Wilms_Tumour_Flashcards
What is Wilms’ tumour and at what age does it typically present?
Wilms’ tumour, also known as nephroblastoma, is a common childhood malignancy typically presenting in children under 5 years old, with a median age of 3.
What are some associations of Wilms’ tumour?
Associations include Beckwith-Wiedemann syndrome, WAGR syndrome (Aniridia, Genitourinary malformations, Mental Retardation), hemihypertrophy, and mutations in the WT1 gene.
What are the common features of Wilms’ tumour?
Features include an abdominal mass, painless haematuria, flank pain, anorexia, and fever. It is unilateral in 95% of cases.
What is the metastasis rate in Wilms’ tumour and common sites?
Metastases occur in 20% of patients, with the lungs being the most common site.
What is the recommended action for a child with an unexplained enlarged abdominal mass?
For children with an unexplained enlarged abdominal mass, a Wilms’ tumour should be considered and a paediatric review arranged within 48 hours.
What are the primary treatments for Wilms’ tumour?
Treatments include nephrectomy, chemotherapy, and radiotherapy for advanced disease.
What is the prognosis for Wilms’ tumour?
The prognosis for Wilms’ tumour is good, with an 80% cure rate.
Describe the histological features of Wilms’ tumour.
Histological features include epithelial tubules, areas of necrosis, immature glomerular structures, stroma with spindle cells, and small cell blastomatous tissues resembling the metanephric blastema.
summarise
Wilms’ tumour
Wilms’ nephroblastoma is one of the most common childhood malignancies. It typically presents in children under 5 years of age, with a median age of 3 years old.
Associations
Beckwith-Wiedemann syndrome
as part of WAGR syndrome with Aniridia, Genitourinary malformations, mental Retardation
hemihypertrophy
around one-third of cases are associated with a loss-of-function mutation in the WT1 gene on chromosome 11
Features
abdominal mass (most common presenting feature)
painless haematuria
flank pain
other features: anorexia, fever
unilateral in 95% of cases
metastases are found in 20% of patients (most commonly lung)
Referral
children with an unexplained enlarged abdominal mass in children - possible Wilm’s tumour - arrange paediatric review with 48 hours
Management
nephrectomy
chemotherapy
radiotherapy if advanced disease
prognosis: good, 80% cure rate
Histological features include epithelial tubules, areas of necrosis, immature glomerular structures, stroma with spindle cells and small cell blastomatous tissues resembling the metanephric blastema
A 3-year-old girl is brought in by her mother. Her mother reports that she has been eating less and refusing food for the past few weeks. Despite this her mother has noticed that her abdomen is distended and she has developed a ‘beer belly’. For the past year she has opened her bowels around once every other day, passing a stool of ‘normal’ consistency. There are no urinary symptoms. On examination she is on the 50th centile for height and weight. Her abdomen is soft but slightly distended and a non-tender ballotable mass can be felt on the left side. Her mother has tried lactulose but there has no significant improvement. What is the most appropriate next step in management?
Switch to polyethylene glycol 3350 + electrolytes (Movicol Paediatric Plain) and review in two weeks
Speak to a local paediatrician
Reassure normal findings and advise Health Visitor review to improve oral intake
Prescribe a Microlax enema
Continue lactulose and add ispaghula husk sachets
Speak to a local paediatrician
The history of constipation is not particularly convincing. A child passing a stool of normal consistency every other day is within the boundaries of normal. The key point to this question is recognising the abnormal examination finding - a ballotable mass associated with abdominal distension. Whilst an adult with such a ‘red flag’ symptom/sign would be fast-tracked it is more appropriate to speak to a paediatrician to determine the best referral pathway, which would probably be clinic review the same week.
What is the most common presenting feature of Wilms tumour?
Recurrent urinary tract infections
Abdominal mass
Fever
Loin pain
Haematuria
Abdominal mass
The correct answer is abdominal mass. Wilms tumour, also known as nephroblastoma, is the most common paediatric renal malignancy. The most common presenting feature is an asymptomatic abdominal mass which is often discovered incidentally by a parent or caregiver while bathing or dressing the child. It typically presents as a smooth, firm and non-tender mass.
The option of recurrent urinary tract infections is incorrect. Although urinary tract infections can occur in patients with Wilms tumour due to ureteral obstruction by the tumour, it’s not a common presenting feature. Recurrent urinary tract infections are more commonly associated with anatomical abnormalities of the urinary tract or vesicoureteral reflux.
Fever is another incorrect option. Fever can be present in cases of Wilms tumour but it’s usually secondary to necrosis or infection within the tumour rather than a primary symptom. It’s also worth noting that fever without other symptoms is more likely to be caused by an infectious process rather than a neoplastic one.
Similarly, loin pain isn’t a typical presenting feature of Wilms tumour. Pain may occur if there’s rapid growth leading to stretching of the renal capsule or if there’s haemorrhage into the tumour but this isn’t common at presentation.
Finally, haematuria, although possible in cases of Wilms tumour, isn’t a frequent symptom either. Haematuria occurs when there’s invasion of the renal vessels by the tumour but this tends not to happen until later stages of disease progression. Therefore, while haematuria might be seen in some cases, it’s less likely to be the initial presenting feature when compared with an abdominal mass.
