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Three cases of intracranial dural chondrosarcoma are reported. The radiologic appearance of this slow-growing, extraaxial malignant tumor is different from the more familiar skull-base chondrosarcoma and may mimic an atypical meningioma. Dural chondrosarcoma tends to be less calcified or even to lack matrix calcification; it is associated with bone erosion but not with bone destruction or hyperostosis; and it usually appears avascular at arteriography.
In cartilage, link protein(s) (LP) stabilize proteoglycan aggregates via their specific association with hyaluronic acid and proteoglycan monomers. Two major link glycoproteins are produced in bovine articular cartilage, designated LP1 (49.5 kDa) and LP2 (44.0 kDa), whereas rat chondrosarcoma produces a single link protein species similar in size to bovine LP2. Although multiple link proteins differ to a significant degree in carbohydrate content, it is not known whether they arise from variable glycosylation of a single common protein core or from complete glycosylation of different protein cores. Biosynthesis of these molecules has been studied under conditions where differences generated by N-linked glycosylation would not be evident. Link proteins were immunoprecipitated 1) from cell-free translation products of total cellular and size fractionated RNA and 2) from cell lysates and medium of cultured chondrocytes using short term radioactive labeling of the protein in the presence and absence of tunicamycin. A 42-kDa link protein precursor is synthesized by cell-free translation of either rat chondrosarcoma or bovine chondrocyte mRNa. An apparently single 41.5-kDa link protein is synthesized with inhibition of N-linked glycosylation by tunicamycin, whereas LP1 and LP2 are the mature products of cultured bovine chondrocytes. The size range of translatable rat chondrosarcoma LP mRNA is 4.0-5.5 kilobase pairs and bovine LP mRNA is 3.0-4.5 kilobase pairs, both much larger than required to encode the link protein molecule. These results suggest that a single link protein precursor gives rise to multiple fully glycosylated forms and that link protein is not synthesized as a significantly larger "pro" form.
The cases of forty-six patients who had dedifferentiated chondrosarcoma were reviewed. Two groups were identified: one in which a low-grade malignant chondrosarcoma was the precursor lesion and one in which a moderate to high-grade malignant chondrosarcoma was the precursor lesion. The radiographic features of these lesions ranged from that of a cartilaginous lesion that appeared to be benign to that of a destructive osteolytic tumor in which the cartilaginous component was overshadowed by the dedifferentiated component. Only three of the forty-six patients survived for more than two years. Resection alone, even when it was wide or radical, was not successful in controlling this lethal sarcoma.
Twelve patients were studied by means of Magnetic Resonance Imaging (MRI) in order to demonstrate either loco-regional recurrence of central chondrosarcomas or degenerative evolutions of exostoses. MRI findings were compared with plain film, scintigraphy, and Computed Tomography. MRI showed loco-regional recurrences by demonstrating their site, extent, and relationship with adjacent structures. In showing the degenerative evolution of exostoses MRI--confirmed at surgery--was superior to plain film in 3 cases out of 7, and to Computed Tomography in 2 cases. The authors discuss MRI findings: chondrosarcomas have low-intensity signal in T1-weighted sequences and high-intensity signal in T2-weighted. Thanks to its high contrast resolution MRI always allowed the detection of the chondrosarcoma. Nevertheless, MRI did not allow the tumor grading--which is due either to a difficult evaluation of the morphology of neoplastic calcifications, or to non-specific-intensity signal.
Saving operations (marginal resection of the bone, segment resection, resection of the joint end, dissection of the tumor, extirpation of the bone with tumor) were fulfilled in 157 patients with chondrosarcomas. In 17 patients operations were associated with cryogenic procedures, 11 patients were given radiation therapy before operation, in 6 patients operations were followed by chemotherapy. It was established that saving operations were indicated in the I-II clinical stages of highly differentiated forms of chondrosarcomas. Radical operations (amputation, exarticulation) are indicated in the III clinical stage of the tumor as well as in anaplastic form of chondrosarcomas regardless of its stage.
The insulin-like growth factor, multiplication stimulating activity (MSA), and insulin were recently shown to stimulate proteoglycan synthesis in monolayer cultures of chondrocytes derived from the Swarm rat chondrosarcoma. Insulin produced significant stimulation at a concentration of less than 1 ng/ml, suggesting that insulin was acting through the insulin receptor rather than through a somatomedin receptor. In this paper we have shown that the insulin-like growth factors IGF-I and IGF-II also are potent stimulators of [35S]sulfate incorporation into macromolecules recovered from the medium and cell layer matrix of the chondrosarcoma chondrocytes. Proinsulin was 3% as potent as insulin in stimulating [35S]sulfate incorporation. We identified receptors for insulin and the insulin-like growth factors, MSA, IGF-I, and IGF-II. Insulin, at concentrations 1000 times the concentration required to produce the biologic response, did not compete for binding of 125I-MSA-II-1 or of 125I-IGF-II and only partially competed for 125I-IGF-I binding. Anti-insulin receptor IgG stimulated proteoglycan synthesis and competed for 125I-insulin binding. Fab fragment prepared from anti-insulin receptor IgG completely blocked the stimulation of [35S]sulfate incorporation into macromolecules by insulin while only partially inhibiting the biologic response to insulin-like growth factors, MSA, IGF-I, and IGF-II. Similarly, the anti-insulin receptor IgG only partially inhibited the binding of 125I-IGF-I and 125I-IGF-II while completely blocking the binding of 125I-insulin. We conclude that insulin stimulates proteoglycan synthesis in the chondrosarcoma chondrocytes by acting through the insulin receptor whereas the insulin-like growth factors probably act through their own receptors.
The content of acid mucopolysaccharides of the interstitial matrix of various structural variants of chondrosarcoma was studied in the operation material of 28 chondrosarcomas. The chondrosarcoma tissue contained a large amount of sulphated mucopolysacharides and a low amount of hialuronic acid. The quantity and quality of mucopolysaccharides directly depended on the degree of maturity of the tumour tissue which was reflected morphologically in the scarcity of intercellular substance in the tissue.
A review of 23 cases of chondrosarcoma of the head and neck is presented. The sites of involvement include the gnathic bones, paranasal sinuses and orbit, nasopharynx, base of skull, larynx and the cervical spine. Histologic grading of the neoplasms into I, II, and III, primarily dependent on deviation from phenotypic cartilage and mitoses, afforded excellent correlation with prognosis and survival. While increasing size of the chondrosarcoma is not always associated with higher grade tumors, rapidly growing and extensive neoplasms are usually grade II or III chondrosarcoma.
Fine needle aspirates of ten chondrosarcomas were evaluated, and the cytologic features of this tumor were described. Some malignant cells in the smears of chondrosarcoma resembled cells of an epithelial origin and could be misinterpreted as carcinoma cells. The morphologic features described for chondrosarcoma should be helpful in avoiding such a diagnostic error.
Mesenchymal chondrosarcoma was found in 11 out of 161 patients with chondrosarcoma of the bone. The assaultive nature of the disease is illustrated by the following data: the period between the manifestation of symptoms and the first visit to the doctor was 5.9 +/- 4.3 months; metastases were detected within 20.56 +/- 6.04 months and 8 patients out of 10 died within the first 5 years. Two histological structures were observed: those of the chondrosarcoma type and hemangiopericytoma-like structures formed of small atypical cells.
The ability of chondrosarcoma cell-derived preparations to stimulate an increase in the motility of bovine capillary endothelial (BCE) cells was quantitated using a phagokinetic track assay which measures the area of tracks produced by BCE cells after ingestion of gold particles. Chondrosarcoma preparations stimulated a 2-fold increase in the mean track area produced by BCE cells in an 18-hr incubation period. The motility-stimulating activity of chondrosarcoma was purified about 120-fold. The most highly purified fractions had molecular weights between 16,000 and 20,000 and stimulated a 2-fold increase in BCE cell motility at concentrations of 10 to 20 ng/ml.
A primary chondrosarcoma of the right main bronchus was removed by pneumonectomy in a 74-year-old woman. The presenting symptoms were dyspnea and cough with a lung mass evident for 18 months. She is well and free of tumor 16 months later. Only eight established cases of primary chondrosarcoma arising from the lung, and four originating from the tracheobronchial tree have been previously described. The long preoperative history and the outcome so far confirm the relatively less aggressive character of the tracheobronchial as compared to the lung subdivision of primary pulmonary chondrosarcoma.
The ability of retinoic acid (RA) to inhibit the growth of three cell lines (Te85, Hs781, and Hs791) derived from human osteosarcomas and two cell lines (Hs705 and Hs819) derived from human chondrosarcomas was studied in culture. The exposure to 10(-5) M RA resulted, within 4 days, in changes in both cell morphology and cell growth. RA-treated cells appeared flat and spread on the substratum more than untreated cells, their exponential growth rates decreased, and their saturation densities were markedly reduced. All these effects could be reversed by removal of RA from the growth medium. The various cell lines exhibited differential susceptibility to the growth-inhibitory effect of RA. The most sensitive was the Hs705 chondrosarcoma. The proliferation of these cells was inhibited 50% by 10(-9) M RA and was completely blocked by 10(-5) m RA. In contrast, the concentrations of RA required for 50% inhibition of Hs791, Te85, Hs819, and Hs781 were 10(-7), 2 X 10(-7), 2.5 X 10(-7), and 2 X 10(-6) M, respectively. Only the Te85 and the Hs781 osteosarcoma cells and cells derived from a chondrosarcoma biopsy were able to form colonies in a semisolid medium, and this growth was dramatically inhibited by RA. These results demonstrate that RA can suppress in these mesenchymal tumor cells the expression of morphological and growth properties frequently associated with transformed cells.
Two classes of oligosaccharides were identified in alkaline borohydride digests of monomer proteoglycans from the Swarm rat chondrosarcoma. The first class consists of three similar oligomers, each of which is linked to the proteoglycan core proteins by an O-glycosidic bond between N-acetylgalactosamine and hydroxyl groups on serine and threonine. The structures of the two smallest oligosaccharides are: (formula: see text). Proteoglycan monomers from the chondrosarcoma contain about 1.3 of these oligosaccharides for every chondroitin sulfate chain. Their structures and distribution on the core protein suggest that they are related to the linkage region between keratan sulfate chains and the core protein of cartilage proteoglycans. The second class of oligomers consists of oligosaccharide-peptides that contain mannose and are probably linked to the core protein by N-glycosylamine bonds to asparagine. There appear to be about 15 of these oligosaccharides/ proteoglycan molecule, and they are primarily located on the core protein nearer to the hyaluronic acid-binding site than both the oligosaccharides linked by O-glycoside bonds and the chondroitin sulfate chains. Chondrocytes isolated from the chondrosarcoma and grown in culture synthesize both classes of oligosaccharides.
The association between histological structure of chondrosarcomas and their clinical course was studied on the material of 138 clinico-morphological observations. The scheme of O'Neal and Ackerman extended and supplemented by S. I. Lipkin was used to determine the degree of morphological maturity of chondrosarcomas. By the degree of maturity the tumours were divided into 3 groups. No relationship between the rate of recurrence and the degree of maturity was observed. On the contrary, the dependence of metastasizing on the maturity of tumor was statistically significant. Difference in the survival among patients with chondrosarcomas of the I, II, and III degree of maturity was quite significant, particularly in the period of 5-9 years after the establishment of the diagnosis and surgical treatment.
The effect of hyperthermia on Swarm rat chondrosarcoma viability was investigated in Sprague-Dawley rats. The tumor blocks (0.33 g per block) were heated to 40 degrees C, 50 degrees C, 60 degrees C, 70 degrees C, and 80 degrees C, respectively. Blocks that were not heat treated served as controls. Three heating sessions (10, 20, and 30 minutes) were performed for each heating group. The tumor blocks were transplanted into 4-week-old Sprague-Dawley rats to observe tumor growth. The tumor cells of the control, 40 degrees C (3 time sessions), and 50 degrees C (10 minutes) groups showed little difference from each other microscopically before and 5 weeks after transplantation. Microscopic evidence of cell destruction was observed immediately after hyperthermia in the groups treated at 50 degrees C (20 minutes) and higher. No tumor growth was found in these groups 35 weeks after implantation. Type II collagen, the specific collagen produced by the tumor, was analyzed with the Northern blot hybridization method. The mRNA could not be identified in the groups treated at > 50 degrees C (20 minutes) at 5 weeks after implantation. This study indicates that the growth of Swarm rat chondrosarcoma was retarded at temperatures > 50 degrees C (20 minutes). The Swarm rat chondrosarcoma is a useful model for studying the effects of hyperthermia on malignant tumor growth.
Primary central nervous system mesenchymal chondrosarcomas are extremely rare tumors composed of primitive mesenchymal cells punctuated by islands of cartilage in various stages of differentiation. Although there are isolated reports of tumors arising in the spinal dura, to our knowledge extraskeletal mesenchymal chondrosarcoma localized to the cauda equina has not been reported in the literature. We present an unusual case of a mesenchymal chondrosarcoma involving the nerve roots of the cauda equina. The clinical, pathological and neuroimaging features are reviewed and discussed.
Hereditary multiple exostosis (EXT) is an autosomal dominant disorder characterized by bony exostoses at the ends of the long bones. Linkage studies have recently suggested that there are three chromosomal locations for EXT genes, 8q24.1 (EXT1), the pericentric region of 11 (EXT2), and 19p (EXT3). As part of a larger study to determine the frequencies of the three EXT types in the United States, we have ascertained a large multigenerational family with EXT and one family member with a chondrosarcoma. This family demonstrated linkage of the disease to chromosome 11 markers. The constitutional and tumor DNAs from the affected family member were compared using short-tandem-repeat markers from chromosomes 8, 11, and 19. Loss of heterozygosity (LOH) in the tumor was observed for chromosome 8 and 11 markers, but chromosome 19 markers were intact. An apparent deletion of the marker D11S903 was observed in constitutional DNA from all affected individuals and in the tumor sample. These results indicate that the EXT2 gene maps to the region containing marker D11S903, which is flanked by markers D11S1355 and D11S1361. Additional constitutional and chondrosarcoma DNA pairs from six unrelated individuals, two of whom had EXT, were similarly analyzed. One tumor from an individual with EXT demonstrated LOH for chromosome 8 markers, and a person with a sporadic chondrosarcoma was found to have tumor-specific LOH and a homozygous deletion of chromosome 11 markers. These findings suggest that EXT genes may be tumor-suppressor genes and that the initiation of tumor development may follow a multistep model.