[Congenital urogenital malformations associated with nephroblastomas. Long-term results of a study in Erlangen]. / Kongenitale urogenitale Fehlbildungen bei Nephroblastomen. Erlanger Langzeitergebnisse.

BACKGROUND: Wilms' tumors are highly malignant tumors of the kidneys and are among the most frequent solid tumors of childhood, which are diagnosed for the most part before the 5th year. Various congenital anomalies are associated with a genetic predisposition and thus an increased risk of developing a Wilms' tumor. PATIENTS AND METHODS: As part of a retrospective analysis, the records from 1965 to 2003 of 66 patients with histologically confirmed Wilms' tumor were reviewed. The aim of this study was to determine the incidence and type of different urogenital malformations as well as the genetic mutations and overall survival in this patient group. RESULTS: The patient cohort comprised 66 patients with histologically confirmed Wilms' tumor: 35 male patients (53%) and 31 female patients (47%). The overall survival after 10 years was 89.4%. All patients underwent radical nephrectomy: transperitoneal approach in 63 and lumbar approach in 3 patients. Eleven (16.7%) patients had diverse urogenital anomalies. A cytogenetic investigation was performed in 38 patients that revealed no pathological findings. The results of molecular genetic testing of tumor specimens were available for seven patients: a genetic mutation was detected in one case (heterozygous mutation R394 in exon 9 of the WT1 gene) which had already been described in the literature in conjunction with Denys-Drash syndrome. Hemihypertrophy with Beckwith-Wiedemann syndrome was present in two (3%) patients. CONCLUSION: In patients with urogenital malformations and Wilms' tumor, it would be important to carry out further molecular genetic testing to identify possible WT1 gene mutations. Close interdisciplinary cooperation between urologists, specialists in human genetics, and pediatricians is imperative.

[Risk factors for intra- and postoperative complications in Wilms' tumor surgery]. / Risikofaktoren intra- und postoperativer Komplikationen in der Chirurgie des Wilms-Tumors.

BACKGROUND: Wilms' tumor is the most common renal tumor in childhood. Preoperative treatment is still under discussion. The aim of this study was to determine, using our own patient collective, the risk factors for and type of intraoperative complications which can occur. In addition, the influence of the surgical procedure and tumor size on the complications and survival rate was analyzed. METHODS AND MATERIALS: A total of 66 patients with Wilms' tumor were retrospectively analyzed. Evaluation included histology, size of the primary tumor as well as neoadjuvant and adjuvant chemotherapy. The total survival rate over periods of 5 and 10 years postoperatively were analysed using Kaplan-Meier survival probabilities. RESULTS: All patients underwent radical nephrectomy: 63 using the transperitoneal and three the lumbar approach. The tumors had a mean size of 9.8 cm (range 2.5-20.0). Twenty patients (30.3%) received neoadjuvant chemotherapy for tumor reduction, while 46 patients underwent surgery without preoperative chemotherapy. Complications occurred in eight patients (15.2%). In two, a the tumor ruptured under surgery, four patients developed an ileus and two suffered cardiac arrest. One patient had postoperative hypertonia and another an incisional hernia. All complications occurred with a tumor size >5 cm or in the patient group without neoadjuvant chemotherapy. The 10 year survival rate was 89.4%. CONCLUSIONS: The risk of complications is associated with the local size of the primary tumor. Through tumor reduction, neoadjuvant chemotherapy influences the expression of the such complications. Transperitoneal tumor nephrectomy is the method of choice in surgery for Wilms' tumors.

[Wilms' tumors in adults]. / Wilms-Tumoren im Erwachsenenalter.

Wilms' tumors develop from persistent, primitive metanephrogenic stem cells. Their biology and etiology in adults is still unknown even though remnants of primitive metanephrogenic tissue, which tends to malignancy, is suspected, and there are very few scientific studies on the biology of Wilms' tumors in adults. Such a tumor occurs at a rate of 0.2/million adults in Europe and the USA. In this article, we describe the course of the disease in two adults with histologically confirmed Wilms' tumors. Both patients underwent a radical nephrectomy followed by chemotherapy as indicated by the SIOP nephroblastoma study.

[Endometriosis of the ureter and urinary bladder]. / Endometriose von Ureter und Harnblase.

Endometriosis is a benign growth of ectopic endometrial mucous membrane which has maintained the histological characteristics and biological reactions of uterine mucous membrane. In only 1-2% of cases does it occur in the urinary system, most commonly in the urinary bladder. Such an endometriosis is often diagnosed very late due both to its commonly asymptomatic course and its rarity. Individual therapy is dependent on the age of the patient, the wish for children and the extent of the growth. For endometriosis covering a large area, surgery is recommended. Methods of choice are laparoscopic bladder resection for the urinary bladder, ureterocystoneostomy using the Psoas hitch for the distal ureter, end to end anastomosis or endoscopic incision for short, proximal cases, and for extended areas, ileum cross-bridge attachment or kidney mobilization using nephropexy.