Learning objectives
Describe the key findings of the classic Wilms tumor.
Analyze the atypical Wilms tumor´s presentation.
Describe the key findings of the atypical Wilms tumor´s presentations in ultrasound,
CT and MR.
Background
Wilms tumor,
or nephroblastoma,
is the most common renal solid mass and abdominal malignancy of childhood and occurs in approximately 1: 10,000 persons.
Its peak incidence is at 3-4 years of age,
and 80% of patients present before 5 years of age.
Is uncommon in neonates,
less than 0.16% of the cases.
Wilms tumor is most frequently sporadically,
up to 90% of cases.
On other occasions,
it occurs in hereditary forms associated with other congenital anomalies such as cryptorchidism,
hemihypertrophy,
hypospadias,
and sporadic aniridia.
The...
Findings and procedure details
Wilms tumor is usually a solitary lesion in a kidney and there is usually no metastasis at the time of diagnosis,
although throughout the disease can develop lung metastases,
liver and rarely in bone and brain.
Occasionally the tumor extends intravascularly to the renal vein and inferior vena cava (ICV).
Below we present the less frequent presentations of Wilms tumor,
showing the most representative images in each case.
Bilateral Wilms tumor.
Usually,
Wilms tumor is a single lesion in a single kidney.
They are large...
Conclusion
Wilms tumor is the most common renal solid mass and abdominal malignancy of childhood.
Although Wilms tumor is a malignant tumor,
the prognosis is excellent with a 5-year survival rate of 90%,
and 70% for metastatic disease.
The classic Wilms tumor´s presentation is usually a solitary lesion in a kidney and there is usually no metastasis at the time of diagnosis.
Although the disease can develop lung metastases,
liver and rarely in bone and brain.
However,
there are other atypical forms of Wilms tumor presentation...
References
Pediatric Renal Masses: Wilms Tumor and Beyond.Lisa H.
Lowe et al.
RadioGraphics 2000; 20:1585–1603.
Renal Tumors of Childhood: Radiologic-Pathologic Correlation Part 1.
The 1st Decade.
Ellen M.
Chung et al.
RadioGraphics 2016; 36:499–522
Wilms Tumor.
Andrew M.
Davidoff. Doi:10.1016/j.yapd.2012.04.001
Inferior vena cava involvement in children with Wilms tumor.
Abeer Al Diab et al.
Pediatr Surg Int.
DOI 10.1007/s00383-016-4034-7
Treatment of relapse Wilms tumor: lessons learned.
Spreafico F,
Pritchard Jones K,
Malogolowkin MH,
et al.
Expert Rev Anticancer Ther.
2009;9:1807–1815.
Patterns of abdominal relapse and role...