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Results for “Neuroectodermal Tumors”

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At least 235 records · Page 13Linked to original sources
PubMed1983

Human monoclonal antibody to a neuroectodermal tumor antigen (OFA-I-2).

Human IgM kappa monoclonal antibody to human tumors of neuroectodermal origin was produced in the spent medium of an Epstein-Barr virus-transformed B-lymphoblastoid cell line, L72. Chemically, the antigen was identified as ganglioside GD2 [Gal NAc beta 1----4 (Neu Ac alpha 2----8 Neu Ac alpha 2---3) Gal beta 1----4 Glc----ceramide]. Twenty-seven mg of pure human IgM were obtained from 10 liters of L72 spent medium using salt and hypotonic precipitation, ultracentrifugation, and Sephacryl-S 300 superfine gel filtration. The monoclonal origin of the antibody was determined by agarose isoelectrofocusing. This human monoclonal antibody may be a particularly useful reagent for immunotherapy trials in cancer patients.

Antibodies, Monoclonal↗
PubMed1982

Primitive neuroectodermal tumor arising in the ulnar nerve. A case report.

Malignant peripheral nerve tumors are rare. A neuroectodermal tumor arose in a 59-year-old man from the ulnar nerve. Typically, the tumor reproduces some of the appearances of cells of the primitive central nervous system. The possibility that a primary tumor of the sympathetic nervous system, especially of the adrenal glands, should be considered in the differential diagnosis. The tumor may arise from a major peripheral nerve and show features of growth patterns of the embryonic central nervous system. Blood stream spread is not uncommon; awareness of this proclivity makes early diagnosis mandatory.

Humans↗
PubMed2003

Primitive neuroectodermal tumors, embryonal tumors, and other small cell and poorly differentiated malignant neoplasms of the central and peripheral nervous systems.

Many different types of small cell, embryonal, and poorly differentiated neoplasms originate within the central and peripheral nervous systems. Because appropriate treatment is based on a correct diagnosis, the surgical pathologist must be familiar both with basic characteristics of each of the numerous entities as well as the spectrum of morphologic features that each may display. The nosology and nomenclature of these tumors have a rich and varied history. One basic distinction is between primitive neuroectodermal tumors of the central nervous system (cPNETs) and primitive neuroectodermal tumors of the peripheral nervous system (pPNETs), which are clinicopathologically and genetically distinct. Among the cPNETs are medulloblastoma, pineoblastoma, cerebral neuroblastoma, ependymoblastoma, medulloepithelioma, primary rhabdomyosarcoma, and atypical teratoid/rhabdoid tumor, whereas the pPNETs comprise the more differentiated end of a spectrum of neoplasms that include skeletal and extraskeletal Ewing's sarcoma.

Central Nervous System Neoplasms↗
PubMed2001

Primitive neuroectodermal tumor involving the frontal skull base in an infant.

We report here a case of a primitive neuroectodermal tumor involving the frontal skull base in a 5-month-old male infant. He presented with rapidly progressing exophthalmos on the left side. Preoperative magnetic resonance imaging revealed a well-demarcated, enhanced mass of 6 cm in diameter in the intradural extra-axial frontal region. The tumor was totally removed by surgery. Pathological findings were consistent with a primitive neuroectodermal tumor (PNET). Intradural extra-axial PNET is very rare, and its prognosis is poor.

Cerebral Angiography↗
PubMed1977

Melanotic neuroectodermal tumor of infancy: its histological similarities to fetal pineal gland.

Striking similarities between the pineals of 107 fetuses and infants and a pigmented neuroectodermal tumor occurring in the right orbital and right frontal regions in a 6-month-old Puerto Rican boy were found. Both the human fetal pineal and melanotic neuroectodermal tumors of infancy are characterized by pigmented (melanin) epithelial cells, small undifferentiated cells, and a fibrovascular stroma. Our findings suggest the fetal pineal may be a normally occurring precursor of the melanotic neuroectodermal tumor of infancy, or that melanin production may be a normal capability of differentiating neuroepithelial cells.

Child, Preschool↗
PubMed1991

Nerve growth factor receptor expression in peripheral and central neuroectodermal tumors, other pediatric brain tumors, and during development of the adrenal gland.

Nerve growth factor (NGF) is important to the survival, development, and differentiation of neurons. Its action is mediated by a specific cell surface transmembrane glycoprotein, nerve growth factor receptor (NGFR). In this study, NGFR expression by human fetal and adult adrenal medullary tissue, peripheral nervous system (PNS) neuroectodermal tumors (neuroblastoma, ganglioneuroblastoma, ganglioneuroma), pediatric primitive neuroectodermal tumors (PNETs) of the central nervous system (CNS), and CNS gliomas was examined by an immunohistochemical technique. Sixty-nine tumors in total were probed in this manner. Nerve growth factor receptor immunoreactivity was confined to nerve fibers and clusters of primitive-appearing cells in the fetal adrenal, and to nerve fibers and ganglion cells of the adult adrenal medulla; adrenal chromaffin cells were negative. In PNS neuroectodermal tumors, there was NGFR expression in tumor cells of 6 of 11 neuroblastomas and 6 of 6 ganglioneuroblastomas or ganglioneuromas. Thirteen of thirty-five CNS PNETs showed NGFR positivity. In most CNS PNETs, NGFR was restricted to scattered single or small groups of cells, but two tumors with astroglial differentiation showed much more extensive immunoreactivity. Most astrocytomas (11 of 14) and all ependymomas (3 of 3) were intensely NGFR positive.

Adolescent↗
PubMed2006

Radiologic findings of peripheral primitive neuroectodermal tumor arising in the retroperitoneum.

OBJECTIVE: The purpose of this article is to present the radiological findings of peripheral primitive neuroectodermal tumors that arise in the retroperitoneum. CONCLUSION: Peripheral primitive neuroectodermal tumors (PNETs) arising in the retroperitoneum tend to be large and aggressive. Although the imaging appearance of peripheral PNETs is nonspecific, these tumors should be considered in the differential diagnosis when one encounters a large retroperitoneal mass with aggressive features.

Adult↗
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↗
PubMed1987

Primitive neuroectodermal tumor cell lines: chromosomal analysis of five cases.

A cytogenetic analysis of primitive neuroectodermal tumor (PNET) cell lines was undertaken. PNET are presumed to be embryologically related to, but clinically and histologically distinct from, other tumors of neuroectodermal origin, including neuroblastoma and retinoblastoma. No single chromosome abnormality was found in all five of the tumors studied. In three of the five cases, however, additional 1q material [either as extra chromosome #1 or i(1q)] was found in all cells, and in two of the five, monosomy 13 was noted in all cells; the possible significance of these findings is discussed.

Cell Line↗
PubMed2000

Peripheral primitive neuroectodermal tumor of the small bowel mesentery: a case showing perforation at onset.

A case of peripheral primitive neuroectodermal tumor of the small bowel mesentery with an uncommon clinical onset is reported. A 40-year-old man was admitted to hospital because of acute severe abdominal pain. Chest X-ray revealed a free air sign beneath the diaphragm. At emergency surgery a mass measuring 11.0 x 8.0 cm with perforation was located in the jejunal mesenteric region. Histologically the resected lesion consisted of sheets of undifferentiated small round cells forming abortive Homer Wright rosettes. Some spindle-shaped cells showed perivascular pseudorosettes. Immunohistochemical study revealed that the tumor cells expressed positivity against CD99 (MIC2), neuron-specific enolase, synaptophysin and vimentin. To the authors' knowledge this is the first documentation of peripheral primitive neuroectodermal tumor of the small bowel mesentery with perforation at onset.

Abdomen, Acute↗
PubMed2004

The Ews/Fli-1 fusion gene switches the differentiation program of neuroblastomas to Ewing sarcoma/peripheral primitive neuroectodermal tumors.

Neuroblastoma (NB) and the Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumor (PNET) family are pediatric cancers derived from neural crest cells. Although NBs display features of the sympathetic nervous system, ES/PNETs express markers consistent with parasympathetic differentiation. To examine the control of these differentiation markers, we generated NB x ES/PNET somatic cell hybrids. NB-specific markers were suppressed in the hybrids, whereas ES/PNET-specific markers were unaffected. These results suggested that the Ews/Fli-1 fusion gene, resulting from a translocation unique to ES/PNETs, might account for the loss of NB-specific markers. To test this hypothesis, we generated two different NB cell lines that stably expressed the Ews/Fli-1 gene. We observed that heterologous expression of the Ews/Fli-1 protein led to the suppression of NB-specific markers and de novo expression of ES/PNET markers. To determine the extent of changes in differentiation, we used the Affymetrix GeneChip Array system to observe global transcriptional changes of genes. This analysis revealed that the gene expression pattern of the Ews/Fli-1-expressing NB cells resembled that observed in pooled ES/PNET cell lines and differed significantly from the NB parental cells. Therefore, we propose that Ews/Fli-1 contributes to the etiology of ES/PNET by subverting the differentiation program of its neural crest precursor cell to a less differentiated and more proliferative state.

Adaptor Proteins, Signal Transducing↗
PubMed2005

Primitive neuroectodermal tumor of the kidney with hyaline cells.

Primitive neuroectodermal tumor (PNET) of the kidney is a rare disease with high malignant potential. It affects young adults and has the tendency towards early metastasis. Relying on recently available immunohistochemistry and cytogenetic investigations, today most authors define PNET as part of the same nosologic entity as Ewing's sarcoma. We present the case of a 22-year-old man with a PNET arising from the kidney with, to our knowledge, a previously undescribed finding of hyaline cells within the tumor.

Adult↗
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↗
PubMed1988

Primitive neuroectodermal tumors of the chest wall.

Primitive neuroectodermal tumor (PNET) is a rare, highly malignant small-cell neoplasm that most often arises from the chest wall or paravertebral region. A patient is described from whom resection, intensive chemotherapy, and bone marrow transplant were unsuccessful. Eleven patients were treated between 1975 and 1985; there was only one long-term survivor (48 months) despite intensive multidisciplinary treatment. The median survival of 49 published cases was 9 months. Conservative resection minimizing disability, radiotherapy, and intensive chemotherapy remain valid options, but use of experimental agents as part of initial therapy seems justified. The role of autologous marrow transplant remains unclear.

Adult↗
PubMed1985

Melanotic neuroectodermal tumor of infancy (retinal anlage tumor).

Six cases of the melanotic neuroectodermal tumor of infancy, including light and electron microscopic findings, are presented. The tumor, now believed to originate from neural crest, is usually cured by wide excision; however, malignant cases have been reported. Histopathologically, this lesion resembles neural retina and retinal pigment epithelium. It may involve the orbit but most frequently occurs in the maxilla. One case presented involved the orbit, another the anterior fontanelle, and four presented in the maxilla.

Facial Neoplasms↗