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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primitive neuroectodermal tumor (PNET) is one of the small round cell malignancies of presumed neural crest origin for which an effective treatment has not yet been established. In the present study, a human cell line, designated KU-9, was established from a 27-year-old male patient with PNET of the retroperitoneal cavity and has been successfully maintained in nude mice and in culture. On histological examination, the primary tumor was composed of poorly differentiated small round cells arranged in clusters showing a variety of mitotic changes, and contained Homer-Wright rosettes. The histopathological appearance of the KU-9 xenografts was similar to that of the primary tumor. Electron microscopy revealed neurosecretory granules and cytoplasmic processes in the xenograft. No significant amplification of N-myc gene was observed in the KU-9 cells. The KU-9 cells showed chromosome numbers ranging from 56 to 61 with consistent structural abnormalities being add(2)(q31), +add(11)(p11.2), +add(13)(p11.1), and +del(22)(q12). Cultured KU-9 cells grew exponentially with a doubling time of about 50 h and a time-dependent increase in medium levels of neuron-specific enolase (NSE) was noted. Serum levels of NSE in KU-9 tumor-bearing nude mice were significantly elevated and a linear relationship between the serum NSE levels and the tumor NSE content or tumor volume was observed, suggesting that serum levels of NSE may reflect the PNET tumor burden and tumor extent. These results indicate that the KU-9 cell line provides a reproducible model system which could be useful in gaining some insight into the histogenesis and oncogenesis of PNET and in establishing an effective treatment for PNET.
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PMID:Establishment of a human cell line secreting neuron-specific enolase from a primitive neuroectodermal tumor of the retroperitoneal cavity. 863 6

The aim of this article is to present a case of primary neuroendocrine tumor (typical carcinoid) of the mandible that occurred in a 46-year-old black woman who was seropositive for the human immunodeficiency virus. Radiologically the lesion presented as a poorly circumscribed honeycomb radiolucency that extended from tooth 21 to the ascending ramus. Histologically the tumor cells were variously arranged in small islands, trabeculae, follicles, and slitlike spaces lined by a single layer of palisaded low-columnar cells. The follicles contained an eosinophilic colloid-like substance. Immunocytochemical staining showed diffuse, intense positivity for MAK 6, pancytokeratin, S-100, and neuron-specific enolase and focal, intense, positive staining for chromogranin A. Electron microscopy showed the presence of interdigitating cell membranes, rudimentary cell attachments, and varying numbers of membrane-bound dense core granules. Special investigations failed to reveal a primary tumor, and no metastases were found. Urine and hematologic assessment did not show any evidence of functional activity. The tumor was resected, and no recurrence or spread has been seen for 2 years. Origin from foregut-derived, immature, and functionally uncommitted endocrine cells is presumed.
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PMID:Neuroendocrine (carcinoid) tumor of the mandible: a case report and review of the literature. 885 Apr 88

The prognosis of children with neuroblastoma (NB) is dependent upon the patient's age at diagnosis, the location of the primary tumor, and histologic tumor cell differentiation. These characteristics, as well as the presumption that NB results from clonal expansion of primitive cells involved in sympathetic nervous system (SNS) development, predict that a model of tumorigenesis based upon normal fetal SNS histogenesis might indicate tumor progenitor status and define biologic and clinical behavior. Immunohistochemistry and in situ hybridization were used to examine a panel of marker gene products predicted or shown to be expressed during SNS development in the normal human fetal SNS from 8 to 24 weeks' gestational age. A similar analysis was performed in a selection of clinical NB tumors, and the results were compared. In a subset of differentiating, often extra-adrenal NB tumors in patients who frequently had a favorable outcome; advancing morphologic tumor cell differentiation spatially paralleled an advancing fetal extra-adrenal chromaffin marker gene expression phenotype (ie, increasing TrkA, TrkC, TH, IGF-2, and neuron-specific enolase expression but a lack of phenylethanolamine N-methyltransferase expression). In these tumors, expression of gene products associated with normal fetal sympathetic ganglionic differentiation (ie, Bcl-2, HNK-1, and neuropeptide Y) was lost with morphologic tumor cell differentiation. In contrast, undifferentiated tumors, the majority of which were high stage, adrenal in origin, and prognostically unfavorable, displayed marker expression characteristics mirroring that of an early fetal ganglionic lineage. Thus, we show that morphologic differentiation in stroma-poor NB tumors, long held as an important prognostic feature in tumor grading systems, often corresponds to an extra-adrenal chromaffin rather than a ganglion cell or adrenal medullary chromaffin phenotype. Understanding the biology of extra-adrenal chromaffin tissues may provide an explanation for the clinically less aggressive nature of differentiating NB tumors and suggest potential mechanisms for spontaneous regression and/or treatment response.
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PMID:A developmental model of neuroblastoma: differentiating stroma-poor tumors' progress along an extra-adrenal chromaffin lineage. 894 Dec 12

We describe a 50-year-old, previously healthy male with metastatic pulmonary blastoma associated with hypercalcemic and hyperosmolar complications which caused his death after 5 days. The primary tumor consisted of epithelial [cytokeratin, beta-hCG CEA, neuron-specific enolase (NSE)-positive] and mesenchymal components (beta-hCG, vimentin, NSE-positive, while the metastases had only a mesenchymal component.
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PMID:Pulmonary blastoma with fatal hypercalcemia. 915 81

Dimorphous cancer of the lung with predominantly peripheral localization was diagnosed in 9 (1.2%) out of 716 patients operated for lung tumor in 1986-1995. The blood-serum levels of neuron-specific enolase, tissue polypeptide antigen, carcinoembryonic antigen, alpha-fetoptotein, CA 19-9 and CA-125 carbohydrate antigens and total activity of alkaline phosphatase were assayed in all the patients before surgery. The study showed an 1.5-times increase in CEA concentration in 5 cases, with primary tumor size being 5 cm and more.
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PMID:[Dimorphous cancer of the lung]. 921 24

Neuroblastoma, a malignant tumor of infancy and childhood, has some very interesting peculiars: good prognosis, even with disseminated disease, propensity to occasionally undergo spontaneous regression, its ability to undergo spontaneous or induced differentiation to a benign ganglioneuroma. Neuroblastoma may originate anywhere along the sympathetic nervous system chain. The most common site of primary tumor is, however, within the abdomen either in the adrenal gland or in a paraspinal ganglions. A great deal of progress has been made in advancing the knowledge of human neuroblastoma at the cellular and molecular viewpoint. The genetic predisposition to develop the tumor is clarified, a specific oncogene amplified (N-myc) in neuroblastoma cells shows precise prognostic significance and the deletion of chromosome 1's short arm has been defined. Work-up in neuroblastoma's diagnosis include the urine assay for catecholamine metabolites (VMA, HAVA, VLA) and serum assay for the specific markers as neuron-specific enolase (NSE), ferritin, GD2 ganglioside. Imaging include CT-scan, MIGB body-scan and the newest monoclonal antibodies scan. Abdominal tumors are shown in about 75% of children > 12 months old. In 2/3 of cases, tumor is widely disseminated at the time of diagnosis. In the period 1979-94 the Italian Group for Neuroblastoma (GCN-AIEOP) collected 1083 cases of tumors and 5-yrs survival was 45% +/- 2.4 for the patients studied in the period 1979-84, which is increased to 58% +/- 3 for the group of patients 1990-94 (p < 0.001). The overall survival was 53 +/- 1.7. About 5-yrs survival at different stages, AIEOP shows that it is increased from 88% +/- 3.3 (1979-84 group) to 91% +/- 2.8 (1985-92) in the stage I and II (280 cases). In the stage IV survival value improved from 79% +/- 7.1 to 84% +/- 7 (132 cases). No statistical improvement can be observed, anyway. Better improvements can be pointed out in stage III (221 cases, survival from 48% +/- 5.2 (79-84 group) to 69% +/- 4.8 (85-92) and stage IV (483 cases, survival from 16% +/- 2.6 to 28% +/- 3.4) (p < 0.001). Finally we can summarize about neuroblastoma: 1) better prognosis in the first year of life; 2) ability to spontaneous regression, first of all, in stage IVs; 3) partial and provisional response to therapy in advanced stages; 4) no recovery increasing despite advancing in surgery and chemotherapy.
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PMID:[Recent advances on retroperitoneal neuroblastoma]. 941 95

A new cell line (YKK) was established from a primary tumor of neuroendocrine carcinoma of the endometrium. Cultured YKK cells are polygonal in shape. The modal chromosome number is 46 XX. The doubling time of the cells, after the 19th passage, is 38 hours and this cell line has been propagated continuously by serial passage over 50 passages for the past 2 years. YKK displays characteristics resembling the original tumor cell from the donor patient: it contains the neuron-specific enolase and chromogranin antibody, produces neuron-specific enolase, and is sensitive to cisplatin, carboplatin, and etoposide. When YKK cells are transplanted subcutaneously together with Matrigel into nude mice they form a very large tumor invading the subcutaneous muscular tissue. This experimental model is useful for studying the mechanism(s) regulating the invasion of this tumor into muscular tissue, and for the establishment of new anti-cancer treatments for neuroendocrine carcinoma.
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PMID:Establishment of a cell line from a neuroendocrine carcinoma of the endometrium and an invasion model. 967 72

A 57-year-old man was admitted with the chief complaint of macrohematuria. Digital rectal examination showed a slightly enlarged, irregular prostate with stony consistency. Serum levels of prostate specific antigen (PSA), neuron-specific enolase (NSE) and progastrin-releasing peptide (ProGRP) were elevated. Transurethral resection (TUR)-biopsy of the prostate revealed small cell carcinoma with poorly differentiated adenocarcinoma. Various radiological examinations revealed metastases to pelvic lymph nodes and liver. He was treated with chemoendocrine therapy consisting of cisplatin, etoposide, flutamide and luleinizing hormone-releasing hormone (LH-RH) agonist. The primary tumor and metastatic lesion decreased and serum PSA, NSE and ProGRP levels were decreased to normal ranges after 5 cycles of chemotherapy. After the 5-cycle chemotherapy, TUR-biopsy proved viable tumor cells. During the additional chemotherapy, tumor markers increased and 4 months later liver metastasis progressed. He died 13 months after diagnosis of small cell carcinoma of the prostate.
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PMID:[Small cell carcinoma of the prostate: a case report]. 978 1

We present an unusual case of epithelioid leiomyosarcoma in the right shoulder. A 10-cm tumor eroding the right scapula occurred in a 59-year-old man and grew into the subclavian vein with extension to the right atrium. An elevated serum level of neuron-specific enolase was also detected. Clinical presentation suggested small cell carcinoma of the lung (Pancoast tumor) with intravenous extension. Eventually, the patient died of respiratory insufficiency. Autopsy revealed a primary tumor in the right shoulder, numerous tumor emboli in the pulmonary arteries, and associated hemorrhagic infarction. Histopathologic features revealed an epithelioid leiomyosarcoma. Furthermore, immunohistochemical and immunoblotting analyses of the tumor demonstrated positive reactions for neuron-specific enolase. We interpreted this peculiar case to be a neuron-specific enolase-producing epithelioid leiomyosarcoma of the soft tissue associated with gross vascular invasion.
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PMID:Neuron-specific enolase-producing epithelioid leiomyosarcoma with gross vascular invasion and intracardiac involvement. 987 Aug 63

A 62-year-old man with a history of a resected rectal polyp was diagnosed 14 years later with right liver and multiple bone metastases. The liver biopsy showed a malignant epithelial tumor that was positive for neuron-specific enolase immunostaining and negative for chromogranin. Electron microscopy was characteristic of that for an endocrine tumor. Most circulating hormonal peptide levels were within normal ranges and only motilin level was elevated. On the right hepatectomy, the three large metastases had a histologic picture suggestive of an endocrine tumor. Immunohistochemistry revealed in some areas numerous tumor cells expressing motilin, and a few cells were strongly positive for pancreatic polypeptide and somatostatin. The retrospective analysis of the rectal polyp showed a similar histology and immunohistochemical profile, indicating that this lesion was the primary tumor. Motilin-positive cells from one of the hepatic lesions were identified on semithin sections and further processed for electron microscopy. Neurosecretory granules were numerous in all cells. Immunoelectron localization enabled us to characterize the motilin-containing neurosecretory granules, which had a mean diameter of 168.3x38.1 nm. Although not all tumor cells were motilin-positive, a diagnosis of motilinoma for the rectal polyp and its hepatic and bone metastases was proposed.
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PMID:Motilin-producing liver and bone metastases evidenced 14 years after resection of a rectal polyp. 1040 8


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