Wilm's tumour is associated with all the following except
(C) (Polycystic kidney) (617- Ghai 8th)Wilm's tumor is not associated with polycystic kidney disease.Wilm's tumor is associated with three distinct syndromes.WAGR syndromeBeckwithQ Wiedemann syndromeDenys-Drash syndrome* Wilm's tumor* Aniridia* Genital abnormalities* Mental Retardation* Enlargement of body organs* Hemihypertrophy* Renal medullary cysts* Adrenal cytomegaly; Abnoraml large cells adrenal cortex* Gonadal dysgenesis (Male pseudo-hermaphrodite)* Nephropathy leading to Renal failureMore about wilm's tumor (also k/a Nephroblastoma):* Wilm's tumor is the most common renal neoplasm & 2nd most common malignant abdominal tumor, in children (Most common abdominal malignant tumor is neuroblastoma)* Peak indidence is between 2 and 5yrs of age.* 5-10% are bilateral; either both kidneys are involved simultaneously (synchronous) or one after the other (metachronous)Clinical features of W.T.- Abdominal mass (most common presenting feature)- Abdominal pain- Vomiting- Hematuria- HypertensionTreatmentHigh cure rates of 80-90% can be achieved with multimodality treatment using surgery, chemotherapy & radiotherapy.Surgical resection is done in all stages followed by chemotherapy (stage I & II) or chemotherapy* radiotherapy (stage III, IV, V).Pre op chemotherapy is given in inoperable & bilateral.Chemotherapeutic agents used are Vincristine, Dactinomycin & cyclophosphamide.* The most important determinants of prognosis is wilm's tumour is Histology and stages of disease* The treatmet of choice for stage Iwilm's tumor is Transperitoneal Radical Nephrectomy (Radical Nephroureterectomy) followed by chemotherapy with or without radiotherapy depending on tumor histology* Surgery is very important in the treatment of all stages of wilm's tumor to provide local control and prevent metastatis spread* Radical nephrouretectomy is recommended in all patientsFeaturesNeuroblastomaWilm's tumorCommon tumorMC intra-abdominal solid tumor in childrenMC renal tumor in childrenAt the time of DiagnosisAlready metastasis in greater then 50% of patientsGenerally confined to the kidneyEncassement of aortaCharacteristicUncommonLung metastasisRareCommonCalcificationCommonRare
Aniridia
Beck with syndrome
Polycystic kidney
Hemihypertrophy
Childhood Tumors