Here we reviewed the molecular genetic mechanism in the development of 4 types of human hereditary kidney cancers. These include von Hippel-Lindau (VHL) disease, hereditary papillary renal carcinoma, familial renal cancers with translocation of chromosome 3, and Tuberous sclerosis. Loss of function of the VHL disease gene is responsible for the von Hippel-Lindau disease and major portion of sporadic clear cell renal carcinoma. Activated c-Met oncogene is responsible for the development in some cases of hereditary papillary renal cell carcinomas and sporadic papillary renal carcinomas. There are several cases of familial renal carcinoma in that translocations of chromosome 3 p are demonstrated. The molecular genetic mechanism of this disease is not known. Several reports show the development of renal cell carcinoma in Tuberous sclerosis patients. TSC 1 or TSC 2 gene may be responsible for these tumors. The detail in this disease not well known. Molecular genetic analyses for hereditary renal cancer identified several oncogenes and tumor suppressor genes in hereditary as well as sporadic renal carcinomas. Future studies may reveal new category of oncogenes or tumor suppressor genes that are involved in the human kidney cancer development.

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(Purpose) To evaluate the results of radical retropubic prostatectomy in patients treated at a single institution.
(Materials and Methods) Between April 1985 and July 1997, 76 patients with prostate cancer underwent radical retropubic prostatectomy, including 73 receiving pelvic lymphadenectomy. The median age and follow-up time were 68 years old and 44 months, respectively. The pathological stage was pT0 in 6 patients, pT2 in 29, pT3 in 39, pT4 in 2, and pN+ in 22.
(Results) The surgical margin was positive in 10% of the pT2 patients and 61% of the pT3 patients. Twelve patients had recurrence. Recurrence was shown by biological failure in 4 patients and clinical failure in 8. The disease-free 5-year survival rates (Kaplan-Meier) were 100% in pT0 patients, 87% in pT2, 72% in pT3, 50% in pT4, 77% in pN-, 75% in pN+, 73% for a positive surgical-margin, and 83% for a negative surgical-margin. There were no statistical differences between any of these factors. However, the disease-free survival rate in pT3 patients with poorly differentiated adenocarcinoma (PDA) who received postoperative radiotherapy combined with hormonal therapy was significantly superior to that in patients with the same characteristics who received hormonal therapy (100% vs 27%; p=0.011). The cause-specific 5-year survival rates were 100% in pT0, 100% in pT2, 92% in pT3, 50% in pT4, 94% in pN-, 93% in pN+, 93% for a positive surgical-margin, 98% for a negative surgical-margin, 100% in the aforementiond pT3 patients with PDA and postoperative radiotherapy combined with hormonal therapy and 86% in pT3 patients with PDA and postoperative hormonal therapy. There were no statistical differences between any of these factors.
(Conclusions) Our results suggest that radical prostatectomy is available for both organconfined and non organ-confined advanced prostate cancer. Postoperative radiotherapy combined with hormonal therapy is especially useful for patients in pT3 with PDA.

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(Purpose) Up to 40% of surgically treated prostate cancers are bound to be understaged by using current diagnostic modalities. To improve on current staging methods for prostate cancer, we evaluated whether nested reverse transcriptase-polymerase chain reaction (RT-PCR) for prostatespecific antigen (PSA) mRNA can improve preoperative staging of prostate cancer.
(Materials and methods) From May 1996 through November 1997, 30 patients with prostate cancer (T1-T3) were evaluated for PSAmRNA in their peripheral blood by nested RT-PCR before radical retropubic prostatectomy.
(Resuls) A highly sensitive RT-PCR assay employed detected one PSA-expressing cell (LNCaP) diluted into ten million mononuclear cells. All 15 controls, including 9 women, showed negative PSAmRNA in their peripheral blood, whereas 16 of 30 (53%) patients with prostate cancer showed positive PSAmRNA in their preoperative peripheral blood. Interestingly, 7 patients with positive PSAmRNA had pathologically organ-confined prostate cancer. No significant relationship was demonstrated between positive PSA-PCR results and clinico-pathological parameters such as clinical stage, pretherapeutic serum PSA, pathological stage, seminal vesicle invasion, lymphnode metastasis, vascular invasion, or Gleason sum.
(Conclusion) Our present study showed that the results of PCR-based PSAmRNA assay had no significant association with clinico-pathological parameters. However, the prognostic significance of detecting the circulating prostate-specific signals requires a longer follow-up, which is currently under study.

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(Purpose) To make the policy of treatment with angiomyolipomas (AML) more clear, we discussed the natural history of angiomyolipomas retrospectively.
(Patients and methods) Between May 1982 and December 1997, 14 patients with AML in 18 kidneys were followed, who were 2 men in 2 kidneys and 12 women in 16 kidneys, 27 to 80 years old. No patients suffered from tuberous sclerosis. Symptoms, initial sizes and changes of the size were evaluated for these patients.
(Results) Ten patients with AML in 14 kidneys were asymptomatic and four patients were symptomatic. But one of the 4 patients had symptoms of abdominal pain and palpable mass which were due to contralateral AML that were treated with nephrectomy, so symptoms due to small AML were seen in 3 cases (2.0cm, 3.5cm, 3.8cm). Among 11 patients in 15 kidneys followed radiologically for more than 6 months, the tumors were unchanged in size in 7 kidneys, which were in all of 6 cases with unilateral solitary tumor and in 1 with bilateral multiple tumors. In other 8 kidneys the sizes of the tumors were increased, which were in the cases with multiple tumors in one kidney or in bilateral cases. Compared to the cases of unilateral solitary AML, the size of AML with multiple tumors in one kidney or in bilateral kidneys significantly increased (p<0.01). Embolization were performed for 4 kidneys, which were in 2 cases with increased tumor in size to more than 4cm in following period, in 1 with dull flank pain, and in 1 with the tumor more than 4cm at diainosis that grew to more than 5cm.
(Conclusions) Unilateral solitary AML was appeared to be hard to increase in size and to have a different natural history from bilateral or multiple tumors.

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(Purpose) Nerve growth factor (NGF) is synthesized in the target organs innervated by autonomic and sensory nerves so as to grow, maintain and/or repair the neurons. The present study evaluated the effects of ischemia on NGF synthesis and voiding function of the rat urinary bladder.
(Materials and Methods) Bladder ischemia was induced by ligating of bilateral internal iliac arteries in the female rats. We examined the changes in the blood flow, histological structure, voiding function and NGF content of the bladder immediately, 1, 7, 14 and 28 days after the surgery. Blood flow was estimated by measuring absorbance of homogenized bladder tissue after dye injection into the abdominal aorta. Voiding function was assessed by continuous cystometry under an awake restrained condition. NGF was quantified by enzyme immunoassay (ELISA method).
(Results) Blood flow decreased to 18% of the control immediately after the vascular ligation, and gradually recovered to 66% of the control on day 28. Histologically, epithelial ablation and thinning of muscle layer were observed on days 1 and 7. These histological disorders gradually improved to normal appearance on day 14. On day 1, while the maximum contraction pressure signigicantly decreased, the contraction frequency and small prevoiding contraction increased. On the other hand, the voiding efficacy markedly decreased on day 7. These functional changes recovered nearly to the control levels after day 14. NGF content transiently increased 2.4 times as the control on day 1.
(Conclusion) The present results will indicate that the voiding function deteriorated by acute ischemia is temporarily compensated by detrusor hyperrefrexia, which may be attributable to an enhansed NGF synthesis, and then improves by the development of collateral blood circulation.

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We present a female case of bladder exstrophy where the patient was followed up for 15 years.
In 1982 primary closure of the bladder and urethra with bilateral iliotomy by the posterior approach was performed at the age of 4 months. However, realignment was necessary. since the wound split open postoperatively. We used a corset specially prepared for this patient to prevent wound dehiscence during the subsequent postoperative course. Four years later, VUR was surgically eradicated.
At the age of 9 years old, her bladder function was satisfactory, demonstrating a normal CM pattern with synergia and Pmax 128cm H₂O on UPP. She had no urge or stress incontinence. The most recent 1VP and DMSA renal scan revealed almost normal findings. VCUG showed no VUR and renal function tests (PSP, Ccr 24) also confirmed no interval deterioration.
We believe that the approximation of the intersymphyseal band at the time of bladder neck closure is the single most important factor for ensuring urinary continence.

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(Object) The objectives of this study are to examine how many cancer patients we can detect among the outpatients whose PSA values are above 4.0ng/ml, and to compare the usefulness of transperineal six sextant biopsy (ss-biopsy) with that of transrectal one.
(Methods) All the male outpatients (above 50 years old) were inspected Tandem-R PSA levels and digital rectal examination (DRE). Among them, 129 patients showed more than 4.0ng/ml of PSA values and/or positive finding of DRE, and underwent subsequent transperineal ss-biopsy
(Results) Cancers were detected in 52 patients (40.3%) without major complications. Among 64 gray zone (PSA 4.1-10.0ng/ml) patients, 17 (26.6%) were found to be cancer by ss-biopsy, mean-while only 2 cancer patients (8.9%) were detected from 23 gray zone ones by traditional directed biopsy. Application of PSA density could not be found practicable to eliminate unnecessary biopsies in the gray zone group.
(Conclusion) Prostate cancer could be found nearly a fourth in the gray zone group of the outpatients. To enhance the detection rate, obtaining at least 6 core samples are recommended from either perineal or rectal root.

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A 64-year-old woman was admitted to our hospital on August 19, 1996 with the chief complaint of microscopichematuria. A solid mass was found in her lower abdomen. An abdominal CT scan suggested a large intrapelvic cystic mass and the existence of a fistulous connection between the mass and the small intestine. The existense of a fistula was confirmed by a preoperative barium enema and a cystscopic study. On September 25, 1996, a suprapubic partial cystectomy and total histerectomy were performed since an intrapelvic abscess was suspected. The cystic mass was observed to adhere to the hollow viscus (uterus, rectum, appendix, ileum and bladder). As a result a part of the ileum and bladder were also removed with the mass. The contents of the cyst included a foul smelling gas and white-green pus. Using a probe, we found two fistulous openings to the ileum and bladder. The histopathological findings indicated a dermoid cyst of the left ovary with thyroid follicles, which are known as “Struma ovarii”.

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A seventy-three year-old patient with prostate cancer underwent radical prostatectomy, followed by total androgen brocking therapy using flutamide and LH-RH agonist. As Hepatic dysfunction (GPT=3, 045IU/l) was noticed by periodic blood analysis, flutamide was stopped and he was hospitalized immediately without any subjective symptoms. Ten days after the admission, he developed massive bleeding from duodenal ulcer, resulting in duodenal perforation. Following the emergecy operation, plasma exchange therapy was repeated against serious hepatic dysfunction. However, he was dead of pneumonia two months after the admission. Autopsy revealed biliary congestion in a small liver, although it was not cirrhotic.
In our patient, hepatic dysfunction was irreversible and prolonged. We strongly recommend to perform serial liver function test from the start of treatment with flutamide, especially during the initial three months. Flutamide should be stopped promptly if significant liver abnormalities are detected.

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