CASE STUDY

Case study

Case study

WILMS' TUMOR is the most widespread primary malignant renal tumor in childhood. It accounts for 6% to 7% of all childhood malignancies in the United States.The reported prevalence of Wilms' tumor is 8 cases for every 1 million children. Wilms' tumor is affiliated with diverse congenital anomalies, such as sporadic aniridia, hemihypertrophy, and genitourinary anomalies. The genitourinary anomalies encompass cryptorchidism, male pseudohermaphroditism, hypospadias, and renal anomalies such as hypoplasia, ectopia, replication anomalies, and horseshoe kidneys.

Horseshoe kidney is a common renal abnormality in which the right and left kidneys are fused. Ahorseshoe kidney outcomes from the abnormal fusion of the metanephric blastemas of the 2 kidneys during the sixth to seventh week of gestation. The fusion of the smaller poles of the kidneys causes a failure of the usual ascent of the kidneys as well as a failure in the usual rotation of the kidneys. The described occurrence of horseshoe kidneys varies from 1 in 312 to 1 in 1,800. The true occurrence likely lies between these 2 extremes, and most accounts cite a occurrence of 1 in 400.

Herein, we report a case of Wilms' tumor associated with a horseshoe kidney along with a reconsider of the literature.

Case report

A5-year old previously wholesome young man offered with an asymptomatic abdominal mass noted on usual personal written test by his prime care physician. The persevering had no heaviness decrease or annals of hematuria. A 10- × 15-cm abdominal mass was found extending from the right flank toward the midline. The patient's blood force was normal, and he was not anemic. An abdominal computed tomography (CT) scan displayed a mass arising from the right side of a horseshoe kidney (Fig 1). Amesenteric angiogram confirmed the occurrence of a horseshoe kidney with an aberrant body-fluid supply from the inferior mesenteric artery to the tumor.

Fig 1. Computerized tomography scan displays a large heterogeneous mass originating from the right side of a horseshoe kidney.

At laparotomy, a very large tumor was found originating from the right edge of a horseshoe kidney, and the right colon and its mesentery were fixed to the lesion so that resection of the tumor would have needed an extensive bowel resection. Biopsy outcomes of the lesion showed Wilms' tumor with favorable histology.

The persevering was classified as having stage III disease based on the presence of whole residual infection in the abdomen and the inability to work out lymph node status. He was registered on the NWTS-V, CCG-4941 and treated with regimen DD4A under the guidelines for inoperable Wilms' tumors

Postoperatively, the persevering underwent 5 weeks of vincristine, actinomycin-D, and doxorubicin chemotherapy for stage III Wilms' tumor with a significant decline in tumor size. At the second-look procedure 6 weeks after the diagnosis, a right heminephrectomy and isthmectomy was performed. The specimen was a 6-cm nephroblastoma arising from the lower district of the right kidney with extrarenal invasion. There was no nodal engagement, the resection margins were free of tumor, and the tumor was microscopically without ...