chondroitin-sulfates and Chondroma

1. This paper reports the structural analysis of proteoglycans and mucopolysaccharides extracted from a human multiple enchondroma (enchondromatosis), a benign cartilage tumor, where growth, but no calcification takes place. The tumors were located inside the phalanges of both hands of a 22-year-old patient and were obtained after surgery. 2. The proteoglycans of chondromas contain only a small amount of keratan sulfate (1.3% of total mucopolysaccharide) and the chondroitin sulfate is composed of 4- and 6-sulfated disaccharide units in approximately equivalent amounts, forming hybrid polymeric chains. Furthermore, the electrophoretic mobility of these proteoglycans in agarose-polyacrylamide large-pore gel indicates that they may occur as a single polydisperse component. This structural pattern is very similar to that of the proteoglycans present in the articular cartilage of normal human newborn and young. In contrast, the proteoglycans of adult articular cartilage contain higher amounts of keratan sulfate (25% of the total mucopolysaccharide) and very small amounts of 4-sulfated disaccharide units (7%) in the chondroitin sulfate molecules. The multiple zones observed in agarose/polyacrylamide large-pore gel electrophoresis indicate the presence of more than one polydisperse component. These findings suggest a correlation between the structural characteristics of the proteoglycans and the occurrence of growth in the cartilage tissue. 3. Although the amounts of proteoglycans extractable from chondromas and from normal young and adult articular cartilages were almost the same, the chondroma proteoglycans interacted with hyaluronic acid to a lesser extent than those from the normal cartilages. This effect may be due to structural changes in the hyaluronic acid-binding region of the proteoglycan monomers.

Topics: Adult; Calcification, Physiologic; Cartilage, Articular; Chemical Phenomena; Chemistry; Child; Chondroitin; Chondroitin Sulfates; Chondroma; Electrophoresis, Agar Gel; Electrophoresis, Polyacrylamide Gel; Flavobacterium; Humans; Hyaluronic Acid; Infant, Newborn; Middle Aged; Proteoglycans; Trypsin

The present investigation endeavors to characterize the mucosubstance content of 170 myxoid and chondromatous tumors and chordomas by histochemical methods. The results obtained using the critical electrolyte concentration (CEC) method as introduced by Scott and co-workers23,24 were compared with those obtained by staining with alcian blue and toluidine blue at different pH's with and without pretreatment with bovine testicular hyaluronidase. Tissues known biochemically to contain different heteroglycans were used as controls: synovial fluid and cock's comb (hyaluronic acid) stained with alcian blue up to a MgCl2 concentration of 0.1 M; fetal cartilage (chondroitin 4- and 6-sulphate) pulposus with notochordal remnants (keratan sulphate) up 10 1.0 M. The staining reaction of intramuscular myxoma and myxoid liposarcoma corresponded to that of synovial fluid and cock's comb (containing hyaluronic acid). Benign chondromatous tumors (osteochondroma, enchondroma, extraskeletal chondroma, chondromatosis in bursae, synovia, and tendon) as well as well-differentiated chondrosarcomas had a similar staining reaction to that of adult cartilage (containing keratan sulphate). However, the intensity of the reaction was lower in these tumors than in the adult cartilage, indicating that the keratan sulphate content of the tumors is lower. Most of the moderately well-differentiated chondrosarcomas, the poorly differentiated chondrosarcomas, and pulmonary metastases of chondrosarcoma, as well as mesenchymal chondrosarcoma and extra-skeletal chondrosarcoma possessed the same staining properties as fetal cartilage, known to contain chondroitin 4- and 6-sulphate but not keratan sulphate. A few of the moderately well-differentiated chondrosarcomas stained up to a MgCl2 concentration of 1.0 M. Three cases of poorly differentiated chondrosarcomas stained with alcian blue up to 0.35-0.45 M in the lowest differentiated areas, indicating the presence of sulphated heteroglycans, as chondroitin 4- and 6-sulphate. Most chordomas possessed the same staining properties as fetal cartilage; however, a few chordomas stained in the same way as notochordal remnants of nucleus pulposus (containing keratan sulphate), which are thought to be the origin of these tumors. The results of staining of the tumors in the present series with the Scott technique corresponds well with toluidine blue and alcian blue at different pH's with and without pretreatment of the sections with testicular hyaluronidas

Topics: Adult; Aged; Alcian Blue; Bone Neoplasms; Child; Chondroitin Sulfates; Chondroma; Chondrosarcoma; Chordoma; Glycosaminoglycans; Histocytochemistry; Humans; Hyaluronic Acid; Hyaluronoglucosaminidase; Hydrogen-Ion Concentration; Keratan Sulfate; Liposarcoma; Myxoma; Osmolar Concentration; Soft Tissue Neoplasms; Tolonium Chloride