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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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At least 739 records · Page 41Linked to original sources
PubMed1975

Carcinogenic action of quinoxaline 1,4-dioxide in rats.

Quinoxaline 1,4-dioxide was fed to 400 rats at levels of 10 mg or 1 mg/kg body weight for 18 months. It produced a high incidence of nasal and liver tumors only in the group fed 10 mg/kg. Of diverse histologies, the nasal tumors included anaplastic carcinomas, adenocarcinomas, squamous cell carcinomas, neuroepitheliomas, basal cell carcinomas, and a single fibrosarcoma. The nasal epithelium not involved in the neoplastic process showed dysplasia and hyperplasia.

Adenocarcinoma↗
PubMed1991

Genetic analysis as an aid in diagnosis for patients with midline carcinomas of uncertain histologies.

The tumors of nine patients with carcinomas of uncertain histogenesis (eight with poorly differentiated carcinomas involving primarily midline structures and one with a diagnosis of seminoma and atypical clinical features) were studied by cytogenetic and Southern blot analyses. Four of the eight patients with poorly differentiated carcinomas had abnormalities of chromosome 12 consistent with a diagnosis of germ cell tumor. These abnormalities comprised an i(12p) in two patients and a del(12q) in a third patient detected by cytogenetic analysis and multiple copies of 12p detected by Southern blot analysis in a fourth patient. Three of these four patients with a diagnosis of germ cell tumor established by genetic analysis achieved a complete response to cisplatin-based chemotherapy. The tumor biopsy of one patient showed a t(11;22) (q24;q12), and this patient had chemotherapy directed to neuroepithelioma. Cytogenetic analysis was unsuccessful for the tumors of three patients; these tumors did not have multiple copies of 12p detected by Southern blot analysis. These patients did not respond to cisplatin-based chemotherapy. One patient with a diagnosis of extragonadal seminoma failed to respond to cisplatin-based chemotherapy and had a second tumor biopsy performed that demonstrated a t(8;14) (q24;q32). This patient's diagnosis was changed to a non-Hodgkin's lymphoma. Thus, genetic analysis provided a diagnosis in six of nine patients. Cytogenetic and molecular analyses are useful clinical tools for the determination of histogenesis in some patients with poorly differentiated carcinomas of uncertain histology.

Adult↗
PubMed2000

Alpha-synuclein expression in central nervous system tumors showing neuronal or mixed neuronal/glial differentiation.

Alpha-synuclein (alpha-synuclein) is a member of a family of cytoplasmic proteins found predominantly and abundantly in the brain, and concentrated in pre-synaptic nerve terminals, near vesicles. We hypothesized that an antibody to alpha-synuclein could be a useful marker of neuronal differentiation in central nervous system (CNS) tumors. Twenty tumors known to have neuronal or mixed neuronal/glial differentiation ( 11 gangliogliomas, 2 anaplastic gangliogliomas, 5 gangliocytomas, and 2 ganglioneuroblastomas), 5 central neurocytomas, and 1 dysembryoplastic neuroepithelial tumor (DNET) were immunostained with a mouse monoclonal antibody raised against human alpha-synuclein. Intense cytoplasmic staining, in some instances extending into the perikarya, was seen in 6 of 11 gangliogliomas, 2 of 2 anaplastic gangliogliomas, and 2 of 2 ganglioneuroblastomas. Alpha-synuclein-positive cells were usually large in size, resembled dysmorphic neurons, and were variably immunoreactive for anti-neurofilament and/or anti-synaptophysin antibodies. In contrast, central neurocytomas, gangliocytomas, and the DNET were negative for cytoplasmic alpha-synuclein expression. Our findings indicate that alpha-synuclein is expressed within the neuronal component of mixed tumors of the CNS displaying more than 1 histophenotype, and/or showing different degrees of anaplasia. Based on currently available data, we conclude that cytoplasmic alpha-synuclein expression is a marker of maturing neurons in these tumors.

Cell Differentiation↗
PubMed1988

Esthesioneuroblastoma or malignant lymphoma? A case report.

Esthesioneuroblastoma is an uncommon tumor arising from the olfactory epithelium of the nasal vault. Differentiation from other tumors is often difficult, but techniques like immunocytochemistry and electron microscopy can help to distinguish a neuroblastoma from a malignant lymphoma with certainty. A patient is presented in whom difficulty was experienced in deciding on the diagnosis; the pathological data are presented to illustrate why confusion about the finite diagnosis can occur despite the use of these techniques. The initial diagnosis was a malignant lymphoma of the poorly differentiated lymphocytic type. The disease responded well to chemotherapy with cyclophosphamide, vincristine, procarbazine, and doxorubicin (COPA). When tumor recurred, rebiopsy of the small cell tumor was considered to be esthesioneuroblastoma, and the patient was treated with cyclophosphamide, vincristine, dacarbazine, and radiotherapy. The patient is now disease-free and has been without evidence of disease for more than 2.5 years; it is more than 5 years since the initial diagnosis of a small cell malignant neoplasm was made. This report illustrates the problems of diagnosis and treatment of this rare condition.

Adult↗
PubMed1989

High-dose chemotherapy and autologous marrow transplantation for esthesioneuroblastoma and sinonasal undifferentiated carcinoma.

Eight patients with recurrent esthesioneuroblastoma or sinonasal undifferentiated carcinoma of the nasal or sinus cavities were treated with high-dose chemotherapy and autologous bone marrow transplantation. All patients had stage C disease initially and had received extensive prior conventional treatment with surgery, radiotherapy, and chemotherapy. Two patients achieved prolonged relapse-free survival for 18+ and 60 months. The latter patient relapsed at 60 months, but died of progressive disease after a second transplant. Two additional patients remain alive without disease progression at 17+ and 31+ months posttransplant. No deaths occurred secondary to toxicity. Progression of tumor accounted for failure in five patients. High-dose chemotherapy and autologous bone marrow transplantation should be considered as a salvage regimen for selected patients who fail conventional therapy for these diseases.

Adult↗
PubMed1990

Chemotherapy of recurrent esthesioneuroblastoma. Case report and review of the literature.

Esthesioneuroblastoma is a rare epithelial tumor of the nasal olfactory mucosa. Its clinical course is characterized by indolent growth and persistent local recurrence and occasional distant metastases despite attempts at cure. Our report documents the response of a patient to a cisplatin-based drug combination and reviews the English-language literature of 25 patients treated with chemotherapy. We conclude that this tumor is sensitive to several different combinations and suggest guidelines for the use of chemotherapy in advanced or metastatic esthesioneuroblastoma.

Adult↗
PubMed2002

Prognostic factors and survival in late adolescent and adult patients with small round cell tumors.

The primary objective of this study is to review the clinical characteristics of 25 patients in the adult and late adolescent age group, diagnosed and treated with small round cell tumors involving soft tissues (extraosseous Ewing sarcoma, rhabdo-myosarcoma, primitive neuroectodermal tumor, and undiffer-entiated small round cell tumors). Additionally, survival and prognostic factors influencing the outcome with multimodality treatment are evaluated. There were 19 males (76%) and 6 females (24%). The median age was 26 years (range: 15-56 years). In 9 patients (36%), the tumor was located at an extremity, whereas 16 patients (64%) had central localizations. Tumor size was larger than 10 cm in 7 patients (29.2%). Six patients (24%) had metastatic disease. Twelve patients (48%) received radiation and 16 patients (64%) underwent surgery. Among the resected tumors, 2 were resected with contaminated margins (12.5%), whereas 2 were radically resected and 12 (75%) were resected with wide margins. All patients were given a median of 4 cycles of multiagent chemotherapy (1-14 cycles). With preoperative chemotherapy, complete regression (CR) of the tumor was achieved in 6 patients (24%). In 4 patients (16%), a partial response was obtained. After the completion of multimodality treatment, 12 patients (48%) had a CR. Progression-free (PFS) and overall survival (OS) for the entire group was 25.0 +/- 10.8% at 1 year and 30.5 +/- 15.5% at 3 years, respectively. Nonmetastatic disease, wide and radical resection, and presence of CR to multimodality treatment were associated with a significantly longer PFS and OS by univariate analysis. By multivariate analysis, CR to multimodality treat-ment was the only independent predictive factor for a longer OS (p: 0.0036, relative risk [RR]: 23.6, 95% CI: 2.8; 198.7) and metastatic presentation was the only independent factor predic-tive for a shorter PFS (p: 0.017, RR. 15, 95% CI: 1.6; 141.2). Large-scale, multicenter studies are required for a better eval-uation of the nonpediatric age group with small round cell tumors.

Adolescent↗
PubMed1984

The neuroectodermal tumor of bone.

Four small round cell malignant tumors of bone occurring in children are described. There was no catecholamine secretion and the clinical, radiologic, and biopsy diagnosis in each was Ewing's sarcoma. Glycogen was sparse both on imprints and in tissue sections. The tumors, when extensively sampled, had areas of a lobular growth pattern and Homer Wright rosettes. The rosettes were always focal and varied in complexity from case to case; they were rudimentary in one instance and markedly fibrillar in the most obvious instance. Neuron-specific enolase was demonstrated in tissue sections and in longterm cell cultures from three of the tumors. The cultured cells put out moderately long beaded processes in serum-free medium but had no catecholamine fluorescence. Electron microscopy of the tumor rosettes and the cultured cells showed processes containing aggregates of microtubules and only one case had rare neurosecretory granules. This study suggests that some small round cell tumors of bone and soft tissue in children, which present as Ewing's sarcoma, are neuroectodermal in nature.

Bone Neoplasms↗
PubMed1991

Renal neoplasms mimicking rhabdoid tumor of kidney. A report from the National Wilms' Tumor Study Pathology Center.

Fifty-six renal neoplasms reviewed by the National Wilms' Tumor Study Pathology Center presented with histologic features that resulted in confusion with rhabdoid tumor of kidney, a usually lethal childhood renal tumor; all were eventually diagnosed as other entities. Conspicuous filamentous cytoplasmic inclusions or large nucleoli, typical findings in rhabdoid renal tumors, were the usual source of diagnostic difficulty. Most, but not all, tumors occurred in pediatric patients. Sixteen were examples of Favorable Histology Wilms' tumor, which invited confusion with rhabdoid tumors either on the basis of filamentous cytoplasmic inclusions (15 cases) or macronucleoli (one case). In most cases, foci of typical Wilms' tumor blastemal aggregation or evidence of definitive nephrogenic differentiation facilitated the correct diagnosis. All 10 patients for whom information about outcome was available were alive at last follow-up. The other 40 renal lesions mimicking rhabdoid tumor of kidney consisted of a clinically and histogenetically diverse group of neoplasms, including anaplastic Wilms' tumor, congenital mesoblastic nephroma, renal cell carcinoma, transitional cell carcinoma, collecting-duct carcinoma, oncocytoma, rhabdomyosarcoma, malignant neuroepithelial tumors, and lymphoma. Most of these lesions could be separated from renal rhabdoid tumors and correctly classified on the basis of careful attention to light microscopic details, but in several cases electron microscopy or immunocytochemical studies were helpful or essential.

Adenoma↗
PubMed1992

Desmin positivity in primitive neuroectodermal tumors of childhood.

In this report, we describe two rosette-forming primitive neuroectodermal tumors that were found to contain desmin by both immunohistochemistry and Western blotting. Electron microscopy on both cases was consistent with primitive neuroectodermal tumors and revealed that the tumor cells contained cytoplasmic bundles of intermediate filaments. In both cases, studies for MyoD1 protein using immunohistochemistry and Western blotting were negative. Thus, the detection of desmin in a pediatric neoplasm does not absolutely exclude the diagnosis of primitive neuroectodermal tumor and should not be considered as prima facie evidence that a small-cell tumor is a rhabdomyosarcoma.

Abdominal Neoplasms↗
PubMed1993

PNET vs MPNST.

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Carcinoma↗
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↗
PubMed1995

MAb O13.

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Antibodies, Monoclonal↗
PubMed1983

Anterior cranial fossa tumors.

The anterior cranial fossa may be invaded by malignant or nonmalignant tumors arising in the orbit, orbital contents, nose, nasopharynx, or frontal, ethmoid, or sphenoid sinuses. Skin and maxillary tumors can also spread to the skull base. One must be aware of this and assess the situation with the neurosurgeon, and use coronal and axial CAT scans. The approach is intracranial and extracranial, with total en bloc tumor resection under frozen section control. In nonmalignant tumors immediate reconstruction is performed; in malignant tumors, especially recurrent ones, the reconstruction is delayed twelve to eighteen months to lessen the chances of later recurrences. With this approach, recurrences have been few and survival rates excellent, even in advanced recurrent lesions.

Carcinoma, Squamous Cell↗
PubMed1990

Vascular lesions involving the cranial base: combined surgical and interventional radiologic approach.

Advantages and possible complications of combined surgical and interventional radiologic approach to vascular lesions involving the cranial base are presented in three case examples. The methodology and technology of endovascular embolization and occlusion techniques, selection of embolic materials, functional testing to prevent neurologic injury and surgical implications of these treatment modalities are discussed. Emphasis is placed on individualization of each combined approach relative to the aforementioned factors.

Catheterization↗
PubMed1992

Neurological deterioration after lumbar cerebrospinal fluid drainage.

Large-bore lumbar spinal fluid drainage is used frequently as part of the preoperative and intraoperative management of patients undergoing cranial base tumor resection. Such drainage allows displacement of the brain with minimal force, thereby potentially decreasing retraction damage to it. We document 2 patients in whom serious complications resulted from lumbar drainage systems. These patients deteriorated into a coma state following cerebrospinal fluid (CSF) drainage. Reinfusion of synthetic CSF solutions caused a brisk return to normal neurological status. These plus other potential complications associated with lumbar drainage, such as persistent CSF leaks into the back and soft-tissue nerve root injury, warranted abandoning the lumbar cistern drainage route of CSF drainage in favor of drainage directly from the intracranial compartment. Depending on the particular operation performed, drainage of CSF near the cribriform plate, the suprachiasmatic cistern, or from the sylvian fissure may be effective sites for CSF drainage. Unlike lumbar drainage, intracranial CSF drainage does not have the added risk of promoting cerebral herniation.

Carcinoma, Squamous Cell↗