MONOSOMY-22 IN A MALIGNANT PERIPHERAL-NERVE SHEATH TUMOR OF THE KIDNEY IN CHILDHOOD - A GENETIC LINK WITH OTHER MALIGNANT PEDIATRIC RENAL NEOPLASMS

Citation
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
Citations number
6
Categorie Soggetti
Cell Biology",Pathology
Journal title
ISSN journal
03090167
Volume
27
Issue
4
Year of publication
1995
Pages
373 - 376
Database
ISI
SICI code
0309-0167(1995)27:4<373:MIAMPS>2.0.ZU;2-G
Abstract
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.