Excessive testosterone production in a patient with Nelson syndrome and bilateral testicular tumors.

Bilateral primary testicular tumors are rare and usually consist of either interstitial cells or hypertrophic testicular adrenal remnant tissue. Their differentiation on clinical presentation and histologic examination remains difficult but is essential because of the different therapeutic approaches. We report a rare case of excessive testosterone production by bilateral testicular tumors in a patient with Nelson syndrome (ACTH-secreting pituitary adenoma after bilateral adrenalectomy in patients with Cushing's disease). Increased ACTH stimulation in this patient supports the thesis of pluripotent cells within the testis which can undergo differentiation to cells which are not only morphologically similar to Leydig cells but also have the functional property of these cells. Our clinical findings support the diagnosis of hyperplasia of adrenal remnant or pluripotent cells rather than a true Leydig cell tumor. We emphasize the need for hormonal evaluations which should be assessed in the context of the size of these nodular tumors prior to therapeutic decisions. In cases with elevated serum ACTH and small nodular hyperplasia, we would favor a 'wait-and-see' strategy with appropriate hormonal therapy. In large tumors with clinical signs of hormonal activity, patient noncompliance with steroid replacement regimens or with local symptoms, scrotal exploration and tumor enucleation are indicated.

[Risk oriented urologic preparation of children for kidney transplantation]. / Risikoorientierte urologische Vorbereitung von Kindern auf die Nierentransplantation.

Among 1050 consecutive renal transplants (Rtx) performed in Heidelberg since 1967 we have gained experience with 163 children and adolescents under the age of 16, receiving a Rtx since 1969. 30% of these were terminally renal insufficient due to congenital obstructive uropathy, mainly infravesical obstruction and/or reflux nephropathy. A special diagnostic work-up (flow chart) for evaluating and eliminating potential pre- and posttransplant risk factors consisted of noninvasive screening methods (ultrasonography and uroflow) followed step by step by more invasive imaging procedures (MR, MCU) and finally invasive methods of evaluating upper urinary tract and voiding disorders (complete urodynamic work-up). We emphasize the preservation or rehabilitation of urinary bladder function, eradication of significant UTI and pre-Rtx surgical treatment of otherwise hard to handle nephrogenic hypertension. Unnecessary psychologically and somatically traumatizing pretransplant diagnostic and therapeutic procedures can be avoided in pediatric non-obstructive nephropathy patients.

[Neurofibromatosis of the bladder in infancy. 10 years' follow-up]. / Neurofibromatose der Harnblase im Säuglingsalter. Verlaufsbeobachtung nach 10 Jahren.

Urinary tract involvement in neurofibromatosis is rare. We found 51 cases documented in the literature, the majority of these (n = 31) were children. More than half of these (n = 18) developed upper urinary tract obstruction. A report is given of a 10-month-old boy with neurofibromatosis of the bladder. Treatment consisted in radical surgery with cystoprostatectomy and uretero-transureterocutaneostomy. Follow-up of 10 years presents a good result. The different modalities of treatment are reviewed in the literature.