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Title: Monosomy 22 in a malignant peripheral nerve sheath tumour of the kidney in childhood: a genetic link with other malignant paediatric renal neoplasms?
Authors: Sciot, Raphael ×
Dal Cin, Paola
Fletcher, C D
De Wever, Ivo
De Vos, Rita
Van Damme, Boudewijn
Van den Berghe, Herman #
Issue Date: Oct-1995
Series Title: Histopathology vol:27 issue:4 pages:373-376
Abstract: 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 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.
ISSN: 0309-0167
Publication status: published
KU Leuven publication type: IT
Appears in Collections:Translational Cell & Tissue Research
Clinical Genetics Section (-)
Surgical Oncology
× corresponding author
# (joint) last author

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