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)

Recent studies in autologous bone marrow or peripheral blood transplantation in solid tumors are discussed. The toxicity and activity of high-dose cisplatin or carboplatin combined with etoposide and other drugs are described. The results of trials in nonseminomatous germ cell tumors, neuroblastoma, ovarian cancer, and brain tumors are detailed and discussed. The impressive antitumor activities noted in certain subgroups should lead to an early application of these strategies before drug resistance becomes prevalent.
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PMID:Autologous bone marrow transplantation in solid tumors. 131 17

Dose-intensive chemotherapy for recurrent or refractory germ cell cancer has evolved over the last decade. Initial experience using high-dose regimens including single agents like cyclophosphamide, thiotepa, and etoposide demonstrated that responses could be obtained in the majority of patients but that they tended to be of short duration. Second-generation studies focusing on this disease site incorporated drugs and principles specific to germ cell cancer. Large studies performed at Indiana University (Indianapolis, IN) and in several European centers have demonstrated that the addition of high-dose carboplatin or cisplatin to other regimens can result in long-term survival of otherwise incurable patients. With the results of initial pilot studies now confirmed, high-dose carboplatin-based chemotherapy is beginning to play a role in first salvage therapy, and in some centers is being used in protocols of initial therapy for poor-risk patients. Based on the success of some of the early studies in germ cell cancer, similar protocols also are being investigated for other disease sites such as ovarian cancer, neuroblastoma, lymphoma, and breast cancer.
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PMID:Dose-intensive therapy for germ cell neoplasms. 132 18

Nuclear Medicine offers screening methods for oncology such as bone and bone marrow scintigraphy. During the last two decades, special procedures have gained widespread application. This paper is centered around the "tumor-specific" radiopharmaceuticals. In patients with thyroid cancer, I-131 still plays a significant role. Ga-67 still has its indications in lymphoma, while in other diseases Tl-201 chloride is now the agent of choice. Especially in thyroid cancer, Tl-201 has proved to be a reliable tumor imaging radiopharmaceutical. More recently, Tc-99m MIBI was introduced for tumor imaging. Tc-99m HMPAO may also be used for tumor scintigraphy, especially in brain lesions. In addition, I-123 IMP has successfully been used for imaging malignant melanoma. Another promising field of tumor diagnosis is receptor imaging. In neuroblastoma and malignant pheochromocytoma, I-131/123 mIBG is the radiopharmaceutical of choice and may be considered as a receptor imaging agent also. First clinical results with In-111 octreotide show potentials as somatostatin-receptor radiopharmaceutical in insulinoma, islet cell carcinoma, medullary and lung cancer, while I-123 estradiol needs some improvement until it may be recommended as diagnostic tool in breast cancer. Since 1978, radiolabeled poly- or monoclonal tumor antibodies and their fragments have gained widespread application. Especially the Tc-99m 225.28S melanoma antibody, I-131 or Tc-99m CEA and In-111/I-131 labeled OC-125 antibodies have proven to be of clinical significance in melanoma, colorectal and ovarian cancer.
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PMID:The role of nuclear medicine in oncology. 138 87

Teniposide and etoposide are third-generation semi-synthetic derivatives of epipodophyllotoxin. Following the initial clinical introduction of teniposide in the 1970s, investigations focused almost exclusively on its analogue, etoposide, because of its formulation, which was felt to have advantages in addition to oral administration. Despite consistently inadequate dosing and scheduling, early phase I and II trial results with teniposide were promising, and current trends encourage a second look. The substantial antitumor activity of teniposide is comparable with that of etoposide, and clinical interest was rekindled when it was shown to have considerable activity against small cell lung cancer (SCLC). In view of the inadequacy of early trials and the premature cessation of clinical study, it is recommended that teniposide be reevaluated for its activity against malignant lymphomas, Hodgkin's disease, leukemias, and SCLC, against all of which its early results were encouraging. In addition, consideration should be given to its activity against brain tumors, neuroblastomas and other childhood solid tumors, and ovarian cancer; its potential value against gastric, hepatocellular, breast, and bladder cancers also should be investigated. Other areas that warrant further study include elucidation of the exact mechanism of action of teniposide, its role in both single- and multiple-agent chemotherapeutic regimens, and resolution of its optimal dose and schedule. Finally, it is suggested that with new routes of administration and improved formulations, teniposide may be expected to play a significant role in the treatment of malignant lymphomas, SCLC, and pediatric lymphocytic leukemia and neuroblastoma.
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PMID:Teniposide in adult solid tumors: a historical perspective. 141 38

In the last decade, several features have improved our knowledge of CNS paraneoplastic syndromes. Patients with paraneoplastic cerebellar degeneration (PCD) and breast or ovarian cancer, but not with other tumors, harbor an antibody against Purkinje cells (called anti-Yo). Clinical features of anti-Yo positive and negative PCD are similar but the latter may have a less progressive clinical course with occasional remissions. In addition to the association of opsoclonus with neuroblastoma, this syndrome has been identified in patients with breast or small-cell lung cancer (SCLC). Patients with opsoclonus and breast cancer have an antineuronal antibody (called anti-Ri) not present if opsoclonus is associated with SCLC or neuroblastoma. Paraneoplastic encephalomyelitis (PEM) is almost always associated with SCLC. Most patients present with sensory neuronopathy, limbic or brainstem encephalitis but involvement of multiple levels is usual. An antibody (called anti-Hu) against neuronal nuclear antigens is present in patients with PEM and SCLC. Autopsy studies demonstrate deposits of anti-Hu specific IgG in the neurons and a predominance of T cells in the inflammatory infiltrates. Treatment of the tumor and immunosuppressors are effective in opsoclonus whereas patients with PCD or PEM with circulating antibodies do not improve.
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PMID:Clinical and pathological advances on central nervous system paraneoplastic syndromes. 144 70

The neu gene in rat neuro/glioblastoma was found to be activated by a single point mutation in the DNA sequence encoding the transmembrane region of the neu-encoded p185 protein. The human homologue of the rat neu gene, termed c-erbB-2 or HER-2, can also be activated in vitro by a similar mutation in the corresponding region. Although the human neu gene was shown to be amplified/overexpressed in a large portion of human breast and ovarian cancer, no reports indicate that the human neu gene is activated by a point mutation in human tumor. To study the possible point mutation of neu gene in human tumors, we characterized the genomic structure in the transmembrane region of human neu gene, which in turn allowed us to determine DNA sequence in this region directly following DNA amplification by polymerase chain reaction. We analyzed 7 tumor cell lines (2 breast cancer, 1 neuroblastoma, 1 rhabdomyosarcoma, and 3 glioma) and 11 tumor tissue samples (8 breast and 3 ovarian cancers). No mutation was found in the transmembrane region of human neu gene. Our results suggest that unlike the rat neuro/glioblastoma, the single point mutation in the transmembrane region of the human neu gene is a rare event in human tumors. In this study, we developed a technique for direct DNA sequencing of the transmembrane region of the human neu gene. This technique makes it possible to screen a large number of tumor samples.
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PMID:Direct sequencing analysis of transmembrane region of human Neu gene by polymerase chain reaction. 220 83

Bone marrow autotransplantation involves the administration of very high doses of chemotherapy or radiation therapy, or both, followed by infusion of autologous hematopoietic stem cells. This treatment was used in the past as a salvage therapy for patients with end-stage cancers. Occasional cures in patients with chemotherapy-responsive malignancies encouraged oncologists to utilize this treatment earlier when a better result might be achieved. This has led to a substantial number of long-term disease-free survivors in non-Hodgkin's lymphoma, Hodgkin's disease, acute leukemia, and neuroblastoma. Studies are currently ongoing in the treatment of breast cancer, multiple myeloma, testicular cancer, and ovarian cancer. Important areas for future investigation include the identification of optimal criteria for patient selection and timing of the therapy, the need for infusion of hematopoietic stem cells as cloned hematopoietic growth factors become available, the identification of the most effective high-dose regimens, and the need for "purging" tumor cells from the marrow before re-infusion. Successfully addressing these issues will increasingly require large comparative trials.
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PMID:Bone marrow autotransplantation. 264 72

Trends in the incidence of childhood cancer in Connecticut are reported and analyzed for the period 1935-1979 by 5-year age groups (0-4, 5-9, 10-14, 15-19 years), using a log linear model method. A threefold increase (P less than .001) in the incidence of ALL in males 0-4 years of age was observed, with significant increases of smaller magnitude seen in males aged 5-9 and 15-19 and females aged 0-4 and 5-9. The incidence of central nervous system cancers also increased in several age groups for both sexes with the largest increase seen in males 0-4 years old. Significant increases in incidence of large magnitude were also observed for Hodgkin's disease, in males aged 15-19 years and females aged 10-19 years, for neuroblastoma in both sexes at ages 0-4 years, and for testis and ovarian cancer at ages 15-19 years. This study of trends in incidence of childhood cancers by 5-year age groups has revealed significant changes, which would not have been as apparent if broader age groups had been used. These results provide relevant data for investigating the etiology of cancer during infancy, childhood, and adolescence. Trends in Connecticut are compared with findings from other registries in the United States and other countries.
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PMID:Trends in the incidence of childhood and adolescent cancer in Connecticut, 1935-1979. 335 39

Marrow transplantation is effective treatment for a number of hematological diseases in patients under the age of 50 who have an HLA-identical sibling donor. It is successful in the treatment of aplastic anemia with 70-85% long-term survival. It offers 10-30% apparent cures for patients with acute leukemia who have relapsed at least once, and for those with chronic myelocytic leukemia in blast crisis. Although still somewhat controversial, it appears to be the treatment of choice for patients with acute nonlymphoblastic leukemia in first chemotherapy induced remission, and for those with chronic myelogenous leukemia in the chronic phase since approximately 50-60% of these patients experience long-term, disease-free survival. Patients with acute lymphoblastic leukemia grafted in second or subsequent remission may expect a 30% "cure" of their disease. Marrow grafting is the only effective treatment for many patients with inherited immunologic deficiencies and certain genetic storage diseases. Cures of congenital Fanconi's anemia, Blackfan-Diamond anemia, osteopetrosis, paroxysmal nocturnal hemoglobinuria and thalassemia major have been achieved. Marrow transplantation is being explored for the therapy of patients with lymphoma, Hodgkin's disease, preleukemia, multiple myeloma, hairy cell leukemia, small cell lung cancer, testicular cancer, ovarian cancer and neuroblastoma. Marrow transplantation has been limited by the fact that many patients do not have HLA-identical siblings and very few have monozygotic twins. More recently, marrow transplants from HLA-nonidentical family members and even from unrelated donors have been successfully explored.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Marrow transplantation: the Seattle experience. 391 47

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


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