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At least 127 records · Page 7Linked to original sources
PubMed2005

Melanotic neuroectodermal tumor in a newborn.

Melanotic neuroectodermal tumor of infancy (MNTI) is an uncommon, fast-growing, pigmented neoplasm of neural crest origin; it primarily affects the maxilla of the infants during the first year of life. Approximately 200 instances of this tumor are reported in the medical literature. We present a case of newborn with MNTI on the left maxillary alveolar ridge. The patient underwent complete surgical resection without maxillectomy. There has been no recurrence in a follow-up period of 1 year. The diagnostic features and management alternatives of MNTI are discussed.

Alveolar Process↗
PubMed1999

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↗
PubMed2003

Findings and clinical course of a localized primitive peripheral neuroectodermal tumor of the kidney.

Primitive neuroectodermal tumor (PNET) is a very aggressive neoplasm, considered to have an unfavorable prognosis, and renal location is extremely rare. We present the findings and clinical course of a patient with localized disease who is free of disease more than 3 years after first presentation. In suspicious cases we recommend to surgically explore renal masses as early as possible.

Adolescent↗
PubMed2000

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↗
PubMed2001

Ewing's sarcoma/primitive neuroectodermal tumor of the chest wall.

Ewing's sarcoma/primitive neuroectodermal tumor is the most common tumor of the chest wall in children and adolescents. It is extremely malignant with a high frequency of both metastatic spread and of local recurrence. Cure requires intensive therapy to control both distant and local disease. Surgery and high-dose radiotherapy can achieve equivalent local control; however, radiation is associated with the additional morbidities of second malignancy and a significant adverse impact on both cardiac and pulmonary function. The optimal therapeutic sequence is initial biopsy followed by induction chemotherapy with subsequent resection of the primary tumor. This approach will achieve the lowest incidence of tumor present at the margins of resection and, hence, need for postoperative radiotherapy. The chest wall is a rare site for tumors in children and adolescents. In a series reported from St Jude's Children's Research Hospital, chest wall tumors constituted only 1.8% of the solid childhood tumors. They are primarily mesenchymal in origin and the Ewing's sarcoma/primitive neuroectodermal tumors (PNET) predominate. This report concentrates on the later tumors. They are recognized to be extremely malignant, and cure in those who present with metastatic disease is very difficult to achieve. Recent advances in our understanding of their cytogenetic basis and optimal treatment are presented.

Chemotherapy, Adjuvant↗
PubMed1995

Dynamics of cell proliferation and cell death during the emergence of primitive neuroectodermal tumors of the immature central nervous system in transgenic mice.

Cell proliferation and cell death play critical roles in embryonic development, postnatal tissue maintenance, and tumor formation. To understand the interplay between cell proliferation and death in tumor formation, we studied these two processes in nascent primitive neuroectodermal tumors that arose postnatally from neuroepithelial cells ventral to the median eminence of transgenic mice (designated rTH-Tag mice) carrying a Simian virus 40 large T antigen transgene driven by a rat tyrosine hydroxylase promoter. Cell proliferation continued in the neuroepithelium of the ventral median eminence in wild-type and transgenic animals for the first 2 weeks of postnatal life but subsided completely in the wild-type mice after 2 weeks of age. In contrast, mitotic activity persisted in these progenitor cells of the rTH-Tag mice, and there was a dramatic increase in mitotic activity after 10 weeks leading to the formation of primitive neuroectodermal tumors despite sustained cell death activity. We conclude that primitive neuroectodermal tumors originate from progenitor cells in the ventral median eminence of rTH-Tag mice in early postnatal life when progenitors fail to respond to signals to exit the cell cycle. Thus, the disruption of mechanisms that regulate cell proliferation and cell death in the developing brain may underlie the emergence of primitive neuroectodermal tumors in the rTH-Tag mice.

Animals↗
PubMed1995

Medulloblastoma/primitive neuroectodermal tumor in 45 adults.

Medulloblastoma/primitive neuroectodermal tumor (PNET) is an uncommon tumor in adults. We reviewed the medical records of 45 patients, 15 years or older, with medulloblastoma/PNET. Most patients presented with symptoms referable to the posterior fossa, and 31 of 45 patients had disease limited to the posterior fossa at the time of diagnosis. Despite initial favorable response to surgical resection, radiation, and chemotherapy, one-half had recurrence 10 to 76 months after initial treatment. Only two of these patients had local recurrence; the remainder had CNS dissemination, systemic metastasis, or both. The recommended approach to medulloblastoma/PNET in adults is similar to that in children, and includes initial staging evaluation, systemic and focal therapy (ie, neuraxis irradiation with posterior fossa boost and chemotherapy), and long-term follow-up to detect late and distant recurrence.

Adolescent↗
PubMed1999

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↗
PubMedDate not supplied

[Primitive neuroectodermal tumor of the kidney in children]

OBJECTIVES: To characterize primitive neuroectodermal tumor of the kidney as a differential diagnosis for Wilms tumor, and to emphasize the severity of the disease. METHODS: We report the case of a patient with a diagnosis of primitive neuroectodermal tumor of the kidney who underwent nephrectomy and complete tumor resection combined with chemotherapy. RESULTS: Initially, the response of the patient to treatment was favorable, with a few events associated with agranulocytosis resulting from the chemotherapy. Ten months after of the end of treatment, the tumor recurred in the paraspinal cervical region. CONCLUSIONS: Primitive neuroectodermal tumors involving the kidney are very rare and extremely aggressive. In such cases, the pathologist has a major role in establishing a final diagnosis through histological and immunohistochemical methods.

Journal Article↗
PubMed2006

Primitive neuroectodermal tumor of the adrenal gland.

We report a rare case of primitive neuroectodermal tumor arising from adrenal gland in adulthood, diagnosed preoperatively as having non-functional adrenocortical adenoma. Laparoscopic adrenalectomy was performed. Immunohistological examination revealed the definite diagnosis as primitive neuroectodermal tumor of the adrenal gland. Although primitive neuroectodermal tumor is a highly malignant neoplasm, there is no evidence of local recurrence and distant metastasis 16 months after surgery.

12E7 Antigen↗
PubMed2003

Peripheral primitive neuroectodermal tumor after radiotherapy.

A 41-year-old man had a peripheral neuroectodermal tumor develop at the distal third of the fibula 4 years after radiotherapy for relapsed villonodular synovitis. This type of sarcoma usually is classified into the heterogeneic group of small round-cell bone tumors as a subdivision of Ewing's sarcomas. The immuno-staining positivity of the neoplastic cells for the neuron-specific enolase allowed the authors to make the diagnosis of a tumor with neuroectodermal origin. When the histologic study confirmed the diagnosis, the patient was treated with chemotherapy, surgical excision of the tumor, and adjuvant radiotherapy. Radiotherapy is thought to be involved in the genesis of osteogenic sarcomas as it has been shown in several reports, but there is no evidence in the literature of a peripheral neuroectodermal tumor developing after radiotherapy.

Adult↗
PubMed2006

Renal primitive neuroectodermal tumor in childhood with intracardiac extension.

Primary primitive neuroectodermal tumors of the kidney are exceptionally rare and usually affect children and young adults. We report the first pediatric case of renal primitive neuroectodermal tumor presenting with tumor extension along the inferior vena cava to the right ventricle. This case highlights that when considering a renal tumor with significant intravascular extension in the pediatric age group, although the most likely diagnosis remains Wilms tumor, other rare entities may also demonstrate similar clinical and imaging features.

Child, Preschool↗
PubMed2004

Primary intraspinal primitive neuroectodermal tumor at conus medullaris.

A primary intraspinal primitive neuroectodermal tumor is very rare, with only 24 cases having been reported in the literature. In general this type of tumor is treated with surgery followed by radiotherapy and chemotherapy; however, the prognosis still remains poor. The case of a primary intraspinal primitive neuroectodermal tumor, at the conus medullaris in a 17 year old male patient is presented. He had suffered from paraparesis, urinary difficulty and lower back pain of 1 month duration. A thoracolumbar MRI demonstrated a 2 x 2 x 8 cm isointense intraspinal mass, on T1-weighted images, with strong contrast enhancement from the T11 to L2 level. There was no clinical or radiological evidence for the existence of an intracranial tumor. A histological examination revealed a small round cell tumor and immunohistochemical characteristics of PNET. The clinical, radiological and pathological features are discussed with a review of the literatures.

Adolescent↗
PubMed1993

Primary neuroectodermal tumors of the ovary. A report of 25 cases.

Twenty-five primary ovarian neuroectodermal tumors occurred in females from 6 to 69 (average, 23) years of age; they had the usual presenting symptoms of abdominal swelling or pain. The tumors, which varied from cystic to solid, ranged from 4 to 20 cm (average, 14 cm) in diameter. Microscopic examination revealed three histologic categories--differentiated, primitive, and anaplastic--with the tumors in the first group having a better prognosis than those in the other two groups. Five of the six differentiated gliomas were pure ependymomas, and one was an ependymoma with an astrocytoma component; none contained teratomatous elements. Two patients with stage I tumors were alive 4 and 5 years postoperatively. The one patient with stage IIA tumor was free of disease at 3 years; one of the two patients with a stage III tumor died of tumor after 5 years, and one had two recurrences but was alive and well at 5 years. Twelve tumors were primitive, resembling medulloepithelioma, ependymoblastoma, neuroblastoma or medulloblastoma. Seven tumors had teratomatous foci of other types, including three dermoid cysts. Three patients with stage I tumors were alive at 7 months, 3 years, and 9 years postoperatively; six of seven patients with stage III tumors died of tumor 2 to 20 months postoperatively, and one was alive with disease at 1 year. Seven tumors were anaplastic, resembling glioblastoma. All contained foci of squamous epithelium. One patient with stage IA tumor died of tumor at 2 years, but two were free of tumor after 3 and 4 years. One patient with a stage IIA tumor died of disease after 5 years; another was alive with tumor at 1 year. One patient with a stage III tumor died after 4 months. The differential diagnosis of neuroectodermal tumors of the ovary includes many primary and metastatic ovarian neoplasms of diverse types, and distinction among them is important. Neuroectodermal tumors should be considered when examining unusual ovarian tumors, particularly if the patient is young.

Adolescent↗
PubMed1992

Heterologous tissue elements in melanotic neuroectodermal tumor of infancy.

Two cases of melanotic neuroectodermal tumor of infancy (MNTI) contained highly cellular stromal areas consisting of spindle cells exhibiting mitotic activity. In one case, single spindle cells exhibited the same immunohistochemical profile as tumor cells forming part of the epithelial component. In the other case woven bone was formed in a dense fibroblastic stroma. These tumor parts were judged to be heterologous tissue elements that also may be observed in other kinds of neuroectodermal tumors and that reflect the potential of the neural crest to differentiate into various mesenchymal tissue types.

Cell Nucleolus↗
PubMed1989

N-myc amplification and neuronal differentiation in human primitive neuroectodermal tumors of the central nervous system.

Despite the phenotypic similarities between primitive neuroectodermal tumors of the central nervous system, childhood neuroblastoma, and peripheral neuroepithelioma, a histogenetic relationship among these neoplasms has not been shown. High levels of N-myc expression occur selectively in developing brain and in some embryonic tumors of neural origin. N-myc amplification and high levels of N-myc expression in childhood neuroblastoma have been correlated with disease stage and prognosis. To determine whether the copy number of the N-myc gene in primitive neuroectodermal tumors of the central nervous system is altered, we examined 20 primitive neuroectodermal tumors by Southern and/or slot blot hybridization to a 1-kilobase N-myc genomic DNA sequence and a 492-base pair N-myc-specific subclone as well as to a 1.1-kilobase albumin complementary DNA sequence as a control for gene copy number. Amplification of the N-myc gene was detected in two cerebellar tumors both of which exhibited neuronal differentiation by light microscopy. These tumors had not been treated previously. Of the remaining 18 tumors, eight were undifferentiated, three showed early neuroblastic, and seven focal glial differentiation. These findings suggest a possible relationship between N-myc amplification and neuronal differentiation.

Brain Neoplasms↗
PubMedDate not supplied

Cytology of neuroectodermal tumors of the brain in crush preparations. A review of 56 cases of deep-seated tumors sampled by CT-guided stereotactic needle biopsy.

Fifty-six brain tumors of neuroectodermal origin were sampled by computed tomographic stereotactic needle biopsy. Crush preparations prepared from tiny tissue fragments displayed distinctive cytologic characteristics of different tumor types in 77% of the cases. The adjunctival value of crush preparations to frozen section diagnosis is discussed, and the cytologic features of different types of neuroectodermal brain tumors in crush preparations are illustrated.

Adolescent↗
PubMed1984

Neuroectodermal tumor and monoclonal antibodies.

Antibody-producing clones were obtained by hybridization of spleen cells from mice immunized with whole cultured human tumor cells and mouse myeloma cells, P3UX59AG8. The tumor cells were derived from a peripheral primitive neuroectodermal tumor. One of the antibodies produced by these clones reacts with the original cell line, SK-PN-DW, and other more differentiated neuroectodermal tumors such as neuroblastomas and melanomas. The cell line SK-PN-DW contains antigenic determinants recognized by monoclonal antibodies raised against melanoma, neuroblastoma, and human fetal brain. These data indicate that this primitive neuroectodermal tumor is derived from the neuroectoderm.

Adolescent↗