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Pediatrics: Renal and Adrenal Oncology
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=== Congenital Mesoblastic Nephroma === ==== Epidemiology ==== *'''<span style="color:#ff0000">Most common renal tumor in infants''' ** Most common renal tumor diagnosed on pre-natal US ** Most common tumor in infants > 4 months of age * '''Mean age at diagnosis of 3.5 months (in contrast, the median age for diagnosis of a Wilms' tumor is 3.5 years)''' ==== Subtypes (2) ==== # '''Classic type''' #* Far more common #* '''Rarely recur,''' provided surgical margins are negative # '''Cellular variant''' #* Consists of atypical spindle cells with frequent mitotic figures (25-30/10 hpf) and necrosis #* Considered a variant of a '''fibrosarcoma''' #* Associated with both '''local recurrence and widespread metastasis.''' ==== Diagnosis and Evaluation ==== *'''Imaging''' **'''Typically infiltrative (whereas Wilms' tumors displace and compress renal architecture)''' ==== Management ==== * '''<span style="color:#ff0000">Radical nephrectomy''' ** '''Biopsy and partial nephrectomy are not recommended''' since the tumour occasionally extends into the hilum or perirenal soft tissue; complete excision is important to prevent local recurrence. ==== Follow-up ==== * Surveillance by interval 6 month abdominal US for the first 2 years is usually recommended for the classic variant and more aggressive follow-up with interval CT or MRI scans of the lungs and abdomen are recommended at 3-6 intervals for the first two years for the cellular variant
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