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 _______.

    <p>mass</p> Signup and view all the answers

    What is not a common symptom of saccrococcygeal teratoma?

    <p>Headache</p> Signup and view all the answers

    Saccrococcygeal teratoma is a type of tumor.

    <p>True</p> Signup and view all the answers

    Match the following symptoms with the correct diagnosis:

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

    What is the primary location of saccrococcygeal teratoma?

    <p>Lower back</p> Signup and view all the answers

    The diagnosis of saccrococcygeal teratoma is usually made by _______________________.

    <p>physical examination and/or investigations</p> Signup and view all the answers

    What percentage of patients with sacrococcygeal teratomas experience urological complications?

    <p>5-30%</p> Signup and view all the answers

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

    <p>Decreased sphincter function</p> Signup and view all the answers

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

    <p>Primary school years</p> Signup and view all the answers

    What is a rare complication of sacrococcygeal teratomas?

    <p>Lower limb impairment</p> Signup and view all the answers

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

    <p>To prevent recurrence</p> Signup and view all the answers

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

    <p>Recurrence of the tumor</p> Signup and view all the answers

    What percentage of patients with sacrococcygeal teratomas experience anorectal complications?

    <p>10-30%</p> Signup and view all the answers

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

    <p>For cosmetic reasons</p> Signup and view all the answers

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

    <p>To prevent recurrence</p> Signup and view all the answers

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

    <p>Increased risk of malignancy</p> 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.

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