Open Partial Nephrectomy for Wilms' Tumor in a Cross-fused Pelvic Ectopic Kidney

doi:10.1016/j.urology.2016.02.035

Urology

Volume 93, July 2016, Pages 188-190

https://doi.org/10.1016/j.urology.2016.02.035 Get rights and content

Wilms' tumor is the most common pediatric solid renal tumor. Cross-fused renal ectopia is a rare congenital anomaly in which the left and right kidneys become fused and fail to ascend from the pelvis and abdomen. We report a case of a 5-year-old girl that underwent open partial nephrectomy on a cross-fused ectopic kidney, “pancake kidney,” after incidental discovery of a solid renal mass found to be a Wilms' tumor. Thorough review of the literature shows that this combination of Wilms' tumor in the setting of cross-fused renal ectopia has only been reported twice previously.

Section snippets

Case Report

A 5-year-old girl with a medical history significant only for cross-fused renal ectopia discovered on prenatal ultrasound was undergoing a routine renal ultrasound for a history of hydronephrosis. Her specific fusion anomaly is of the pancake kidney variety, as 80% of the surfaces of the two renal units were fused together in a round configuration in the lower mid abdomen. The ultrasound revealed a 3 cm well-circumscribed, centrally located round heterogeneous mass with cystic components

Discussion

A recent report from the National Wilms Tumor Study Group has shown that the rate of Wilms' tumor in patients with a horseshoe kidney fusion abnormality is almost twice as high as in the general population.¹⁴ Cross-fused renal ectopia is a less common fusion abnormality than horseshoe kidney, and the pancake kidney presented in this case is one of the rarest of the six subtypes of cross-fused renal ectopia.³ It is unclear exactly how much higher rate of Wilms' tumor in these patients is

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There are more references available in the full text version of this article.

Cited by (6)

Crossed fused renal ectopia diagnosed in an adult: Case report
2024, International Journal of Surgery Case Reports
Crossed fused renal ectopia is one of the rare congenital anomalies in which a kidney is found on the side opposite to its normal anatomical location and is fused with the contralateral kidney. Most patients are asymptomatic and diagnosed incidentally.
In this case report we present a 60 year old woman diagnosed with a left to right crossed fused renal ectopia with fusion of the left kidney to the inferior pole of the right kidney. The patient presented with vague abdominal pain and diagnosis was made with abdominal ultrasound and CT urography.
Though usually asymptomatic, a number of anomalies and complications can be associated with crossed fused renal ectopia including obstruction, stone disease, and tumors. Evaluation and diagnosis usually requires cross sectional imaging. Patients who are asymptomatic and without complications can be managed conservatively.
Crossed fused renal ectopia is one of the rare congenital anomalies which can be associated which needs cross-sectional imaging for adequate evaluation. Associated anomalies and complications should be carefully looked for since management depends on it.
Wilms tumor in a left pelvic kidney: A case report
2020, International Journal of Surgery Case Reports
Citation Excerpt :
The majority of anomalies and syndromes associated with Wilms tumor involve the genitourinary tract, including renal ectopia, ureteral duplication, renal hypoplasia, cryptorchidism, hypospadias, and disorder of sexual development [2]. Though rare, Wilms tumor is more commonly associated with other forms of renal ectopia like horseshoe kidneys or crossed fused renal ectopia than with pelvic renal ectopia [3,4]. There have been few reports written on malignancies in a pelvic kidney [9–13].
Wilms tumor(nephroblastoma) is the most common renal tumor in childhood.The most frequent anomalies and syndromes associated with this tumor involve the genitourinary tract. The occurrence of Wilms tumor with renal ectopia is rare.When present,Wilms tumor is usually found in association with other forms of renal ectopia like horseshoe kidneys or crossed fused renal ectopia. However, reports of the occurrence of Wilms tumor in unilateral pelvic renal ectopia is uncommon. We report a case of Wilms tumor in a pelvic left kidney.
A 10 year old girl presented to the out-patient department with a one year history of lower abdominal mass, pain and hematuria. Examination revealed a mass in the suprapubic region. Radiological investigations showed a tumor in a left pelvic kidney. She was managed with preoperative chemotherapy, surgery and adjuvant chemotherapy. Histopathologic examination of the nephrectomy specimen confirmed the diagnosis of nephroblastoma.
The occurrence of Wilms tumor in association with renal ectopia is rare. There are few reports of tumors arising in unilateral pelvic ectopic kidneys. A multi-modal treatment approach involving the use of chemotherapy, surgery and radiotherapy (for high-risk tumors) in the management of Wilms tumor gives good outcome.
Tumors arising from a pelvic kidney should be considered as part of the differential diagnosis of lower abdominal masses in children.
Advanced-stage Wilms tumor arising in a horseshoe kidney of a 9-year-old child: a case report
2021, Journal of Medical Case Reports
A rare congenital fusion anomaly of the urinary tract: Pancake kidney
2019, Turkish Journal of Urology
Crossed-fused renal ectopia with renal calculi: Two case reports and a review of the literature
2019, Medicine (United States)
Pancake kidney: An incidental finding in a young man
2018, BMJ Case Reports

: Financial Disclosure: The authors declare that they have no relevant financial interests.

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Pediatric Case ReportsOpen Partial Nephrectomy for Wilms' Tumor in a Cross-fused Pelvic Ectopic Kidney

Section snippets

Case Report

Discussion

Urol Oncol

J Urol

Urology

J Pediatr Surg

J Pediatr Surg

Crossed fused renal ectopia: challenges in diagnosis and management

J Indian Assoc Pediatr Surg

Pediatric Case Reports
Open Partial Nephrectomy for Wilms' Tumor in a Cross-fused Pelvic Ectopic Kidney