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Monosomy 22 in a malignant peripheral nerve sheath tumour of the kidney in childhood: a genetic link with other malignant paediatric renal neoplasms?

1995; Wiley; Volume: 27; Issue: 4 Linguagem: Inglês

10.1111/j.1365-2559.1995.tb01529.x

1365-2559

Raf Sciot, Paola Dal Cin, Christopher D.�M. Fletcher, Ivo De Wever, Robin Vos, Boudewijn Van Damme, Herman Van den Berghe,

Neurofibromatosis and Schwannoma Cases

A recurrent malignant spindle cell neoplasm of the kidney, occurring in a 11‐year‐old girl and corresponding to a malignant peripheral nerve sheath tumour is reported. The neurogenic differentiation was substantiated by the presence of PGP 9.5 neurofilament and S‐100 protein positivity and by the ultrastructural features. Cytogenetic analysis revealed monosomy of chromosome 22 in the tumour while the constitutional karyotype was normal. Primary malignant peripheral nerve sheath tumour of the kidney is extremely rare and should, on morphology, be differentiated from stromal predominant Wilms' tumour and clear cell sarcoma of the kidney. Involvement of chromosome 22 has been described in a number of malignant renal tumours of childhood, such as Wilms' tumour (deletion), clear cell sarcoma (translocation), and rhabdoid tumour (deletion and translocation), thus suggesting common molecular mechanisms in pediatric malignant renal tumours.