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Periosteal chondrosarcoma: a histopathological and molecular analysis of a rare chondrosarcoma subtype
Author(s) -
Cleven Arjen H G,
Zwartkruis Evita,
Hogendoorn Pancras C W,
Kroon Herman M,
Briairede Bruijn Inge,
Bovée Judith V M G
Publication year - 2015
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/his.12666
Subject(s) - chondrosarcoma , wnt signaling pathway , histogenesis , pathology , biology , immunohistochemistry , ossification , cartilage , cancer research , medicine , anatomy , signal transduction , microbiology and biotechnology
Aims Periosteal chondrosarcoma is a rare, malignant cartilage‐forming neoplasm originating from the periosteal surface of bone. We collected 38 cases from the archives of the Netherlands Committee on Bone Tumours, with the aim of studying histological features and evaluating the involvement of isocitrate dehydrogenase 1 ( IDH 1), EXT , Wnt/β‐catenin, the pRB pathway ( CDK 4 and p16), and the TP 53 pathway (p53 and MDM 2). Methods and results Histology showed a moderately cellular matrix with mucoid–myxoid changes and, in 42% of cases, formation of a neocortex. Occasional intramedullary extension (26%) and subsequent host bone entrapment (40%) were seen. Histological grading revealed grade 1 (53%) and grade 2 (45%). The EXT 1 protein was normally expressed, and mutations in IDH 1 were observed in only 15% of cases. pR b signalling was deregulated by loss of p16 expression in 50% of cases, and Wnt signalling was lost in 89%. No alterations were found in CDK 4, p53, or MDM 2. Conclusions We report the first large histological and molecular study on periosteal chondrosarcoma showing that histopathological examination and molecular aberrations do not predict prognosis. Although the mutation frequency of IDH 1 was low, we confirm the supposed relationship with central chondrosarcoma. Moreover, we identify loss of canonical Wnt signalling and deregulation of pR b signalling as possible events contributing to its histogenesis.