Multilocular cystic nephroma

Case contributed by Dr Hazem M Almasarei


Abdominal distension. On examination there is a huge left-sided abdominal mass, which is hard and non-tender.

Patient Data

Age: 11 months
Gender: Male

Large multiloculated cystic non-enhancing lesion arising from the upper pole of the left kidney. Associated moderate hydronephrosis with displacement of the adjacent vessels contralaterally without intraluminal filling defects. Right lower lobe consolidation.


Specimen received fresh/formalin fixed "LEFT KIDNEY".

Procedure: nephrectomy.

Specimen integrity: intact. Specimen size: 17 x 10.5 x 6 cm.

Tumour site: Most of the kidney. Tumour Size: 14 x 12 x 12 cm. Tumour Focality: Unifocal. 

Macroscopic extent of the tumour: tumour limited the kidney. 

Tumour appearance: multiloculated cyst containing serous fluid.  


Multiloculated cyst lined by cuboidal to hobnailed simple epithelium. The cyst contents are mucoid in appearance. The fibrous septa are uniform, thin and delicate.

Shows the uniform and thin fibrous septa.

Immunostain highlights the uniform and delicate fibrous septa.

Case Discussion

Surgery: large left kidney tumour (weight 1.3 kg). No ascites. No peritoneal metastases. Small puncture in the tumour after resection but with no spillage into the abdomen. No tumour in the renal vein.

Pathology: no blastemal elements seen. The mass is confined to the kidney. The ureteric and renal vein margins are free of tumour.

Provisional diagnosis: cystic nephroma.

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Case information

rID: 51901
Published: 11th Nov 2017
Last edited: 15th Sep 2018
Inclusion in quiz mode: Included

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