Nephroblastomatosis

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Classification according to ICD-10
C64 Malignant neoplasm of the kidney, excluding the renal pelvis
ICD-10 online (WHO version 2019)

The nephroblastomatosis is a rare kidney disease , in which immature embryonic kidney tissue ( metanephric blastema ) through the birth remains out, and with a significantly higher risk of malignant degeneration is connected.

Nephroblastomatosis should not be confused with nephroblastoma , a solid, malignant kidney tumor in childhood.

The kidney of a premature infant with nephroblastomatosis, enlarged 10 times and with pronounced deformation of the lobe architecture (panlobular shape)

Distribution and pathogenesis

The fetal metanephrogenic blastema develops into regular kidney parenchyma with glomerula and tubules of the kidney during pregnancy . In about 1% of the kidneys, residual nephrogenic tissue can be found on autopsy as a remnant of this metanephrogenic blastema.

In most infants, these blastemic remnants disappear within the first 4 months of life, but they do not regress; flat lesions with deformation and enlargement develop in the kidney parenchyma.

Classification

Depending on the positional relationship to the kidney lobes, the following forms can be distinguished:

  • perilobar form
  • intralobar form
  • panloar form with early significant enlargement of the kidney

Clinical manifestations

As a precancerous condition , it is usually an incidental finding . Both kidneys are often affected. In up to 50% of the cases, the nephroblastomatosis develops into a Wilms tumor .

There is an association with Beckwith-Wiedemann syndrome , hemihypertrophy , aniridia , multilocular cystic nephroma and multicystic kidney dysplasia (aplastic cystic kidney).

diagnosis

Diagnosis is made through medical imaging , usually sonography ; the lesions are usually less echogenic.

In computed tomography , the lesions are only recognizable as a recess after the administration of contrast medium , since they hardly absorb any contrast medium.

In magnetic resonance imaging , the changes can best be defined on T1- weighted measurements after administration of the contrast medium. Long-term follow-ups are necessary because of the potential for degeneration.

Differential diagnosis

Renal lymphomas in particular are to be distinguished .

therapy

In addition to close monitoring, active treatment options such as chemotherapy or wedge resections from the kidney are discussed.

literature

  • MS Horger, A. Fendel: To the cover picture. Bilateral nephroblastomatosis with secondary Wilms tumor manifestations. In: RöFo : Advances in the field of X-rays and nuclear medicine. Volume 175, No. 4, April 2003, pp. 453-454, PMID 12765189 .
  • P. Bandlow, L. Kopka, A. Pekrun, M. Lakomek: The nephroblastomatosis: a precancerous disease of Wilms' tumor. In: Current Radiology. Volume 4, No. 4, July 1994, pp. 195-197, PMID 7918709 .
  • B. Stöver, M. Brandis, M. Langer: Integrative imaging of nephroblastoma and nephroblastomatosis .. In: Der Radiologe. Volume 33, No. 12, December 1993, pp. 668-674, PMID 8303024 (review).
  • SK Fernbach, KA Feinstein, JS Donaldson, ES Baum: Nephroblastomatosis: comparison of CT with US and urography. In: Radiology. Volume 166, No. 1 Pt 1, January 1988, pp. 153-156, doi: 10.1148 / radiology.166.1.2827229 , PMID 2827229 .

Web links

Individual evidence

  1. ↑ P. Günther, JP Schenk, J. Tröger, K.-L. Waag: Nephroblastomatosis in childhood: follow-up and therapy determination using novel MR volumetry and 3D visualized operation planning . 2004
  2. ↑ a b c W. Schuster, D. Färber (Ed.): Children's radiology. Imaging diagnostics . Volume 2, Springer 1996, ISBN 3-540-60224-0 .
  3. ↑ a b c G. J. Lonergan, MI Martínez-León, GA Agrons, H. Montemarano, ES Suarez: Nephrogenic rests, nephroblastomatosis, and associated lesions of the kidney. In: Radiographics: a review publication of the Radiological Society of North America, Inc. Volume 18, No. 4, 1998 Jul-Aug, pp. 947-968, doi: 10.1148 / radiographics.18.4.9672980 , PMID 9672980 (review) .
  4. ↑ Radiopaedia Nephroblastomatosis
  5. ↑ a b c P. Reimer, PM Parizel, F.-A. Stichnoth (Ed.): Clinical MR Imaging. A practical approach . 2nd Edition. Springer, 2006, ISBN 3-540-31530-6 , p. 542
  6. ^ JB Beckwith, NB Kiviat, JF Bonadio: Nephrogenic rests, nephroblastomatosis, and the pathogenesis of Wilms' tumor. In: Pediatric pathology / affiliated with the International Pediatric Pathology Association. Vol. 10, No. 1-2, 1990, pp. 1-36, PMID 2156243 (review).
  7. ↑ Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ)