Wilms tumor commonly presents as:
Correct Answer: Abdominal mass
Description: Ans: c (Abdominal mass) Ref: Nelson, 18th ed, p. 2141; 17th ed, p. OPGhai, 6th ed, p. 574Most of the patients with wilms tumor present as asymptamatic abdominal mass.Wilm tumour* Also called nephroblastoma* Most common cancer of kidney in children* Second most common abdominal malignancy in children (neuroblastoma is the most common abdominal malignancy)* Most important prognostic factor is its histologic type* It is a complex mixed embryonal neoplasm of kidney composed of 3 elements-- blastema, epithelia, stroma* Usually occurs between 2-5 years of age* Incidence of bilateral wilm tumour is 7%* Chromosomal anomaly associated with wilms tumor is deletion of chr 11 pi 3 (WT1 gene)Significant associated anomalies includeWAGRDennys Drash syndromeWilms tumorAniridiaGenitourinary abnormalityMental retardationWilms tumorEarly onset renal failureMesangial sclerosisMale pseudohermaphroditismBeckwith Wiedman syndromeWilm organomegaly of kidney, adrenal, liver and pancreasHemihypertrophyMacroglossiaOmphaloceleMost common and earliest manifestation is painless abdominal lump (usually discovered by the mother while routinely bathing the baby)Histologically divided into two types* Unfavourable histology* Favourable histologyUnfavourable histologyThree sub types:* Anaplastic* Clear cell -- commonly metastasise to bone* Rhabdoid -- commonly metastasise to brain
Category:
Pediatrics
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