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)

The treatment of Wilms' tumor is based on initial surgical removal followed by clinical and histologic staging. Chemotherapy provides the major adjunctive therapy in virtually all Wilms' tumors, radiotherapy being used in some situations. Exceptions to this are the treatment of bilateral Wilms' tumors and large intracaval extension. Bilateral Wilms' tumor is treated with initial biopsy and staging, adjunctive chemotherapy, and/or radiation therapy and bilateral partial nephrectomy after there is maximum resolution of tumor. Similarly, extensive caval extension of tumor may be treated preoperatively with chemotherapy and radiotherapy followed by resection. Nephroblastomatosis, a precursor of Wilms' tumor, is a common associated finding at exploration. It requires alteration in management and may change the prognosis. Sarcomas of the kidney and congenital mesoblastic nephroma represent the spectrum of severity of solid renal masses in children. Neuroblastoma is the most common solid tumor in children, and its prognosis is largely dependent on the age of the patient and the stage of disease. Chemotherapy and radiotherapy is adjunctive treatments have been disappointing. Immunotherapy holds some promise for the future. Testicular tumors are unusual in children. Those that occur in infancy are most often benign teratomas that require orchiectomy alone. Malignant germ cell tumors in children are most often yolk sac tumors and respond to surgery and chemotherapy. Lymph node dissection is indicated only in paratesticular rhabdomyasarcoma. Other genitourinary rhabdomyasarcomas occur in the bladder, prostate, vagina, and uterus. After maximum decrease in tumor volume with chemotherapy and radiotherapy, surgical exploration and resection of remaining tumor probably represent the best form of treatment. Organ-sparing procedures should be carefully selected in that they may worsen the prognosis.
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PMID:New concepts in the treatment of genitourinary cancer in childhood. 267 29

Of a total of 1561 patients registered in the Intergroup Rhabdomyosarcoma Study (IRS) as of May 1983, 78 patients (5%) were younger than 1 year of age. These infants did not differ from the older children (1-20 years of age) in male/female ratio, clinical grouping, or survival rates. Infants younger than 1 year of age had a significantly greater frequency of undifferentiated sarcoma, 18% versus 7% in older children (P less than 0.005). Infants also had a significantly greater proportion of cancers with botryoid pathology, 10% versus 4% in older children (P less than 0.005). When reviewed by the newly proposed IRS cytopathologic classification, there was no difference in pathologic types between the two age groups. Infants younger than 1 year of age had a higher rate of bladder-prostate-vagina primary tumor sites than older children, 24% versus 10% (P less than 0.05). In addition, infants tended to receive less of the prescribed doses of chemotherapy and radiation therapy than older children, and to develop more toxicity to treatment than older children. Despite these differences, the overall survival curve for the two age groups appears to be similar. In contrast to Wilms' tumor and neuroblastoma, in which age (less than 1 year) is a favorable prognostic factor, age does not appear to be an important prognostic factor in rhabdomyosarcoma.
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PMID:Infants younger than 1 year of age with rhabdomyosarcoma. 377 10

Organ preservation is one of the requirements for maintaining the high quality of life after the treatment of malignant solid tumors in children. We analyze our recent results of renal preservation in the abdominal neuroblastoma and describe 3 cases of successful organ preservation in pelvic malignancy. Between 1982 and 1996, 29 patients with adrenal or retroperitoneal neuroblastoma, that extended into the surrounding tissues and organs and/or to the contralateral side, underwent delayed primary excision. Before 1982, 9 patients were treated and only one ipsilateral kidney was preserved. On the other hand, 13 of 20 kidneys were preserved after 1987. Adoption of cis-platinum deepened our awareness of preserving the kidney. Furthermore, we have altered our strategy since 1987 to continuing preoperative chemotherapy until the size of the tumor becomes as small as it is estimated completely resectable by our own index, which is derived from computed tomography. Kidneys are shielded during intraoperative irradiation, and the tumor relapsed from the shielded area of retroperitoneum in one patient. Four of 14 preserved kidneys became atrophic and lost their function. The bladder and the rectum were left intact at tumor resection after intensive chemotherapy in two patients with pelvic (one retroperitoneal and one vaginal) rhabdomyosarcoma. A yolk sac tumor of the vagina responded completely to chemotherapy with cis-platinum, etoposide and bleomycin. The patient has survived 7.5 years without any local and distant relapse in spite of the fact that surgery, aside from several diagnostic biopsies, was not undertaken.
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PMID:[Organ preservation in the treatment of malignant solid tumors in children]. 912 2