Wilms tumor in a child with trisomy 13

A 4-year-old black boy with trisomy 13, a history of frequent urinary tract infections, and a horseshoe kidney with painless gross hematuria was examined. An abdominal mass was detected and surgically resected. Examination of the surgical specimen revealed a Wilms tumor. Given the concurrence of trisomy 13 and Wilms tumor and the presence of another such case in the literature, there may be just cause to suspect a locus on chromosome 13 that affects the probability of developing Wilms tumor. Given the increasingly longer survival of patients with trisomy 13, clinicians may need to be aware of the possibility of renal malignant disease in this population of patients.     

Screening for Wilms tumor in children with Beckwith-Wiedemann syndrome or idiopathic hemihypertrophy

BACKGROUND: Children with Beckwith-Wiedemann syndrome and idiopathic hemihypertrophy (BWS/HH) are at increased risk for developing Wilms tumor and screening with abdominal sonography is frequently recommended. However, there is a paucity of published data supporting this strategy. The purpose of this study was to determine whether sonographic screening at intervals of 4 months or less reduced the proportion of late-stage Wilms Tumor (WT) in children with BWS/HH. PROCEDURE: A case series analysis was employed to compare the proportion of late-stage (stage III or IV) Wilms tumor in patients with BWS/HH who were screened with sonography (n = 15) to the proportion of late-stage Wilms tumor in unscreened patients with BWS/HH (n = 59). Patients were identified from the BWS Registry and from previously published studies. Screened patients had sonograms at intervals of 4 months or less. RESULTS: None of the 12 screened children with Wilms tumor had late-stage disease, whereas 25 of 59 (42%) of unscreened children had late-stage Wilms tumor, a difference that was statistically significant (P < 0.003). Three children had false positive screening studies. They were operated on for suspected Wilms tumor but the lesions proved to be complicated renal cysts (n = 2) or nephroblastomatosis (n = 1). CONCLUSIONS: This study suggests that children with BWS/HH may benefit from screening sonograms at intervals of 4 months or less. However, false positive screening exams may result in unnecessary surgery. Given the rarity of BWS/HH, a larger, prospective international screening study is necessary to determine if the benefits of screening outweigh the risks.     

Renal cell carcinoma as a second malignant neoplasm in a patient with non-syndromic hemihypertrophy and previous Wilms tumor

Survivors of childhood Wilms tumors are at an increased risk of second malignant neoplasms. Recently, it has been postulated that renal cell carcinoma is among the malignancies for which this population is at risk. We present the unique case of an adult Wilms tumor survivor with non-syndromic hemihypertrophy (NSHH) who developed renal cell carcinoma. This case highlights the need for close follow-up in two populations: adults who have survived Wilms tumor and those with NSHH.     

Screening of children with hemihypertrophy,aniridia, and Beckwith-Wiedemann syndrome in patients with wilms tumor: A report from the national Wilms tumor study

To evaluate the usefulness of regular radiographic screening to detect an asymptomatic intraabdominal tumor in patients with an increased risk of developing Wilms tumor, we reviewed the files of patients with hemihypertrophy, aniridia, or Beckwith-Wiedemann syndrome who were registered on the National Wilms Tumor Studies. Screening was employed infrequently in the management of children with hemihypertrophy, with only 25% (6/24) of those whose hemihypertrophy was identified more than 30 days prior to the diagnosis of Wilms tumor undergoing such examinations. Most patients with aniridia were evaluated regularly for the occcurrence of Wilms tumor. There were more stage 1 tumors identified in patients whose tumor was detected only through radiographic evaluation. The role of routine radiographic screening needs to be carefully evaluated in a homogeneous group of patients such as those with aniridia using a prospective study design to determine if such screening improves the survival rate of children with this rapidly growing, but readily treatable form of childhood cancer. © 1993 Wiley-Liss, Inc.     

High-dose methylprednisolone treatment of hepatic veno-occlusive disease in a child with Wilms tumor

Veno-occlusive disease (VOD) is a rare complication of chemotherapy in children with cancer. In the literature successful treatment of severe VOD has not been well determined. The authors report a 5-year-old boy with Willms tumor whose condition was complicated with VOD while receiving chemotherapy. He was treated successfully with high-dose methylprednisolone. After therapy, the clinical signs of VOD, such as abdominal ascites, oliguria, and jaundice, disappeared without any adverse effects of therapy.     

Solid and cystic tumor of the pancreas in a child

A solid and cystic tumor of the pancreas in a 10-year-old girl was removed completely by pancreatectomy. Immunohistochemical and electron microscopic examinations disclosed acinar cell origin. Thirteen collected cases of these tumors in children whose diagnoses were confirmed by histochemistry and electron microscopy were reviewed. More conservative surgery may be recommended because of their benign nature. Offprint requests to: Y. Hata     

Adriamycin cardiomyopathy in a child with Wilms tumor and liver metastases (author's transl)]

Nephrectomy with irradiation was performed in a 3;2 year old boy with Wilms tumor stage IV (livermetastases (liver metastases). Six months later a hemihepatectomy was performed on account of the liver metatases which persisted under a combined Actinomycin Vincristin therapy. A monotherapy with Adriamycin was instituted. The patient developed acute congestive heart failure. The patient developed acute congestive heart failure and showed typical EKG changes after a total dose of 700 mg/M2 of Adriamycin. In comparison to adults children reportedly tolerate somewhat higher total doses of Adriamycin. Therefore it is discussed, if in this case a limitation of liver function by hemihepatectomy and by irradiation has favored the appearance of the Adriamycin-induced cardiomyopathy.