Standard preβexamination protocol β must be demonstrated:
Action: Introduce yourself, explain the examination, and obtain verbal consent.
Key observations:
Systematic examination:
Systematic approach:
Assess:
π Case Presentation β (fill in during exam)
This is a _____-year-old _____ child, brought with _____ (abdominal swelling / abdominal pain / haematuria). On examination, the child appears _____ (well/unwell), with _____ (pallor / hypertension / aniridia). There is a _____ (firm / smooth / non-tender) mass in the _____ (right/left) flank, measuring approximately _____ cm. The mass _____ (does / does not) cross the midline, and _____ (moves / does not move) with respiration. Growth parameters: weight _____ percentile, height _____ percentile. Additional findings: _____ (haematuria / hypertension / syndromic features).
β’ Wilms tumor (nephroblastoma) β most common renal malignancy
β’ Neuroblastoma β adrenal, crosses midline, calcifications, catecholamines
β’ Mesoblastic nephroma β neonatal, benign
β’ Clear cell sarcoma of kidney β aggressive, bone metastases
β’ Rhabdoid tumor of kidney β aggressive, young infants
β’ Renal cell carcinoma β rare in children, older adolescents
β’ Hydronephrosis β cystic, fluctuant, may be bilateral
β’ Polycystic kidney disease β bilateral, family history
β’ Non-Hodgkin lymphoma β bilateral, rapid, B symptoms
β’ Hepatoblastoma β right upper quadrant, AFP elevated
β’ Gonadal germ cell tumor β pelvic, Ξ²-hCG/AFP
β’ Ovarian teratoma β female, pelvic/abdominal mass
β’ Abdominal ultrasound β first-line; shows intrarenal mass, IVC thrombus
β’ CT abdomen & pelvis β with contrast; defines extent, contralateral kidney
β’ Chest CT β lung metastases (most common site)
β’ MRI abdomen β if IVC thrombus suspected, or pregnancy
β’ Urinalysis β haematuria (18% cases), proteinuria
β’ CBC β anaemia, thrombocytosis, polycythaemia
β’ LFTs, renal function β baseline for chemotherapy
β’ Coagulation profile β acquired von Willebrand disease
β’ WT1 gene β 11p13; associated with WAGR, Denys-Drash
β’ WT2 (11p15) β Beckwith-Wiedemann syndrome
β’ CTNNB1, WTX β Wnt pathway variants
β’ Loss of heterozygosity (LOH) β 1p & 16q (poor prognosis)
β’ Gain of 1q β adverse prognostic factor
β’ TP53 β anaplastic histology
β’ DICER1 β associated with cystic nephroma and Wilms
β’ Urinary catecholamines (VMA/HVA) β exclude neuroblastoma
β’ AFP, Ξ²-hCG β exclude hepatoblastoma/germ cell tumour
β’ Chest X-ray β lung metastases
β’ Bone scan β if clear cell sarcoma or rhabdoid suspected
β’ Brain MRI β if rhabdoid or clear cell sarcoma
β’ Serum LDH β may be elevated in aggressive disease
β’ IVC thrombus β Doppler US, MRI/CT with contrast
β’ Hypertension β BP monitoring; renal artery Doppler
β’ Renal failure β serum creatinine, GFR
β’ Pulmonary metastases β chest CT
β’ Liver metastases β abdominal CT/MRI
β’ Bone metastases β bone scan (if high-risk histology)
β’ Bilateral disease β CT/MRI of both kidneys
πΉ Management β Across Organ Systems
Radical nephrectomy β via transabdominal approach; early ligation of renal vein to prevent tumour embolisation. Lymph node sampling essential.
Risk-adapted: Vincristine + Actinomycin D (stages IβII); + Doxorubicin (stages IIIβIV, anaplastic). Regimen DD4A or M per COG.
Flank/abdominal radiation for stage III (residual disease, tumour spill, nodal involvement). Lung radiation for pulmonary metastases (if incomplete response).
ACE inhibitors (captopril, enalapril) or calcium channel blockers. Monitor BP daily; may resolve post-nephrectomy.
High-calorie diet, nutritional support during chemotherapy. Monitor for nausea, mucositis, and weight loss.
Anticoagulation if indicated. Surgical thrombectomy or cardiopulmonary bypass if extending into right atrium.
Transfusions (PRBC, platelets) for cytopenias. G-CSF for neutropenia. Anti-emetics, pain management.
Preoperative chemotherapy; nephron-sparing surgery (partial nephrectomy) to preserve renal function.
π Prognosis
π Followβup Schedule