Nephroblastomatosis: clinicopathologic significance and imaging characteristics

Intraoperative ultrasound guidance for needle biopsy of a contralateral intrarenal "tumorlet" in a child with Wilms' Tumor.

“…1 It is found in 12-33 per cent of kidneys with Wilms' tumor. 2 Three types of multi focal superficial nephroblastomatosis have been described, In "nodular renal blastema," the nodules of meta- nephric blastema cells resemble \Vilms' tumor but are only microscopic. 3 -5 The second subtype, the "sclerosing metanephric hamart oma," may be a grossly visible nodule or plaque composed of a scle· rotic stroma, primitive epithelium, and no mitoses or primitive blastema.…”

Multifocal nephroblastomatosis: clinical significance and imaging

“…The pancortical (infantile) form is diffuse in nature and in its true form is probably incompatible with life [2,17]. The superficial diffuse (late infantile) form is more common and, in fact, most of the cases of the diffuse type of nephroblastomatosis reported There appears to be an interrelationship between the superficial diffuse form and the multifocal form and both have been associated with Wilms' tumor [17].…”

“…Several forms exist which have a variable association with Wilms' tumor. A diffuse form has been described and its radiographic characteristics have been noted in the literature [1,2]. A multifocal form also exists.…”

Kernspintomographische Diagnostik benigner und prämaligner abdomineller Tumoren im Kindesalter

“…Eine intraspinale Beteiligung lag bei den benignen Tumoren nicht vor. Andererseits schwanken die Häufigkeitsangaben der Nephroblastomatose bei Patienten mit Wilms-Tumor zwischen 13-40% [4,8], bei einem bilateralen synchronen Wilms-Tumor steigen sie auf 99% [3]. Die Nephroblastomatose beruht auf einer Fehlentwicklung der Nieren, die mit der Persistenz von metanephrogenem Gewebe in den Nieren verbunden ist.…”