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