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)

Forty-two infants and children with retroperitoneal solid tumors underwent second operative procedures. Twenty-three children had stage III (4) or stage IV (19) neuroblastoma and 19 had Wilms' tumor. This report suggests that second-look procedures are quite beneficial in selected cases. In children with Wilms' tumor, second procedures are most useful in those patients inadequately staged with flank operations, in cases of bilateral tumors, and in children with initially unresectable tumors following cytoreduction. Patients with significant tumor spill at a previous procedure may also benefit from early reoperation. Late recurrence of tumor, especially in children with unfavorable histology and/or hematogenous liver metastases, carries an ominous prognosis. Ten of the 19 patients with Wilms' tumor are long-term survivors (52.6%). Patients with initially unresectable (stage III) neuroblastoma occurring in the pelvis respond well to second-look resection. Children with primary tumors surrounding the celiac axis eventually died despite subsequent resection. Although the efficacy of primary tumor resection in cases of metastatic neuroblastoma remains controversial, stage IV patients with tumor resection had a longer duration of survival. Our only long-term survivors had their primary tumors resected. Second-look or delayed abdominal operations in metastatic cases may be useful in assessing the effectiveness of treatment and for sampling of retroperitoneal lymph nodes as a prognostic indicator.
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PMID:The role of second-look procedures in the management of retroperitoneal tumors in children. 609 86

A 1 1/2 year old child developed profuse watery diarrhoea, shown to be due to excessive plasma vasoactive intestinal peptide (VIP) levels, whilst on treatment for metastatic neuroblastoma. Because it was unresponsive to alternative treatment, an attempt was made to control the diarrhoea with a somatostatin infusion. The attempt failed despite the fact that serum VIP levels were substantially reduced. Possible reasons for failure are discussed and the importance of plasma VIP as a marker for maturation in neuroblastoma emphasised.
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PMID:Intractable diarrhoea in a patient with vasoactive intestinal peptide-secreting neuroblastoma. Attempted control by somatostatin. 611 74

Ten children with recurrent metastatic (stage IV) neuroblastoma received local radiation therapy, supralethal chemotherapy, and total-body irradiation. Rescue with infusions of either allogeneic (four patients) or autologous (six patients) bone marrow followed. The drugs given to the first two patients were individualized combinations based on previous tumor responses. Both patients died with recurrent tumor three and nine months posttransplant. The eight remaining patients were treated more uniformly with local irradiation, VM-26, doxorubicin, melphalan (L-phenylalanine mustard), and 1,000-rad total-body irradiation in three fractions. Two of these patients had cardiac dysfunction and received no doxorubicin. Three children died in the immediate posttransplant period with disseminated fungal infections. A fourth relapsed and died nine months posttransplant. As of December 1, 1983, two children who received allogeneic marrow grafts have survived in complete remission for 54 and 36 months, and two children who received autologous marrow grafts have survived in complete remission for 35 and 22 months. These results suggest that relapsed metastatic neuroblastoma can be controlled by supralethal combinations of chemotherapy and irradiation coupled with bone-marrow rescue.
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PMID:Treatment of advanced neuroblastoma with supralethal chemotherapy, radiation, and allogeneic or autologous marrow reconstitution. 637 56

Neuroblastoma (NB) is a common tumor of childhood, presenting "unique" characteristics: i.e., different prognosis in relation to age, high rate of metastases at diagnosis, capacity of spontaneous regression, strong immunogenicity. The embryologic derivation of NB has been recently clarified: NB derives from the embrional sympathetic nervous tissue; its enzymatic activity is determined mainly by environmental factors. A number of clinical and laboratory aspects influence the fate of children with NB: extention of disease and age are the most important, followed by site of primary, histology, pattern of metastatic spread, immunologic characteristics. Among laboratory tests, many are correlated with the clinical course: urinary excretion of sympathetic amines, serum levels of ferritin, C3 complement fraction, LDH, IgM, neurono-specific enolase. In the recent years the development of monoclonal antibodies techniques has greatly improved. In NB, a number of membrane molecule determinants have been discovered, against which specific monoclonal antibodies can be profitably directed for diagnostic and therapeutic purposes. NB cells grow in vitro in the soft agar system; in this assay resistance and sensitivity of tumor cells can be tested with sufficient accuracy and may predict drug effect in vivo. Therapy of disseminated neuroblastoma is unsatisfactory till now. Promising techniques include autologous or allogeneic bone marrow infusion following supralethal chemotherapy, and administration of substances, such as retnoids, able to promote neuroblastoma cells differentiation in vivo.
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PMID:[Neuroblastoma]. 639 28

Metastatic neuroblastoma cells in bone marrow aspirates were examined by the formaldehyde induced fluorescence histochemical method. With this method we could easily identify abnormal cells as metastatic neuroblastoma cells by observing catecholamine green colored fluorescence in their cytoplasma. This formaldehyde induced fluorescence histochemical method is significantly useful for the diagnosis of metastatic neuroblastoma of the bone marrow.
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PMID:Detection of metastatic neuroblastoma cells in bone marrow aspirates by formaldehyde induced fluorescence histochemistry. 665 Feb 26

A case of neuroblastoma in an infant who showed evidence of spontaneous incomplete regression and subsequent relapse is reported. Interpretation of this patient's course is that the patient's tumor was heterogeneous in terms of cell compartments with susceptibility to spontaneous lysis. It is assumed that later activation of a cell compartment with uncontrollable proliferation occurred. Problems of assigning risk to infants like this include the need to have better biochemical or biological markers which will distinguish those infants who will have progressive disease, or will temporarily remit and then relapse from those who will spontaneously and permanently remit. Until appropriate risk assignment and early diagnosis of tumor recurrence are available, infants with disseminated neuroblastoma should receive limited treatment with chemotherapy or radiation at least until tumor regression is documented.
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PMID:Death from progression following spontaneous regression of infantile neuroblastoma. 665 Dec 33

We studied the relation between the DNA content of neuroblastoma cells and the response to therapy in 35 infants under one year of age with a diagnosis of neuroblastoma. Using flow cytometric techniques, we found that in 27 cases the primary malignant stem line consisted of neuroblasts with hyperdiploid DNA content, ranging from 1.07 to 2.42 times the finding in normal diploid cells. All remaining cases had diploid stem lines. Diploidy was more common in infants with clinical Stage D neuroblastoma (metastases beyond regional lymph nodes) than in those with other, less advanced stages: 6 of 10 as compared with 2 of 25 (P = 0.003). Of 17 evaluable patients with unresectable hyperdiploid tumors, 15 had complete responses and two had partial responses to cyclophosphamide and doxorubicin; six others with diploid tumors did not respond (P = 0.00001). We also found that each of the four infants with Evans' Stage IV-S neuroblastoma, an unusual form of disseminated neuroblastoma with a relatively good prognosis, had hyperdiploid tumor cells of clonal origin. We conclude that in neuroblastoma of infants, hyperdiploidy of tumor cells is associated with a better response to chemotherapy than is diploidy.
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PMID:Cellular DNA content as a predictor of response to chemotherapy in infants with unresectable neuroblastoma. 673 17

Chromosomes were prepared directly from primary tumor and bone marrow aspirates of an 8 months old infant and a 2 years old boy with metastatic neuroblastoma. A total of 765 tumor metaphases were counted after Giemsa-banding and partially karyotyped. "Double minute" chromosomes were found in the metaphases of both primary tumor and metastases of the infant, but only in the primary tumor of the older child. In both patients, structural imbalances (in addition to other translocations) were seen in the short arm of chromosome no. 1. The modal chromosome number was 46 in the infant's primary tumor and bone marrow metastases, but 84 in the primary tumor and 46 and over 100 in the bone marrow metastasis of the older child. Clonal evolution of metastasizing neuroblastoma cells is discussed.
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PMID:[Chromosomal studies in metastatic neuroblastoma]. 676 16

Thirty-three children with Evans stage IV neuroblastoma were treated with an intensive chemotherapy regimen reported by Helson to be highly effective. The purpose of the study was to determine whether the toxic regimen was manageable by different investigators and to increase the sample of patients. Remission induction therapy consisted of courses repeated every four weeks: Cyclophosphamide (CTX) 80 mg/kg IV, with IV fluids, and furosemide on days 1 and 2; vincristine (VCR) 0.03 mg/kg IV 12 hours after cyclophosphamide; trifluoro-methyl-2-deoxyridine (F3TdR) 45 mg/kg IV push, and papaverine (PAP) 45 mg/kg (12-hour infusion) under cardiac monitoring on days 3 and 4. Initially during maintenance, courses of therapy were reduced to two days. Because this was found to be ineffective therapy, the courses were extended to four days. Some of the patients who achieved response were removed from the protocol and placed on different maintenance therapy. Seventeen of 21 children newly diagnosed and 6/12 children previously treated for metastatic neuroblastoma achieved partial or complete remission. Eight of 16 newly diagnosed patients achieving response are still alive, six without evidence of disease for periods of time ranging from 20 to 41 months. The median of the administered drug dosages was 100% of the recommended dosages. Seventy percent of the 229 courses given were initiated at correct interval. Therapy had to be delayed on the others because of toxicity. The value of the four-drug combination is limited because of side effects related to myelosuppression which resulted in severe complications and frequent hospitalizations.
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PMID:Intensive chemotherapy for metastatic neuroblastoma: a Southwest Oncology Group study. 678 Jul 76

We performed coagulation studies in 16 patients with advanced neuroblastoma. Four of these patients had major hemorrhagic or thromboembolic complications. Abnormal coagulation screening tests were seen in all patients with active metastatic disease. We also measured plasma fibrinopeptide A, a sensitive measure of intravascular thrombin generation. Increased concentrations of fibrinopeptide A were found in each patient studied with active metastatic disease. Coagulopathy is a frequent finding in metastatic neuroblastoma and may cause severe morbidity. Laboratory studies suggesting either hyper- or hypocoagulability are frequent findings.
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PMID:Coagulopathy of disseminated neuroblastoma. 687 12


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