Wilm's Tumour Clinical Patterns

Wilms' Tumour

• Occasionally, teratoid Wilms' tumour can contain cartilaginous, adipose, or muscle tissue
• Unfavorable histology is characterized by anaplasia, often seen in higher clinical stages
• Clinical patterns include:
  • Sporadic (>90%): no other association, otherwise healthy
  • Recognized associations with congenital anomalies (5%): GU anomalies
  • Familial/hereditary (1-2%): multiple, bilateral, earlier age of onset
  • Syndromic (rare): 90% can be achieved with Stage I-III tumors, even with metastatic disease

Liver Tumours

• Third most common intra-abdominal malignancy in children
• Children often appear sick, lethargic, with fatigue, bone pain, weight loss, fever, sweating, and anemia

Neuroblastoma

• Investigations include:
  • Specific laboratory studies:
    • Elevated vanillylmandelic acid (VMA) and homovanillic acid (HVA) in urine: metabolites of catecholamines
• Antenatal complications include:
  • Polyhydramnios and premature delivery
  • Fetal hydrops (placentomegaly, ascites, pleural effusions)
• Delivery should ideally be via C-section (particularly if tumor is >5 cm) to minimize risks of rupture, hemorrhage, or dystocia during birth
• Presentation includes rectal pain, constipation, and/or a mass
• Diagnosis is made by physical examination and/or investigations

Wilm's Tumor

• Incidence: 10 per million children, with approximately 100 cases each year in the UK
• Accounts for 10% of all pediatric malignancies
• Typically affects children before the age of 8, with a peak at 3-4 years
• Male to female ratio: 0.9:1 (unilateral), 0.6:1 (bilateral), with a slight predominance in girls
• Nearly all cases are renal, with occasional extra-renal cases
• 88% of cases are solitary, while 12% are multicentric
• 93% of cases are unilateral, while 7% are bilateral (85% synchronous, 15% metachronous)
• Higher incidence in Africans compared to Caucasians and East Asians

Pathology

• 90% of cases have a favorable histology, characterized by a classic triphasic pattern (tubular epithelial, blastemal, and stromal elements)

Sacrococcygeal Teratoma (SCT)

• Antenatal complications: polyhydramnios, premature delivery, fetal hydrops (placentomegaly, ascites, pleural effusions)
• Ideal delivery method: C-section (particularly if the tumor is >5 cm) to minimize the risks of rupture, hemorrhage, or dystocia during birth
• Presentation: rectal pain, constipation, and/or a mass
• Diagnosis: physical examination and/or investigations
• Complications:
  • Urological: decreased sphincter function (incontinence) in 5-30% of cases
  • Anorectal: decreased sphincter function (incontinence or constipation) in 10-30% of cases
  • Lower limb impairment (rare)
  • Cosmetic: secondary surgery usually delayed until primary school years
  • Recurrence if coccyx is not removed or there is malignancy at initial excision

This quiz covers the clinical patterns and characteristics of Wilm's tumour, including sporadic, familial, syndromic, and associated congenital anomalies.