R. Sciot et al., MONOSOMY-22 IN A MALIGNANT PERIPHERAL-NERVE SHEATH TUMOR OF THE KIDNEY IN CHILDHOOD - A GENETIC LINK WITH OTHER MALIGNANT PEDIATRIC RENAL NEOPLASMS, Histopathology, 27(4), 1995, pp. 373-376
A recurrent malignant spindle cell neoplasm of the kidney, occurring i
n a 11-year-old girl and corresponding to a malignant peripheral nerve
sheath tumour is reported. The neurogenic differentiation was substan
tiated by the presence of PGP 9.5 neurofilament and S-100 protein posi
tivity and by the ultrastructural features. Cytogenetic analysis revea
led monosomy of chromosome 22 in the tumour while the constitutional k
aryotype was normal. Primary malignant peripheral nerve sheath tumour
of the kidney is extremely rare and should, on morphology, be differen
tiated from stromal predominant Wilms' tumour and clear cell sarcoma o
f the kidney. Involvement of chromosome 22 has been described in a num
ber of malignant renal tumours of childhood, such as Wilms' tumour (de
letion), clear cell sarcoma (translocation), and rhabdoid tumour (dele
tion and translocation), thus suggesting common molecular mechanisms i
n pediatric malignant renal tumours.