Presentation
Abdominal lump, hematuria
Patient Data
A large heterogenous predominantly cystic mass having an internal solid component is seen in the mid and upper part of the right kidney. The lesion also has numerous thick and thin internal septations. The soft tissue components show moderate to intense diffusion restriction.
Superiorly the lesion extends outside the kidney and compresses the liver. The IVC is compressed and mildly displaced medially. No thrombus is seen within.
PC system of the right kidney is moderately dilated and compressed by the mass.
The right ureter is moderately dilated and contains hypointense (all sequences) components along its whole length.
Lobulated heterogeneous soft tissue intensity areas are seen within the right vesicoureteric junction and urinary bladder which show moderate diffusion restriction.
Histopathology report-
Microscopic description: sections from the cauliflower-like growth reveal the features of a malignant tumor with the following features:
cysts lined with flat/cuboidal/hobnail cells (or are denuded)
epithelial elements consist mainly of mature and immature/abortive tubules and small papillae resembling immature glomeruli
nephroblastomatous epithelial elements
islands of undifferentiated blastema and differentiated mesenchymal elements
focally, the septal elements protrude into the cystic spaces in microscopic papillary folds
the growth has extended up to distal margin of ureter
lymphovascular invasion: seen
Histopathological diagnosis - nephroureterectomy with bladder cuff: favors, cystic partially differentiated nephroblastoma.
Case Discussion
Cystic partially differentiated nephroblastoma is a rare childhood renal tumor. It is a part of the spectrum of multicystic renal tumor. The other entities include cystic nephroma and cystic Wilm's tumor. Cystic partially differentiated nephroblastoma frequently affect male children under 2 years old. Treatment options are surgery, neoadjuvant or adjuvant chemotherapy and radiotherapy.
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