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Cystic Nephroma: Benign Multilocular Cyst of the Kidney

Definition:

Cystic nephroma is a rare, benign, multilocular cystic renal tumor. It consists of multiple noncommunicating cysts separated by septa.

Epidemiology:

Overall rare, with a peak incidence in early childhood and a second peak in adulthood.

Etiology

In pediatric cystic nephroma, the cause may be a DICER1-associated tumor syndrome. In adult cystic nephroma, there is a close relationship to MEST (mixed epithelial and stromal tumor).

Diagnosis:

Renal ultrasonography, abdominal CT, or MRI. Imaging cannot reliably distinguish cystic nephroma from other malignant cystic renal tumors, such as renal cell carcinoma or Wilms tumor. In pediatric cystic nephroma, genetic testing for DICER1 should be considered.

Treatment of cystic nephroma

Because benign and malignant lesions cannot be reliably distinguished, surgical removal is indicated. When technically feasible and oncologically appropriate, partial nephrectomy is preferred (Okada, 2003).

References

Okada, T.; Yoshida, H.; Matsunaga, T.; Kouchi, K.; Ohtsuka, Y.; Saitou, T.; Horie, H. & Ohnuma, N. Nephron-sparing surgery for multilocular cyst of the kidney in a child.
J Pediatr Surg, 2003, 38, 1689–1692

  Deutsche Version: Benigne multilokuläre Nierenzyste

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