Multilocular cystic nephroma

Case contributed by Dr Hazem M Almasarei

Presentation

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.

Photo

Specimen received fresh/formalin fixed "LEFT KIDNEY".

Procedure: nephrectomy.

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

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

Macroscopic extent of the tumor: tumor limited the kidney. 

Tumor appearance: multiloculated cyst containing serous fluid.  

Pathology

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 tumor (weight 1.3 kg). No ascites. No peritoneal metastases. Small puncture in the tumor after resection but with no spillage into the abdomen. No tumor 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 tumor.

Provisional diagnosis: cystic nephroma.

 

Thanks to Dr. Tamadur Mahasneh. Consultant Pathologist. King Hussain Cancer Center. Amman, Jordan.

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