CASE 10917 Published on 30.07.2013

Recurrent Wilms\' tumour in a young girl: a case report

Section

Uroradiology & genital male imaging

Case Type

Clinical Cases

Authors

Iodice V, Rossi P, Tarantini G, Raffo L, Sarti E

Diagnostic and Interventional Radiology,
University of Pisa,
Via Roma 67,
56125 Pisa, Italy
Patient

18 years, female

Categories
Area of Interest Abdomen, Liver, Oncology, Kidney, Paediatric, Pelvis, Genital / Reproductive system female, Abdominal wall ; Imaging Technique CT, Image manipulation / Reconstruction, MR
Clinical History
An 18-year-old woman came to our attention because of recurrent abdominal pain arising after the right nephrectomy performed for Wilms' tumour of the right kidney. At age of 14 the girl was diagnosed with Sertoli-Leydig cell tumour of the right ovary and treated surgically.
Imaging Findings
The first CT examination (Fig.1), performed two months after the right nephrectomy because of recurrent abdominal pain, showed:
-abdominal masses with upper hydric density and poorly enhanced (arterial phase), with heterogeneous contrast enhancement during portal phase;
-other masses in iliac fossa.
To determine their nature, a biopsy was performed with the response: recurrent nephroblastoma; so the girl started chemotherapy.
After two months she repeated a CT (Fig. 2) that showed dimensional reduction of the pre-existing masses but the appearance of a new pelvic mass. An MRI (Fig. 3) was necessary to complete the study of the pelvic lesion. Having regard to the location and extent of the masses the patient underwent surgery with excision of the pelvic mass together with the right bowel, the right adrenal gland and the left ovary.
The first CT (Fig. 4) after surgery revealed remaining masses. The girl started chemotherapy again and underwent another CT 6 months later (Fig. 5) that showed a new mass in the rectum muscle.
Discussion
Wilms' tumour (or nephroblastoma) is the most frequent abdominal tumour in children [1].
Generally it is an isolated sporadic tumour, but approximately 10% of cases are associated with some genetic syndromes (WAGR syndrome and Denys–Drash syndrome). Wilm s' tumour arises from immature kidney cells.
Usually it affects children aged 3 to 4 and becomes much less common after age 5; it is extremely rare in the adults (less than 1% of all renal tumours).
Most children present with a palpable abdominal mass. Other common signs are haematuria (in 20-30% of the patients), hypertension (in 25 % of children, presumably because of increased renin activity), abdominal pain and fever.
In adults the tumour shows different behaviour and prognosis, the typical signs are a severe backache and a progressive weakness. In the adult, Wilms' tumour has an aggressive clinical course and prognosis is significantly worse than the Wilms' tumour in children [2], also because of the high recurrence and the lower response rate to chemotherapy. After the suspicion, ultrasound, CT and MRI will be performed to make the diagnosis, but the definitive one can be made only by biopsy. There are no defined treatment protocols for adults. Many authors suggest using the pathologic staging used for paediatric patients [3] and the same childhood protocols for the treatment; a multimodal therapeutic approach should be performed [4, 5]. However, the treatment outcome in adults is disappointing.
Relapse is very common depending on the tumour histology: between 15% and 50% (anaplastic tumours) [6].
Relapse occurs early (within 2 years of diagnosis), affecting lung and abdomen (60% and 30% respectively), more rarely bones or brain. The histology of the tumour and the initial therapy are the most important factors affecting survival after relapse. Anaplastic tumours have the worst prognosis.
Differential Diagnosis List
Recurrent Wilms' tumour
Neuroblastoma
Multilocular cystic nephroma
Polycystic kidney disease
Final Diagnosis
Recurrent Wilms' tumour
Case information
URL: https://www.eurorad.org/case/10917
DOI: 10.1594/EURORAD/CASE.10917
ISSN: 1563-4086

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