A Case of Renal Cell Carcinoma(Adenocarcinoma) in Nine Years Old Girl

A 9 years old girl has been admitted to hospital because of 5 days history of gross hem aturia. Physical examination on admission, She was eassentially normal except enlarged tonsils. Hb, Hct, WBC and Platelets were also nornal, ESR was 5mm/Hr. ASO titer was less than 1:60, C3 was 58mg%(normal:43-200mg%), BUN 11mg%, Creatinine 0.8mg% Serum cholesterol 160mg%, Total Protein 7.1mg% with albumin 4.9mg%, 24 hours urine protein 209mg and creatinine clearance 84ml/min/1.7m2. PT and PTT were also within normal range. Throat and urine cultures revealed no microorganisms. Excretory urogram, renal scan, renal angilgram showed 3.5X4cm sized space occuupying lesion on right upper pole of kidney. Right neprectomy was performed under impression of intramural wilms' tumor and followed intravenovenous injection of actinomycin D gamma/kg. Histopathological finding revealed partially clear dell type renal cell carcinoma which is extremely rare in pediatric age. Regional lymphnode and vessels disclosed no malignant cells. She received 3,000 rad radiati on on tumor bed on the 3rd postoperative day and two weeks later, hemotherapeutics were followed with actinomycin D 15 gamma/kg for 5 days per week for total 8 weeks and vincristine 1.5mg/M2/week for total 12 weeks. Six months later afeter completion of above treatment, she is doing well and attending to school.

A Case of Uhls Anomaly

Congenital hypoplasia of the right ventricular myocardium, also known as parchment heart or Uhl's anomaly, is a rare congenital heart defect. It was first described in Oslers principles and Practice of Medicine in 1905 and reviewed by Segall We found 29 reported cases, but there was no reported case in Korea. Recently, we experienced a case of Uhls anomaly associated with patent ductus arteriosus, atrial septal defect, pulmonary atresia and tricuspid abnormality in a 3 month-old infant, which was confirmed by autopsy. We present a case with a brief review of litteratures.