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)

We reviewed the autopsy, clinical, and radiographic records of 39 patients with metastatic skeletal disease (age range 18 months-20 years). There were 11 different primary tumors responsible for skeletal metastases, of which neuroblastoma was the most common (16 patients). Other commonly occurring tumors giving rise to secondary skeletal deposits were rhabdomyosarcoma (seven), teratoma-teratocarcinoma (four), and Wilms tumor (three). Overall patient survival ranged from 2 weeks to 72 months, with an average of 10.2 months. Those patients presenting without skeletal metastases at the time of diagnosis of the primary tumor had a survival time averaging 16.2 months (range 3-72 months), whereas those patients presenting initially with skeletal metastases at the time of diagnosis of the primary tumor had a survival time of 8.5 months (range 0.5-23 months).
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PMID:Metastatic skeletal disease in the pediatric population. 298 4

Skeletal involvement in childhood nonosseous tumors can be due to primary involvement, arrosion or metastasis, secondary due to therapy induced alterations or osteomyelitis following diminished immunity. The occurence of bone changes differs widely from those in adults. Neuroblastoma, rhabdomyosarcoma and malignant lymphoma are discussed in detail. Rare tumors are listed for synopsis. As diagnostic screening method skeletal scintigraphy is recommended, whereas in localized disease X-rays should be performed. Beside roentgenmorphology-particularly in primary disease-localisation, frequency and age dependency may give essential diagnostic hints. Prognosis depends on primary tumor.
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PMID:[Skeletal manifestations of malignant, nonosseous tumors in childhood]. 298 8

Small, round, blue-cell tumors (SRCT), including rhabdomyosarcoma, Ewing's sarcoma of bone and soft tissue, mesenchymal chondrosarcoma, small cell osteosarcoma, hemangiopericytoma, neuroblastoma, peripheral neurectodermal tumor (peripheral neuroepithelioma of bone and soft tissue), and the malignant small cell tumor of the thoracopulmonary region described by Askin (Askin's tumor), are often difficult to distinguish by light microscopy. We have evaluated the cytogenetics of these tumors by studying 24 tumor explants in short-term culture and 22 tumor cell lines. In Ewing's sarcoma (a tumor of unknown histogenesis), and in peripheral neuroepithelioma and Askin's tumor (tumors with evidence of neural origin), we have observed an indistinguishable t(11;22) translocation.
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PMID:Cytogenetic characterization of selected small round cell tumors of childhood. 300 99

Modern multidisciplinary treatment of childhood cancer has made extent of disease evaluation important for proper treatment planning. Accurate staging is essential to cooperative group studies and for comparing treatment modalities at different centers. Operative staging plays an important role where clinical or imaging methods are limited, as in abdominal Hodgkin's disease or regional nodal metastasis. Operative staging is carried out either as a special diagnostic procedure, as in lymphoma, or as part of a planned surgical resection of a solid tumor. For lymphomas: Operative staging of abdominal Hodgkin's disease is required where protocols include involved field irradiation and sparing of normal growing tissue in the child. In non-Hodgkin's lymphoma, bulky abdominal tumor may be surgically evaluated after intensive chemotherapy either in delayed primary surgery or in second look procedures. Residual tumor may be excised or tagged with clips for localized irradiation to the tumor sparing normal abdominal organs. For solid tumors: During surgical resection of neuroblastoma, Wilms' tumor and rhabdomyosarcoma, the correct procedure involves regional staging either by formal node dissections or by multiple biopsies to determine extent of spread. Regional node dissections are often part of a correct cancer operation for cure, but also give staging information unobtainable by other methods. The surgeon must plan every procedure carefully with the aim of curing the patient and also deriving maximum information from the operation to enable correct planning of further treatment.
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PMID:The objectives and importance of operative staging of children with cancer. 301 92

We have investigated 56 histologic and 13 cytologic specimens of malignant round cell tumors of childhood. The immunohistological detection of intermediate filament polypeptides, neuron specific enolase (NSE) as well as leukocyte common antigen (LCA) allows the histological classification of such tumors. Neuroblastomas demonstrate a positive reaction with antibodies to neurofilaments and NSE independent of differentiation. Rhabdomyosarcomas could be labeled by the desmin antibody, while Ewing sarcomas, malignant lymphomas as well as nonmuscular sarcomas only express vimentin. In nephroblastomas the intermediate filament specific antibodies reveal expression of keratin and vimentin in blastema cells, while tubules are only labeled by the keratin antibody. In undifferentiated nephroblastomas, which lack formation of tubules blastema cells are keratin negative and vimentin positive. Thus antibodies to intermediate filaments, LCA and NSE seem to be useful tools to distinguish the so called "round cell tumors" of childhood.
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PMID:[Significance of immunohistologic methods in the differential diagnosis of solid tumors in childhood]. 301 2

Hypercalcemia, often associated with certain types of adult tumors, has also been described in pediatric neoplasms. In childhood, the more common associations include lymphoma, leukemia, rhabdomyosarcoma and rarely neuroblastoma. However, recently, several infants with hypercalcemia were described having renal tumors without bone metastases. The following is a case report of a 2-month-old infant who presented with severe hypercalcemia and a large right-sided abdominal mass, which at surgery was diagnosed as a cellular mesoblastic nephroma.
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PMID:Hypercalcemia in association with mesoblastic nephroma: report of a case and review of the literature. 302 25

Melphalan (L-phenylalanine mustard) is a bifunctional alkylating agent that is commonly administered orally to treat a wide variety of malignancies, including cancers of the breast and ovary, as well as multiple myeloma. Although commercially available in Europe and Canada, intravenous (IV) melphalan remains investigational in the United States. The role of IV melphalan in cancer chemotherapy is not well defined, despite its manageable toxicity and higher and more predictable blood levels following IV administration compared with oral administration. In addition, unlike oral melphalan, an extensive phase I evaluation of IV melphalan has not been undertaken. At lower doses (eg, 30 to 70 mg/m2), both as a single agent and in combination, the activity of IV melphalan has been evaluated in only a limited number of diseases. However, striking activity has been observed in previously untreated patients with rhabdomyosarcoma, a disease not generally considered responsive to alkylating agents. When administered at high doses (greater than 140 mg/m2) requiring bone marrow reinfusion, melphalan effects a high response rate (but no improvement in survival) in a variety of nonhematologic tumor types, including resistant tumors such as melanoma and colon carcinoma. In contrast, in poor-prognosis patients with non-Hodgkin's lymphoma, Hodgkin's disease, multiple myeloma, or neuroblastoma, high-dose melphalan-containing regimens have yielded both high response rates and improved survival, despite considerable toxicity. Additional clinical trials will be necessary to define the spectrum of activity of lower doses of IV melphalan and to define subgroups of patients most likely to benefit from high-dose melphalan.
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PMID:The systemic administration of intravenous melphalan. 305 5

Autologous bone marrow transplantation is a procedure that allows for the delivery of high doses of chemotherapy and radiation to treat pediatric malignancies. There have been many studies showing a dose-response curve for many of the drugs that have been used for cytoreductive therapy in autologous bone marrow transplantation. These dosage ranges are achievable in many of the preparative regimens used in autologous bone marrow transplantation. The results in neuroblastoma, Hodgkin's disease, non-Hodgkin's lymphoma, Ewing's sarcoma, rhabdomyosarcoma, osseous sarcoma, other soft tissue sarcomas, and acute leukemias (acute lymphoblastic leukemia and acute non-lymphoblastic leukemia) are reviewed. The question of purging of bone marrow during autologous bone marrow transplantation is addressed. The different techniques of purging are reviewed and the advantages and disadvantages of each are discussed. Finally, new areas of treatment and future directions of autologous bone marrow transplantation are addressed.
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PMID:Autologous bone marrow transplantation in children. 306 35

Ifosfamide was given to 61 patients with malignant solid tumors diagnosed before the age of 21 years. In this phase II study, all patients received 1.6 g/m2/day X 5 iv over 15 minutes followed by mesna at a dose of 400 mg/m2 iv at 15 minutes and 4 and 6 hours after ifosfamide. Responses were observed in five of 15 patients with osteosarcoma, two of ten with neuroblastoma, two of six with Wilms' tumor, two of five with rhabdomyosarcoma, four of eight with other soft tissue sarcomas, one of one with retinoblastoma, one of two with germ cell tumors, one of one with B-cell lymphoma, and one of one with a primitive neuroectodermal tumor. Fifty-nine of 61 patients had received prior alkylating agent therapy which included cyclophosphamide, cisplatin, mechlorethamine, melphalan, or dacarbazine. Fourteen of 19 responses developed in patients whose tumors were resistant to treatment with cyclophosphamide. A patient with malignant Schwannoma who had received no prior chemotherapy developed a complete response which lasted 12 months. A patient with brain metastases of osteosarcoma has had complete response for greater than 2 years. Complete response was also observed in a patient with B-cell lymphoma. Toxicity consisted of mild to moderate nausea and vomiting, transient reversible myelosuppression, occasional elevation of serum BUN or creatinine, and transient neurotoxicity characterized by somnolence, confusion, weakness, tremor, hallucinations, or seizures. We conclude that ifosfamide is an important alkylating agent without apparent complete cross-resistance with cyclophosphamide, and as such should be further investigated for determination of its activity in patients with pediatric neoplasms and considered for incorporation into phase II-III trials for certain tumors.
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PMID:Phase II trial of ifosfamide in children with malignant solid tumors. 310 34

Children undergoing ABMT, a procedure which entails massive doses of chemotherapy along with total-body irradiation, are candidate to develop severe gastrointestinal toxicity and prolonged anorexia requiring administration of Parenteral Nutrition (PN) for variable periods. We report a series of 35 consecutive children affected by malignancies who underwent 37 courses of PN after ablative therapy followed by ABMT. Age ranged from 8 months to 17 years; 16 were females, 19 males. There were 23 cases of neuroblastoma, 5 of Wilms' tumor, 3 of acute myelogenous leukemia, 2 of Ewing's sarcoma, 1 case each of rhabdomyosarcoma and acute lymphoblastic leukemia. All patients developed severe neutropenia for 9-42 days (median 18 d). Fever occurred in all patients; sepsis was documented in 10. Duration of PN ranged from 10 to 64 days (23 +/- 9; mean +/- SD). PN solution, containing crystalline L-Aminoacids (8.5%) mixed with 33% glucose, minerals, trace elements and vitamins provided for children a caloric intake of 49.8 +/- 17.3 Kcal/Kg/day with a nitrogen intake of 0.26 +/- 0.27 g/Kg/day. Nutritional assessment, utilizing percent ideal body weight, serum protein electrophoresis, C3, pseudocholinesterase and fibrinogen, was performed at the beginning and at the completion of each course of PN. Mean percent ideal body weight was 95.8 before PN, 98.5 on last day of PN (p less than 0.0005). Other parameters did not change significantly. No metabolic complication nor severe electrolyte imbalance were observed except for 5 patients who developed hypokalemia in coincidence with administration of Amphotericin B.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Autologous bone marrow transplantation in children. Use of parenteral nutrition]. 311 38


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