Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Five cases of ovarian tumor with an exclusive or almost exclusive malignant neuroectodermal composition are reported. Four of them were unquestionably primary, but one, although probably primary, was possibly metastatic from the adrenal gland. The ages of the four patients with tumors in the former category ranged from 13 to 17 with an average of 15 years; the fifth patient was 18 years old. The presenting symptoms were similar to those of other ovarian cancers. Two of the definitely primary tumors were pure while two others contained minor foci of mature teratoma. Only one of the four patients with an unquestionable primary ovarian tumor survived for more than 7 years; the others died of tumor from 2 months to almost 4 years postoperatively. The fifth patient presented with unilateral ovarian involvement by neuroblastoma and abdominal metastases; autopsy over 6 months later revealed involvement of both adrenal glands. This case appears to be the first recorded case of neuroblastoma presenting clinically as a primary ovarian cancer.
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PMID:Malignant neuroectodermal tumor of the ovary, a distinctive form of monodermal teratoma: report of five cases. 711 57

A thorough understanding of the incidence, clinical presentation, treatment, prognosis, and psychosocial issues surrounding children with solid tumors enables the nurse to actively participate on the health care team. Although significant advances over the past two-and-a-half decades to improve the outcomes of children with cancer have occurred, there remains room for continued improvement, especially among children with advanced-stage nephroblastoma, neuroblastoma, HCC, and teratoma.
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PMID:Solid tumors in children. 752 19

We report the light microscopic and immunohistochemical features of vascular proliferations associated with 26 extracranial neural and neuroendocrine neoplasms including esthesioneuroblastoma, neuroblastoma/ganglioneuroblastoma, the primitive neural component of immature teratoma, mediastinal teratoma, primitive neuroectodermal tumor, intra-abdominal desmoplastic small cell tumor, Merkel cell carcinoma of the skin, and thyroid medullary carcinoma. These vascular proliferations were similar to those associated with high-grade glial neoplasms and were characterized by tufts of vessels with a glomeruloid configuration or by long cords of vessels. Immunohistochemical evaluation documented the presence of endothelial cells, perithelial cells, and basement membrane components within the foci of proliferating vessels. We propose that these vascular proliferations represent a characteristic feature of the neuroendocrine/neural neoplastic phenotype and that they possibly arise as the result of angiogenic factors produced by the neoplastic cells. The presence of these distinctive vascular lesions in the stroma of a poorly differentiated neoplasm should alert the pathologist to the possibility of the neoplasm being of a neural or neuroendocrine nature.
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PMID:Florid vascular proliferation associated with neural and neuroendocrine neoplasms. A diagnostic clue and potential pitfall. 877 95

Sinonasal teratocarcinosarcoma (SNTCS) is a very unusual and aggressive neoplasm characterized by the combination of malignant teratoma and carcinosarcoma features, of which less than forty cases have been reported in the literature. We report on a 75-year-old man with SNTCS that involved the left ethmoid, maxillary and sphenoidal sinuses. The tumor showed a complex histological pattern with mature and immature glands, benign squamous and malignant poorly differentiated epithelia, as well as neuroblastoma-like tissue and sarcoma component with rhabdomyoblastic differentiation. This peculiar blend of tissue types makes the diagnosis of this entity a difficult challenge, especially in small biopsies or in tumors only partially removed. This tumor must be differentiated from several types of carcinomas, esthesioneuroblastoma, craniopharyngioma, malignant mixed tumor of salivary gland type and germ cell tumors. The present case represents, to our knowledge, the third SNTCS described in the european literature.
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PMID:Sinonasal teratocarcinosarcoma: an unusual neoplasm. 756 86

The karyotypes of 47 pediatric brain tumors (14 cerebellar pilocytic astrocytomas, six cerebral pilocytic astrocytomas, seven anaplastic astrocytomas and glioblastomas, nine medulloblastomas [PNETs], one cerebral neuroblastoma, four ependymomas, and seven miscellaneous other neoplasms) are presented. Most of the pilocytic astrocytomas and ependymomas had normal karyotypes. In contrast, the majority of the anaplastic astrocytomas-glioblastomas were abnormal. The abnormalities included losses and structural abnormalities of chromosomes 9, 13, and 17, and double minutes. There were no losses of chromosomes 10 and 19q or gains of chromosome 7, which are among the most common abnormalities of adult glioblastomas. The chromosomal abnormalities in the medulloblastomas were similar to those reported in the literature but less frequent. Four tumors (choroid plexus papilloma, meningioma, cerebral malignant rhabdoid tumor, and immature teratoma) had losses of chromosome 22.
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PMID:Chromosomal abnormalities in 47 pediatric brain tumors. 762 8

The in vitro cytotoxic effects of docetaxel (Taxotere; RP56976, NSC688503) proved both time and concentration dependent. Amongst thirteen human cell lines from various tumor types, exposure to increasing concentrations of docetaxel over 24 hrs resulted in a plateau-shaped dose response curve, suggesting that increased cell kill becomes more dependent on increased exposure duration than on concentration. IC50 concentrations (reducing survival by 50%) ranged from 0.13-3.3 ng/ml, with three neuroblastoma lines proving most sensitive and three breast and two colon carcinoma lines showing least sensitivity. There was significant cross-resistance to docetaxel in the classic multidrug resistant (MDR) Chinese hamster ovarian (CHO) CHRC5 line and the human lymphoblastoid CCRF-CEMVLB1000 line, as well as in two vincristine (VCR)-selected MDR MCF-7 sublines. All four of these MDR sublines overexpress P-glycoprotein (Pgp), as did a 6-fold docetaxel-selected resistant CHO subline. As an apparent corollary, in two human teratoma lines selected for etoposide resistance and showing some cross-resistance to VCR and in two CHO sublines expressing low levels of VCR resistance, yet all proving Pgp positive, no docetaxel cross-resistance was identified. Verapamil modulated docetaxel resistance only in sublines expressing resistance to the drug and overexpressing Pgp. Four other human tumor sublines selected for resistance to 5-fluorouracil, cisplatin or teniposide, showed a lack of cross-resistance to docetaxel. Furthermore, cross-resistance to docetaxel was not apparant in four epipodophyllotoxin-selected resistant sublines with alterations in topoisomerase II, indicating its effectiveness against tumor cells expressing the topoisomerase II-related MDR phenotype. Our observation that docetaxel cross-resistance was not automatically expressed by classic MDR tumour cells appears of interest and of potential clinical relevance.
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PMID:Differential cytotoxic effects of docetaxel in a range of mammalian tumor cell lines and certain drug resistant sublines in vitro. 789 35

Immature teratoma occurs occasionally in the brain of children and contains a large amount of immature neuronal tissue. These primitive neuronal components are a good target for studying the morphology of primitive neuroectodermal tumors, including neuroblastoma, ependymoblastoma, medulloepithelioma and so on. Primitive neural tubes are immunohistochemically and ultrastructurally studied in two cases of primary immature teratoma of the child brains, compared to true rosettes in a case of neuroblastoma, primitive neural tube in the fetal rat brain (9 to 13 days of gestational age). The study also extends to the pathology of PNET. Ultrastructurally the primitive neural tube like structures in two teratomas were virtually identical those of developing fetal rat brains and true rosettes in a neuroblastoma. However, these tubular structures are different from each other in immunohistochemistry. These differences are considered to reflect the different developmental lineage of the tumor cells, that is, neuroblastoma produces only neuroblastic cells, and primitive neural tubes in teratoma both neuroblastic and glioblastic cells. Rejuvenation of neuroblastoma cells seems to render a VIM-positivity of the tumor cells.
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PMID:[Immunohistochemical and ultrastructural studies of intracranial immature teratoma--a comparative observation on neuroblastoma, PNET and ependymal tumor, with a special reference to rosette structures]. 816 54

During the past 43 years, 628 patients with a primary mediastinal tumor underwent surgical operation in our institution. Of those patients, 106 patients (16.9%) were children of 15 yr of age or less and 522 patients (83.1%) were adults. 47 (44.3%) of the 106 children had neurogenic tumors, while 21 patients (19.8%) had a teratoma. 189 (36.2%) of the 522 adults had thymoma and 89 patients (17.1%) had teratoma. In the 106 children there were 71 benign tumor patients (66.9%) and 35 malignant tumor patients (33.1%). There were 277 (53.1%) benign and 245 (46.95) malignant tumors in the 522 adults. The adults had significantly more malignant tumors than the children (p < 0.01). In our series of malignant mediastinal tumors in children, 14 patients (13.3%) had a lymphoma and 11 patients (10.5%) had a neuroblastoma. On the other hand, in the adults, 127 patients (24.3%) had a thymoma and 66 patients (12.6%) had a lymphoma. Regarding benign mediastinal tumors, in children, 30 patients (28.5%) had a ganglioneuroma and 19 patients (18.0%) had a teratoma. In the adults, 68 patients (13.0%) had a teratoma and 62 patients (11.9%) had a thymoma. In the clinical manifestation, 51 child patients (48.1%) and 191 adult patients (36.6%) were asymptomatic. As symptoms due to compression or direct invasion to adjacent structures, dyspnea was seen in 16 child patients (15.1%), and chest pain occurred in 59 adult patients (11.3%). Dyspnea was significantly more common in children than in in adults (p < 0.01). Emergency operations were performed in children more than in adults.
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PMID:[Primary mediastinal tumors in children--comparison with mediastinal tumors in adults]. 828 88

A primary neoplasm of the right adrenal gland in a 4-year-old boy was discovered after the patient developed bowel obstruction following an appendectomy. Until the histologic examination, the tumor was thought to be a neuroblastoma. However, the intra-adrenal tumor was composed of blastematous nodules, primitive tubules, and glomeruloid structures whose overall composition resembled a Wilms' tumor. Other than a single focus of mucinous glands, the tumor lacked the range of somatic tissue types of a teratoma. Approximately 50 cases of putative extrarenal Wilms' tumor have been reported. The retroperitoneum is one of the more common primary sites, yet our case is the first documented example of a neoplasm with features of a Wilms' tumor arising in the adrenal. Based on the embryologic and anatomic relationship between the adrenal gland and kidney, it is somewhat surprising that other instances of similar appearing tumors have not been described before the present case. Our patient was managed on a Wilms' tumor protocol and remains tumor free 15 months after surgery.
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PMID:A unique dysembryonic neoplasm of the adrenal gland composed of nephrogenic rests in a child. 854 Jun 3

110 cases of sacrococcygeal lesions seen in the Guangzhou Children's hospital were reviewed. 42 cases were anomalies, 66 cases were tumors and 2 were inflammatory granulomas. As compared to data in western literatures, the morbidity of anomalies in this series was lower than that of tumors, whereas in western reports the morbidity of anomalies was much higher than that of tumors. Another interesting point was that the morbidity of meningocele (MC) in this series was much higher than that of myelomeningocele (MMC) of spina bifida (7:1). According to a study in Australia, the MC:MMC was 1:5. We suggest that endodermal sinus tumor should be considered as an independent entity of there was no other tissue component (organlike component) found in tumor section. We also suggest that if neuroblastoma and/or other immature neuroectodermal component is observed in a teratoma, it should be classified as malignant rather than benign.
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PMID:[Analysis of 110 cases of sacrococcygeal lesions in children]. 874 72


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