Eleven patients with bone metastasizing renal tumor of childhood (BMRT
Ch) who were diagnosed and treated in our departments over a 20-year p
eriod were reviewed. The incidence among all malignant renal tumors in
children up to 15 years of age in our files was 3.7%. The male to fem
ale ratio was 10.1. The morphology had a uniform, predominantly clear
cell finely vascularized pattern in all of them. Various previously re
cognized variations of the classic pattern such as hyalinization, nucl
ear palisading and cyst formation were observed in some patients. Elec
tron microscopy showed that coarse vacuolization of the tumor seen at
the light microscopic level was due to distended extracellular spaces
surrounded by tumor cells and their cytoplasmic processes. Immunohisto
chemistry of the tumor cells revealed positive vimentin in all cases,
and focally positive muscle specific actin in one. A prominent feature
was fascicles of perivascular spindle cells. They were considered to
be tumor cells rather than stomal elements since their morphology was
close to the appearance of the polygonal tumor cells. The follow up da
ta were available for ten patients. Another patient died of lymph node
dissemination and gastrointestinal bleeding. Six patients survived fo
r a period of 29 months to 14 years following the diagnosis.