Cases reported "Wilms Tumor"

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1/7. Treated Wilm's tumor in childhood as potential risk factor for second thyroid cancer.

    The potential risk of a treatment-induced second neoplasia affecting the thyroid is well known after radiation therapy for several types of cancer, but few cases have been related to incidental irradiation for Wilms' tumor. We report a case of a papillary thyroid carcinoma discovered in a young patient 15 years after treatment of a Wilms' tumor. An 18-year-old man was referred to our Endocrinological Department for a single 3 cm nodule in the right lobe of the thyroid. His past medical history included at the age of 2 years surgical resection, chemotherapy (actinomycin-D and vincristine) and cesium radiation therapy to the right side for a Wilms' tumor in stage III: a total dose of 7700 rads was delivered to an area of 17 x 10 cm in the right flank. After fine-needle demonstration of a follicular thyroid lesion, the patient underwent right lobectomy, followed by total thyroidectomy for histologic diagnosis of a follicular variant papillary cancer. Residual thyroid tissue was ablated by iodine-131 administration (3700 MBq), but scanning after therapeutic iodine showed radioactive uptake in the left regional lymph nodes, with elevated serum thyroglobulin off therapy (830 ng/ml). magnetic resonance imaging confirmed the presence of lymph node enlargements and bilateral neck dissection was performed, followed by radioiodine treatment (3700 MBq) and thyroxine suppressive therapy. After 3-year follow-up the patient is disease-free. Although few cases of thyroid cancer have been reported in the literature after irradiation for a Wilms' tumor during childhood, this association should be considered in the long-term follow-up.
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2/7. Parotid carcinoma after autologous bone marrow transplantation for relapsed nephroblastoma.

    Abdominal irradiation, especially if associated with doxorubicin administration, increases the risk of a secondary malignant neoplasm (SMN) after treatment of nephroblastoma. Secondary malignant salivary tumors are rare and usually occur in patients with previous cranial irradiation. The authors describe the case of a parotid mucoepidermoid carcinoma arising 13 years after diagnosis of nephroblastoma. This patient showed no characteristics reported in the literature as statistically significant for the development of an SMN. The authors believe that long-term, regular clinical examination is necessary even in patients at low risk of developing an SMN.
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3/7. Severe hepatic toxicity after treatment with single-dose dactinomycin and vincristine. A report of the National Wilms' Tumor Study.

    dactinomycin is an antitumor antibiotic with known activity against many pediatric solid tumors. Administration of dactinomycin using a single-dose schedule was incorporated into the design of the National Wilms' Tumor Study 4 (NWTS-4). This was done to determine whether laboratory and preliminary clinical data, suggesting that such a schedule was associated with increased antitumor effect and/or decreased normal tissue toxicity, could be validated in a large clinical trial. Five patients treated with regimens EE-4 or K-4, regimens that included single-dose dactinomycin and no abdominal irradiation, experienced severe hepatic toxicity. The clinical courses of these patients suggested that multiple factors, including the administration of other hepatotoxic agents, contributed to the toxicity observed. The study has been modified by decreasing the dose of dactinomycin from 60 micrograms/kg to 45 micrograms/kg. Further evaluation of other potential contributing factors, such as the use of halogenated hydrocarbon inhalational anesthetic agents, is needed.
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4/7. Severe encephalopathy associated with ifosfamide administration in two children with metastatic tumors.

    We studied two children with recurrent cancer, who during treatment with ifosfamide developed severe encephalopathy characterized by coma of several days duration. ifosfamide was used as a single drug without mesna as an uroprotector. A 1-hour infusion of ifosfamide (1.8 g/m2) was given on days one and two of a planned 5-day course. Encephalopathy was associated with severe electroencephalographic abnormalities, e.g. slowing in delta range in one patient and electrographic seizures in another. Both, clinical and electroencephalographic features of encephalopathy were reversible.
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5/7. Cutaneous toxicity following the administration of dactinomycin.

    dactinomycin (AMD) is an effective drug in the management of several malignant disorders and has been used for almost 40 years. skin and subcutaneous toxicities following extravasation are well known and can be harmful. Similarly radiation-recall is a well established phenomenon following the administration of AMD. We report a patient who developed a localized brawny erythema in the crural folds and the axillae, likely due to AMD. This rare skin complication of AMD seems to benefit from topical corticosteroid treatment, although postinflammatory hyperpigmentation may take months to disappear.
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6/7. Asymptomatic adult Wilm's tumor (nephroblastoma) incidentally detected by CT.

    Wilms' tumor (nephroblastoma), the most common renal neoplasm in children, is rarely found in adults. A 73-year-old woman with asymptomatic adult Wilms' tumor, incidentally detected by CT, is reported. CT and MRI showed a small mass with homogeneous enhancement after the administration of contrast medium. ultrasonography demonstrated a well-defined echogenic mass with a halo-like, peripheral hypoechoic area. Selective angiography showed no tumor vessels. Although renal cell carcinoma should be considered in the differential diagnosis, it is still difficult to distinguish from small Wilms' tumor like this case.
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7/7. Toxicity, pharmacokinetics, and in vitro hemodialysis clearance of ifosfamide and metabolites in an anephric pediatric patient with Wilms' tumor.

    PURPOSE: We evaluated the in vitro hemodialysis ratio and subsequent toxicity and pharmacokinetics of ifosfamide in an anephric patient with Wilms' tumor. methods: An in vitro model was used to determine the extraction ratio of ifosfamide by dialysis. The toxicity and plasma concentrations of ifosfamide, chloroacetaldehyde, and 4-hydroxyifosfamide were then determined over 24 h after a single 1.6 g/m2 dose of ifosfamide. plasma concentrations were also measured before and after ten dialysis sessions during four courses of ifosfamide therapy. RESULTS: The in vitro hemodialysis model showed that ifosfamide was cleared with an extraction ratio of 86.7 /-0.5% and remained constant even at low concentrations of drug. The mean decrease in vivo following hemodialysis for ifosfamide, chloroacetaldehyde, and 4-hydroxyifosfamide were 86.9%, 77.2%, and 36.2%, respectively. The pharmacokinetic parameters for ifosfamide using model-independent methods were calculated: Vd = 0.23 l/kg, t1/2 = 4.8 h, and ClT = 3.30 l/h per m2. ifosfamide-associated neurotoxicity was noted within hours of drug administration and improved rapidly following hemodialysis. CONCLUSIONS: The results of our study suggest that the pharmacokinetics of parent ifosfamide may not be substantially altered in patients with renal failure. Hemodialysis was shown to remove ifosfamide, chloroacetaldehyde, and 4-hydroxyifosfamide from the blood stream. Hemodialysis was also shown to reverse ifosfamide-related neurotoxicity.
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