Abstract
BackgroundHorseshoe kidney (HK) is one of the most common renal fusion abnormalities, with an incidence of 1:400 in the normal population. However, Wilms tumor (WT) arising in an HK is a rare occurrence. We report the case of a 9-year-old boy who presented with an advanced WT in an HK and also highlight the management challenges in a resource-poor setting such as ours.Case presentationThe patient was a 9-year-old Nigerian boy presented to the Pediatrics Outpatient Clinic of the University of Maiduguri Teaching Hospital (UMTH) with a history of progressive abdominal swelling, weight loss, abdominal pain, and cough. Abdominal examination revealed an irregular, firm, and non-tender mass in the right lumbar region. A computed tomography (CT) scan of the abdomen showed a heterogeneously dense mass that was predominantly to the right side of the abdomen and crossed the midline to the left side, where it continued with the relatively normal renal tissue. Chest CT revealed pulmonary metastases. A diagnosis of WT in an HK was made. The patient had a 6-week course of neoadjuvant chemotherapy, and a right nephrectomy and left partial nephrectomy was performed. The final histologic diagnosis of WT was made. Radiotherapy was intended but was not available in our facility, and the parents could not afford referral to another center.ConclusionsChildren with a clinically suspected HK with WT should undergo a careful imaging evaluation such as CT before any surgical intervention. Neoadjuvant chemotherapy to reduce tumor bulk might be a good treatment method to reduce surgical morbidity and aid in complete excision and potential for preserving renal function.
Highlights
Horseshoe kidney (HK) is one of the most common renal fusion abnormalities, with an incidence of 1:400 in the normal population
Even though Wilms tumor (WT) is rare when it occurs in association with renal ectopia, it is generally more commonly seen with HKs than other forms of ectopia such as pelvic renal ectopia [5, 6]
We describe a rare case of advanced WT arising from the HK of a 9-year-old child
Summary
Children with an abdominal mass mostly have delayed presentation and should, be carefully examined by imaging studies, including the use of CT. Due to delay in presentation and sometimes diagnosis, in addition to a limited number of drugs for chemotherapy and difficulties involved in surgeries of malignancies in children, increased morbidity is the rule in most developing countries like Nigeria. Other challenges include lack of facilities like radiotherapy, funds to cover the treatment of the malignancy, and other comorbid conditions. Most of these challenges have played out in the index case, as clearly depicted by his inability to return for follow-up
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