of sarcomatous areas consisting of spindle-shaped cells with abundant argyrophilic fibers. Case 2: A 10-year-old girl. CT scan revealed a heterogeneous enhanced mass with a cyst and calcification in the right parietal lobe. Operation and radiotherapy were performed. Twelve months after operation, she is still alive. Most of the surgical specimens showed sarcomatous structure with abundant argyrophilic fibers. In these tissues, there were medulloblastomatous and ependymomatous features. Papillary arrangement of cells was also present. In part, there was oligodendrogliomatous structure. Case 3: A 2-year-old girl. CT scan revealed a heterogeneous enhanced mass in the right frontal lobe. The tumor tissue was composed of an aggregation of undifferentiated small round cells with Homer Wright rosettes.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain Neoplasms↗
PubMed1993

Neoplastic meningitis as the presentation of occult primitive neuroectodermal tumors.

Seven children and young adults initially presented with subacute meningitis and/or increased intracranial pressure. The diagnosis of neoplastic meningitis secondary to a primitive neuroectodermal neoplasm was delayed by the absence of an obvious primary tumor. The neuroradiologic appearance was that of a basimeningeal infiltrative process, complicated by communicating hydrocephalus or "pseudotumor cerebri." Myelography was important in the diagnosis of disseminated meningeal malignancy in four cases. Cerebrospinal fluid cytologic diagnosis was insensitive but ultimately confirmed in five cases. All seven patients experienced progressive disease despite neuraxis radiotherapy and intensive chemotherapy; six have died. Systemic dissemination to bone and/or peritoneum occurred in three patients while on therapy. In two, a primary parenchymal brain or spinal cord tumor could not be identified at postmortem examination. The presentation of a primitive neuroectodermal tumor as subacute meningitis without an evident primary tumor heralds an aggressive and refractory neoplasm.

Adolescent↗
PubMed1999

Gangliosides and neutral glycolipids in ependymal, neuronal and primitive neuroectodermal tumors.

Neutral glycolipid and ganglioside compositions were determined on 11 ependymal tumors, 12 medulloblastomas, 6 other neuronal tumors of the brain, 4 peripheral neuroblastomas, 1 cerebral primitive neuroectodermal tumor (PNET), and 1 PNET of the thoracic wall. Within the group of tumors that can demonstrate neuronal phenotypes, there was an association between the degree of neuronal differentiation usually demonstrated by these tumors and the proportions of both GD1a and 1b-pathway gangliosides. The amount of globoside also correlated with the amount of 1b pathway gangliosides. Patients with medulloblastomas whose 1b gangliosides made up over 15% of the total gangliosides survived longer that those with lower proportions of 1b gangliosides. The only gangliosides in the choroid plexus papilloma were GM3 and GD1a, but other ependymal tumors had significant amounts of GD1b and its metabolic precursors. Ependymoma and anaplastic ependymoma had similar neutral glycolipid compositions, which were different from subependymoma, which lacked ceramide monohexoside and ceramide dihexoside. These differences in glycolipid compositions suggest that there may be fundamental biological differences between these types of ependymal tumors.

Adult↗
PubMed1997

p21 (WAF1/Cip1/Sdi1/Pic1) mRNA is expressed in neuroblastoma cell lines but not in Ewing's sarcoma and primitive neuroectodermal tumor cell lines.

The p21 protein inhibits the activity of cyclin-Cdk complexes and suppresses cell cycle progression. Wild type p53 can induce p21, but mutated p53 cannot. Previous studies have demonstrated that mutation of p53 is absent in neuroblastoma (NB). These reports prompted us to examine whether p53 induced p21 in NB. We examined the expression of p21 and p53 mRNA in eight NB, two Ewing's sarcoma (ES) and two primitive neuroectodermal tumor (PNET) cell lines by Northern blot analysis, and sequenced p53 cDNA of these cells. Although p53 mRNA was detected in all analyzed cell lines by Northern blot analysis, p21 mRNA was detected in six NB but not in two NB, two ES and two PNET cell lines. We detected the point mutation of p53 at codon 273 (CGT to TGT) in one NB and two ES cell lines. The non-transforming substitution at codon 72 (CCC to CGC) was detected in all analyzed cell lines. One PNET cell line had a large deletion of p53 cDNA. These results showed that p21 mRNA was usually expressed in NB but not in ES and PNET. This may suggest that the down stream of the p53 signal transduction pathway in NB is different from that of the closely related tumors of ES and PNET.

Blotting, Northern↗
PubMed2002

Role of radiotherapy in the treatment of supratentorial primitive neuroectodermal tumors in childhood: results of the prospective German brain tumor trials HIT 88/89 and 91.

PURPOSE: To evaluate the outcome of children with supratentorial primitive neuroectodermal tumors after surgery, irradiation, and chemotherapy and to identify factors predictive for survival. PATIENTS AND METHODS: Sixty-three children in the prospective trials HIT 88/89 and HIT 91 were eligible. Complete resection was performed in 21 patients. Patients were randomized for preirradiation chemotherapy, consisting of two cycles of ifosfamide, etoposide, methotrexate, cisplatin, and cytarabine (n = 40), or chemotherapy after irradiation, consisting of eight cycles with cisplatin, vincristine, and lomustine (n = 23). Irradiation volume was recommended to encompass the neuraxis with 35.2-Gy total dose followed by a boost (20.0 Gy) to the primary tumor site (n = 54). Seven patients were irradiated to the tumor region only with a total dose of 54.0 Gy. RESULTS: Overall survival at 3 years was 48.4%. Progression occurred in 38 children, with local recurrences in 27 patients. The only significant prognostic factor was dose and volume of radiotherapy (progression-free survival after 3 years was 49.3% with correct treatment compared with 6.7% for 15 children with major violations of radiotherapy). Ten early progressions occurred during adjuvant therapy (eight before and two during radiotherapy), nine of them treated with preirradiation chemotherapy. There was a positive trend in outcome for nonmetastatic and pineal tumors. CONCLUSION: Significant predictive factors were dose and volume of radiotherapy. Volume of irradiation should encompass the whole CNS with additional boost to the tumor region. Local doses of at least 54 Gy and a craniospinal dose of 35 Gy are necessary. Preirradiation chemotherapy seems to increase risk of early progression.

Adolescent↗
PubMed1990

Overexpression of the pseudoautosomal gene MIC2 in Ewing's sarcoma and peripheral primitive neuroectodermal tumor.

Ewing's Sarcoma (ES), the second most frequent bone tumor in childhood and adolescence, and the probably closely related peripheral primitive neuroectodermal tumor (pPNET) share a unique cytogenetic translocation between chromosomes 11 and 22. Both of them expose high amounts of a glycoprotein on their cell surface, which can be specifically detected by the mAb HBA-71. The cDNA coding for the HBA-71 antigen was isolated by screening a cDNA expression library constructed from a pPNET-derived cell line. Nucleotide sequencing revealed the HBA-71 antigen to be the product of the pseudoautosomal gene MIC2 previously identified by the mAb 12E7 in haematopoietic cells. This antigen is a glycoprotein with a molecular weight of about 29,000 and is expressed in low amounts in most human cell lines and probably normal tissues and tumors with only a few exceptions. In T-cells the antigen is involved in cell adhesion processes. In ES- and pPNET-derived cell lines MIC2 expression is significantly enhanced. No gross changes in posttranslational modification could be observed. The high expression results in easy and specific detection of the antigen in immunocytochemical analysis of paraffin embedded tissue sections making HBA-71 a useful tool in tumor diagnosis.

12E7 Antigen↗
PubMed2001

A case of canine primitive neuroectodermal tumor (PNET).

A 2-year and 6 month-old, female, Golden Retriever showed circling behavior and seizure. By magnetic resonance imaging (MRI) examination, a mass was found on the surface of the left cerebral hemisphere, invading to the left temporal muscle. The skull bone between them was destroyed. The dog was euthanized and necropsied. Histologically, the mass contained a lot of undifferentiated anaplastic cells, forming Homer-Wright rosettes and pseudopalisading patterns. Thus, the case was diagnosed as primitive neuroectodermal tumor (PNET).

Animals↗