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<http://schema.org/articleBody>
"A diagnosis of clear cell chondrosarcoma of the ulna was made in a patient with Von Hippel-Lindau disease  VHL   After surgery  genetic analysis of the tumor tissue showed loss of heterozygosity at the VHL gene locus  Immunohistochemical analysis confirmed loss of expression of the VHL protein in the tumor cells  In addition  abundant Cyclin D1 expression in the tumor was observed  Chondrosarcoma has been described before in a VHL patient and VHL protein expression has been correlated to tumor grade in a series of sporadic chondrosarcomas  In this report  we show that clear cell chondrosarcoma may be a rare but canonical VHL manifestation through a cell-autonomous mechanism involving somatic loss-of-heterozygosity of the VHL tumor suppressor gene  We discuss the relevance of this observation with regard to the pathogenesis of clear cell chondrosarcoma in the context of VHL "
<https://www.mysciencework.com/publication/facets/journal/Familial%2520Cancer>