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

The resistance that many cancer patients show to the progress of their disease, attested to by well documented cases of spontaneous regressions as in neuroblastoma, hypernephroma, choriocarcinoma and malignant melanoma, and the long-term dormancy of multiple metastases seen particularly after removal of a primary mass, can be explained only by host defense mechanisms. Attemps at immunotherapy over the years are reviewed and new directions are presented.
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PMID:Immunologic approach to cancer therapy. 111 68

Multiple primary tumors are a common mode of presentation in familial neuroblastoma but must be differentiated from metastatic disease. The cases of 2 siblings with multiple neuroblastomas are presented and 44 additional cases of neuroblastoma reviewed. It was found that primary tumors localize to the posterior mediastinum, the adrenals, and the paravertebral ganglia whereas metastatic disease is found in the anterior and middle mediastinum, bones and para-aortic nodes. Based on these observations, the diagnosis of multiple primary tumors can be made and should lead to early consideration of familial neuroblastoma.
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PMID:Familial neuroblastoma presenting as multiple tumors. 113 58

A 17-month-old Thai female with neuroblastoma presented with an abdominal mass and the classical findings of kwashiorkor. Concomitant with effective repair of the child's protein deficit, the mass enlarged dramatically and metastases were noted. This is the first known report of such an occurrence.
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PMID:Can the growth of a neuroblastoma be influenced by a child's nutritional state? Observations in a patient treated for kwashiorkor and later given a restricted diet. 115 41

Bond, J.V. (1975). Archives of Disease in Childhood, 50, 691. Clinical significance of catecholamine excretion levels in diagnosis and treatment of neuroblastoma. The pattern of urinary excretion of vanillylmandelic acid (VMA) was studied in 50 children with neuroblastoma. There were 10 disease-free survivors, and 4 children who survive for 3 or more years with residual nonmetastatic disease, 36 children died with widespread metastic disease. The urinary VMA level was raised at diagnosis in 8 of the 10 disease-free survivors but rapidly returned to normal after treatment. In 3 out of 4 children with residual tumour, VMA remains persistently raised. In 28 of the 36 children who died VMA was raised at diagnosis and remained so throughout the course of their disease. In the majority of the children with metastatic disease the finding of a raised VMA revealed the nature of the primary tumour. Measurement of urinary VMA is a simple diagnostic test which confirms the presence of neurblastoma and avoids the need for more complex investigations. The prompt return to normal levels in the disease-free survivors confirms the importance of serial VMA estimations in assessing response to treatment and ultimate prognosis. The other good prognostic features in the surviving patients were young age at diagnosis, primary tumour in the thorax, and histologically well-differentiated tumours which had not metastasized.
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PMID:Clinical significance of catecholamine excretion levels in diagnosis and treatment of neuroblastoma. 119 Aug 18

The roentgenologic diagnosis and differentiation of mediastinal neurogenic tumors are possible on the chest roentgenogram as a rule. The soft tissue mass may be ill-defined and the tumor "ghost-like" in the case of primary neuroblastoma, but it is usually obvious in ganglioneuroma and metastatic disease. The presence of calcifications differentiates neurogenic tumors from other posterior mediastinal tumors of childhood. They are common in primary and rare in secondary disease. Rib erosions and displacement are striking in neuroblastoma (after a few months of age), more subtle in ganglioneuroma, and absent with secondary involvement. In 3 out of 7 posterior mediastinal neuroblastomas the diagnosis and treatment were delayed, as the adjacent rib changes were not appreciated for some time. "Dumbbell" shaped tumors are usually associated with vertebral changes and myelography is indicated even in the absence of neurologic deficit. Thoracic deformity and disability subsequent to laminectomy, radiation therapy, or both, are present in all survivors.
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PMID:Mediastinal neuroblastoma and ganglioneuroma. The differentiation between primary and secondary involvement on the chest roentgenogram. 120 Feb 37

The murine C1300 neuroblastoma model has been evaluated as a possible model for children with widespread metastatic disease. Drug toxicity studies were conducted in adult A/J mice with various doses of antitumor agents. Adriamycin, BCNU, bleomycin, guanazole, acronycine, isophosphamide, DTIC, ICRF-159, cyclophosphamide, vincristine, and vinblastine were adminstered intraperitoneally to random groups of normal mice. After identification of appropriate doses, chemotherapy studies were conducted with varius regimens of drugs. Chemotherapy was administered to adult A/J mice when their subcutaneously implanted tumors measured 1.0-1.7 cm in diameter. Antitumor drugs can be classified into three groups according to drug efficacy. BCNU, cyclophosphamide, and isophosphamide were extremely active. Cytosine arabinoside was reported to be active against this murine tumor in a previous publication. Drugs with minimal activiyt which deserve further evaluation included adriamycin, guanazole, ICRF-159, DTIC, and vinblastine. Inactive drugs were acronycine, bleomycin, 5-fluorouracil, and vincristine. These experiments suggest that children with metastatic neuroblastoma may respond to cyclophosphamide, isophosphamide, and BCNU, while DTIC, adriamycin, ICRF-159, guanazole, and the vinca alkaloids may also be effective. The results suggest that agents selected by the C1300 model should be given adequate clinical trials.
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PMID:Murine neuroblastoma: further evaluation of the C1300 model with single antitumor agents. 120

Four patients with skeletal metastases from retinoblastomas are presented. Radiologically, the metastases showed periosteal new bone formation and permeative-type bone destruction, particularly involving the long bones of the extremities, and they tended to be bilateral. The metastases resembled those of neuroblastoma and, to a lesser extent, medulloblastoma. The relationship between these three tumors is discussed.
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PMID:Skeletal metastases from retinoblastoma. 122 22

The authors stress the difficulty of diagnosis of a neuroblastoma in its undifferentiated when the demonstration of nervous differentiation is necessary. The factors which may play a role in the prognosis of these tumours are analysed. In this series are included the age of the child, the location of the tumour, the existence of certain early metastases (bone), the possibility of complete surgical removal and alterations in the tumour secondary to chemotherapy and radiotherapy. Histoenzymatic study of histio and organotypic cultures as well as chromosomal analysis were possible in a number of cases.
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PMID:[Neuroblastoma in children. Anatomoclinical study of 46 cases]. 122 54

Seventeen patients with olfactory neuroblastoma seen at the Massachusetts General Hospital and Massachusetts Eye and Ear Infirmary from 1941 to 1971 are presented. Data on age, sex, symptoms, physical, and roentgenographic findings, and therapeutic results are evaluated. A system of pretherapy staging is proposed in which for Group A, the tumor is limited to the nasal cavity; in group B, the tumor is localized to the nasal acvity and paranasal sinuses; and in group C, the tumor extends beyond the nasal cavity and paranasal sinuses. Olfactory neuroblastoma is a radioresponsive, and, to a limited extent, radiocurable tumor and varies in aggressiveness. Of 17 patients in this series, 13 or 76% were alive without disease following treatment by surgery, irradiation, and combination of these two methods. Uncontrolled primary lesions with or without metastases accounted for all therapeutic failures. A treatment policy for this disease is presented.
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PMID:Olfactory neuroblastoma. A clinical analysis of 17 cases. 126 Jun 76

Primitive neuroectodermal tumor (PNET) is a small round cell malignancy arising in soft tissue and bone, predominantly in older children and adolescents. We report the cytomorphologic features and findings of ancillary studies of eight fine needle aspiration (FNA) biopsies from three patients (7-year-old male, 12-year-old female, 9-year-old female). Two of the biopsies suggested the initial diagnosis of PNET of the chest wall, while the remaining six documented recurrent or metastatic disease. In one of these cases the primary diagnosis made by FNA biopsy enabled the pediatric oncologists to give specific therapy for the unresectable tumor and achieve remission. Local recurrences included the chest wall (two cases), pleura (one case) and pericardium (one case), while metastatic disease involved the supraclavicular lymph node and breast. All the cases consisted of small malignant cells with a high nuclear/cytoplasmic ratio and hyperchromatic nuclei without prominent nucleoli. Homer Wright rosettes were seen in only two of the aspirates, and neuropil and ganglion cells were not present. Ancillary studies, including electron microscopy (two cases), immunocytochemistry (four aspirates from two cases) and cytogenetics (11/22 translocation, one case) performed on the aspirated material were aids in making a specific diagnosis and excluded other small round cell tumors of childhood, such as malignant lymphoma, rhabdomyosarcoma and Ewing's sarcoma. The differential diagnosis between PNET and neuroblastoma can be difficult on the basis of an FNA biopsy alone, although light microscopic morphologic differences exist. Clinical features (e.g., age, primary site, metastatic patterns), catecholamine levels, electron microscopy and cytogenetics are necessary in establishing the correct diagnosis.
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PMID:Fine needle aspiration cytology of primitive neuroectodermal tumors. A report of these cases. 132 99


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