Multilocular cystic nephroma

Classification according to ICD-10
D30.0	Benign neoplasm of the urinary organs - kidney
ICD-10 online (WHO version 2019)

A multilocular cystic nephroma is a rare benign tumor of the kidney consisting of multiple, separated by septa cysts in the kidney.

Synonyms are: cystic nephroma, English multilocular cystic nephroma; mixed epithelial stromal tumor (MEST); renal epithelial stromal tumor (REST)

The name was coined in 1956 by the doctors L. K. Boggs and Paul Kimmelstiel .

distribution

The disease has two peaks in incidence, one in infants between 3 months and 4 years of age and then in adults between the ages of 5 and 6. In children, the male sex is affected in 75%, and in the adults somewhat more the female sex.

There is an association with the pleuro-pulmonary blastoma associated with a mutation in DICER1 - Gen .

pathology

These are multilocular cystic kidney tumors consisting of chambered cysts that are surrounded by a thick connective tissue capsule and compressed parenchyma . Limescale deposits , bleeding or necrosis rarely occur.

Clinical manifestations

Clinical criteria are:

Manifestation in childhood
Painless palpable mass in the abdomen
possibly hematuria or urinary tract infection

diagnosis

A reliable diagnosis is hardly possible with the help of imaging methods .

Differential diagnosis

In children, the cystic Wilms tumor and multicystic kidney dysplasia are to be differentiated , in adults the multilocular cystic or clear cell renal carcinoma .

therapy

Treatment is usually carried out by (partial) nephrectomy .

literature

G. Hammon, KH Deeg, et al. a .: multilocular cystic nephroma. In: Monthly Pediatrics. Vol. 151, 2003, p. 308, doi: 10.1007 / s00112-002-0438-7
Christopher Wilkinson, Victor Palit, Jennifer Thomas et al. :: Adult multilocular cystic nephroma: Report of six cases with clinical, radio-pathologic correlation and review of literature. In: Urology Annals. Vol. 5, 2013, p. 13, doi: 10.4103 / 0974-7796.106958
Ian MF Silver, Alexander H. Boag, Donald A. Soboleski: Multilocular Cystic Renal Tumor: Cystic Nephroma. In: RadioGraphics. Vol. 28, 2008, p. 1221, doi: 10.1148 / rg.284075184 .

Individual evidence

↑ ^a ^b Urology textbook
↑ ^a ^b ^c ^d ^e Radiopaedia
^ LK Boggs, P. Kimmelstiel: Benign multilocular cystic nephroma: report of two cases of so-called multilocular cyst of the kidney. In: The Journal of urology. Vol. 76, No. 5, November 1956, pp. 530-541, PMID 13377464 .
↑ DA Hill, J. Ivanovich, JR Priest, CA Gurnett, LP Dehner, D. Desruisseau, JA Jarzembowski, KA Wikenheiser-Brokamp, BK Suarez, AJ Whelan, G. Williams, D. Bracamontes, Y. Messinger, PJ Goodfellow: DICER1 mutations in familial pleuropulmonary blastoma. In: Science. Vol. 325, No. 5943, August 2009, p. 965, doi: 10.1126 / science.1174334 , PMID 19556464 , PMC 3098036 (free full text).
↑ emedicine

[urologie-1] Urology textbook

[rp-2] Radiopaedia

[3] LK Boggs, P. Kimmelstiel: Benign multilocular cystic nephroma: report of two cases of so-called multilocular cyst of the kidney. In: The Journal of urology. Vol. 76, No. 5, November 1956, pp. 530-541, PMID 13377464 .

[4] DA Hill, J. Ivanovich, JR Priest, CA Gurnett, LP Dehner, D. Desruisseau, JA Jarzembowski, KA Wikenheiser-Brokamp, BK Suarez, AJ Whelan, G. Williams, D. Bracamontes, Y. Messinger, PJ Goodfellow: DICER1 mutations in familial pleuropulmonary blastoma. In: Science. Vol. 325, No. 5943, August 2009, p. 965, doi: 10.1126 / science.1174334 , PMID 19556464 , PMC 3098036 (free full text).

[5] ↑ emedicine