Matrix biochemistry and cell biology of dedifferentiated chondrosarcomas.
Dedifferentiated chondrosarcoma is an uncommon mesenchymal neoplasm comprised of two different components, low-grade conventional chondrosarcoma and high-grade non-cartilaginous sarcoma. In order to gain better insight into the biology of this tumor, we investigated a large series of dedifferentiated chondrosarcomas by looking at the composition of the extracellular tumor matrix within each of the distinct histological components. Our results showed that the well-differentiated portion of the tumors showed matrix components largely similar to conventional chondrosarcomas or enchondromas. In contrast, the high-grade portions showed a variety of staining patterns related to the matrix being formed. Cartilage-specific proteoglycans and collagens were consistently absent, except in areas showing a chondroblastic osteosarcoma histomorphology. Instead, the most dominant immunostaining was received for type I collagen. Type III and VI collagens were concentrated in the areas showing a fibroblastic phenotype. Our results lend further support to the notion that dedifferentiated chondrosarcoma represents transdifferentiation of a cell towards various blastic mesenchymal cell lineages, most commonly osteoblastic and fibroblastic, but occasionally chondroblastic as well. There was no difference in the clinical outcome of patients with differing high-grade tumor types, emphasizing that grade is a more important predictor of biological behavior than the direction of tumor differentiation.
Institute of Pathology, University of Erlangen-Nürnberg, Erlangen, Germany.
Kerstin
K
Dornauer
Stephan
S
Söder
Carry Y
CY
Inwards
Judith V M G
JV
Bovee
Thomas
T
Aigner
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001859:Bone Neoplasms / Q000378:metabolism* / Q000473:pathology; D054337:Cell Dedifferentiation; D002813:Chondrosarcoma / Q000378:metabolism* / Q000473:pathology; D024061:Collagen Type III / Q000378:metabolism; D024142:Collagen Type VI / Q000378:metabolism; D005109:Extracellular Matrix / Q000378:metabolism*; D016326:Extracellular Matrix Proteins / Q000378:metabolism*; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D006025:Glycosaminoglycans / Q000378:metabolism; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis
D024061:Collagen Type III; D024142:Collagen Type VI; D016326:Extracellular Matrix Proteins; D006025:Glycosaminoglycans