The Japanese Journal of Urology

THREE CASES OF DENYS-DRASH SYNDROME WITH GONADOBLASTOMA

Denys-Drash syndrome (DDS) is characterized by rapidly progressing nephropathy, Disorders of Sex development, and Wilms tumor. Gonadal removal is recommended owing to the increased risk of developing gonadal tumors; however, the optimal timing remains uncertain. Herein, we report three cases with gonadoblastoma discovered in excised gonads. Case 1 involves a 13-year-old girl for whom peritoneal dialysis (PD) was initiated at 6 months of age. The left gonad removal at 9 months revealed hypoplastic testicular tissue. At 2 years and 8 months, kidney transplantation and bilateral nephrectomy were performed. A living kidney transplant was received at the age of 10 years. Subsequently, virilization signs and right gonadal swelling led to a diagnosis of gonadoblastoma during right gonadectomy at 13 years and 7 months. Case 2 involves a 6-year-old girl for whom PD was initiated 1 month after birth. Left nephrectomy was performed at 1 years and 11 months, followed by right nephrectomy at 5 years and 4 months. At the age of 6 years and 1 months, living kidney transplantation and simultaneous right gonadectomy revealed gonadoblastoma with a dysgerminoma component, without metastasis. Left gonadectomy 3 months post-transplantation revealed testis pathology. Case 3: involves a 4-year-old girl for whom PD was initiated 9 days after birth. Bilateral nephrectomy was performed at 1 year of age. Bilateral gonadectomy at 4 years and 5 months revealed bilateral ovotestis with a left gonadoblastoma component. While gonadoblastoma has limited malignant findings, it serves as the precursor of highly malignant gonadal tumors, highlighting the importance of timely excision. In DDS cases, determining the optimal timing for gonadectomy remains complex owing to variations in nephropathy progression. The priority given to PD initiation and Wilms' tumor prevention further complicates excision timing. Notably, the case of dysgerminoma in our 6-year-old patient appears to be one of the youngest cases in the literature.

THE USE OF CT-GUIDED MARKING FOR THE LAPAROSCOPIC RESECTION OF A SOLITARY RETROPERITONEAL TUMOR

When resecting small tumors or tumors with an irregular margin, a marking technique is conducted prior to the surgery. CT-guided marking techniques are common in pulmonary surgery, but it is rarely used in abdominal or urological surgery. We performed a marking technique for a small tumor that was undetectable by ultrasound using CT guidance prior to laparoscopic resection.A 63 year-old woman, two years after total hysterectomy for uterine stromal sarcoma, underwent combined right kidney resection and retroperitoneal tumor resection for a giant recurrence. Two months after the surgery, micro recurrence was observed in the vicinity of the right intestinal psoas muscle which upon follow up, the tumor size increased to 1 cm. Surgical resection of the small recurrent tumor was planned. Since it was difficult to detect by ultrasound, preoperative CT-guided marking was performed. Retroperitoneal laparoscopic resection was performed the following day. The histopathological diagnosis was endometrial stromal sarcoma.