Wilm's Tumour Clinical Patterns

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Questions and Answers

What is a common symptom of saccrococcygeal teratoma?

Nausea

Rectal pain (correct)

Fever

Headache

Saccrococcygeal teratoma is typically diagnosed with surgery.

False

What are the investigations used to diagnose saccrococcygeal teratoma?

Physical examination and/or investigations

Patients with saccrococcygeal teratoma may present with a _______.

mass Signup and view all the answers

What is not a common symptom of saccrococcygeal teratoma?

Headache Signup and view all the answers

Saccrococcygeal teratoma is a type of tumor.

True Signup and view all the answers

Match the following symptoms with the correct diagnosis:

Rectal pain = Saccrococcygeal teratoma Headache = Migraine Fever = Infection Nausea = Food poisoning Signup and view all the answers

What is the primary location of saccrococcygeal teratoma?

Lower back Signup and view all the answers

The diagnosis of saccrococcygeal teratoma is usually made by _______________________.

physical examination and/or investigations Signup and view all the answers

What percentage of patients with sacrococcygeal teratomas experience urological complications?

5-30% Signup and view all the answers

What is a potential complication of sacrococcygeal teratomas in the anorectal region?

Decreased sphincter function Signup and view all the answers

When should secondary surgery for cosmetic reasons be performed in patients with sacrococcygeal teratomas?

Primary school years Signup and view all the answers

What is a rare complication of sacrococcygeal teratomas?

Lower limb impairment Signup and view all the answers

Why is it essential to remove the coccyx in patients with sacrococcygeal teratomas?

To prevent recurrence Signup and view all the answers

What is a potential long-term consequence of not removing the coccyx in patients with sacrococcygeal teratomas?

Recurrence of the tumor Signup and view all the answers

What percentage of patients with sacrococcygeal teratomas experience anorectal complications?

10-30% Signup and view all the answers

What is the primary reason for performing secondary surgery in patients with sacrococcygeal teratomas?

For cosmetic reasons Signup and view all the answers

Why is it crucial to remove any malignant tissue in patients with sacrococcygeal teratomas?

To prevent recurrence Signup and view all the answers

What is a potential consequence of not removing any malignant tissue in patients with sacrococcygeal teratomas?

Increased risk of malignancy Signup and view all the answers

Study Notes

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

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Description

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