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Lack of response of rat chondrosarcoma chondrocytes to interleukin-1 to produce proteoglycan- and collagen-degrading enzymes.

Chondrocytes obtained from rat articular cartilage and Swarm rat chondrosarcoma were maintained in culture. Human interleukin-1 (IL-1) was added at confluency, and three days later the spent media was evaluated for proteoglycan- and collagen-degrading enzymes. Unlike chondrocytes from articular cartilage from several species, chondrocytes from Swarm rat chondrosarcoma did not respond to IL-1 stimulation. This lack of sensitivity to IL-1 may be a useful biochemical marker to classify various types of chondrosarcomas. However, it appears that the use of chondrosarcoma chondrocyte cultures are not useful as a source for metalloproteinases.

Animals↗

Chondrosarcoma. Increasing grade of malignancy in local recurrence.

44 chondrosarcomas were collected during a 22-year period. 40 were classic, 3 mesenchymal, and 1 clear-cell chondrosarcoma. Five (12,5%) were secondary, arising in enchondromatosis, osteochondromatosis and solitary osteochondroma. All the classic chondrosarcomas, initial tumours as well as recurrences, were histologically graded I-III on the basis of cellularity, nuclear size, frequency of lacunae containing multiple nuclei and mitotic rate (Evans). 19 were initially grade I, 14 grade II, and 7 grade III. 18 patients had at least one local recurrence, and 8 of them had 2 or more recurrences. 12 of the 18 patients developed a higher grade of malignancy in the recurrences; only in 6 patients was the grade of malignancy unchanged; 2 of them were initially grade III tumours, however. Because of the high recurrence rate and the risk of increasing malignancy in recurrence, we conclude that the initial treatment should be radical (resection en block, or amputation) even in low grade chondrosarcomas.

Adult↗

A new histologic approach to the differentiation of enchondroma and chondrosarcoma of the bones. A clinicopathologic analysis of 51 cases.

Fifty-one cases of central, hyalin cartilage tumors of the long and flat bones were analyzed. Although Grade 2 and 3 chondrosarcomas could be diagnosed on the basis of cytologic features alone, low-grade chondrosarcoma could not be adequately differentiated from pure, benign enchondroma(s) by cytology alone. The tumors can be distinguished by a new histologic approach based on tissue patterns. The crucial enchondroma patterns consist of multiple nodules of hyalin cartilage separated by normal marrow in conjunction with partial to complete encompassing plates of lamellar bone that conform to the irregular shapes of the cartilage nodules. The chondrosarcomatous patterns consist of a single confluent mass of cartilage, which commonly permeates the marrow, "trapping" host lamellar bone on all sides, and which forms bands of fibrosis between the confluent peripheral cartilage lobules. Other less common patterns included cartilagenous infiltration of the Haversian systems or marrow fat and/or the development of a soft tissue mass. A central secondary chondrosarcoma is defined as one that shows the combination of both the enchondromatous and chondrosarcomatous patterns. All 18 of the pure enchondromas diagnosed by the methods proposed in this article behaved with strict benignity (i.e., without evidence of recurrence or metastasis with an average follow-up period of 7.2 years). The 33 primary and secondary chondrosarcomas diagnosed using the described patterns behaved with the predicted frequency of recurrence, metastasis, and patient demise.

Adolescent↗

The pancreatic islets in chondrosarcoma. A qualitative and quantitative study in humans.

Pancreatic islets in 12 control and 15 chondrosarcoma cases have been studied both qualitatively and quantitatively. There was definite hypertrophy and hyperplasia of islets in the chondrosarcoma patients. The mean percent of large islets was 15% for the control group and 41% for chondrosarcoma patients. The latter had a greater number of cells per islet and a greater number of alpha cells as well as beta cells than the control series; this result might of course be merely a reflection of the increased islet tissue mass in chondrosarcoma patients.

Adenoma, Islet Cell↗

Hormonal and metabolic regulation of human chondrosarcoma in vitro.

Prostaglandin A1 has a profound inhibitory effect on uridine incorporation into RNA of normal cartilage whereas N6-monobutyryladenosine 3',5'-cyclic monophosphate is either stimulatory or without an effect. Sera from intact and growth hormone-treated hypophysectomized rats stimulate RNA synthesis but serum from untreated hypophysectomized rats does not. The present study investigated the in vitro regulation of [3H]uridine incorporation into RNA of six human chondrosarcomas to determine if malignant human chondrocytes are under similar metabolic and hormonal regulation. Prostaglandin A1 (25 micrograms/ml) markedly inhibited uridine incorporation in all six tumors (56 to 80%). N6-Monobutyryladenosine 3',5'-cyclic monophosphate (1 mM) inhibited uridine incorporation in five tumors (20 to 50%). Uridine incorporation was stimulated by growth hormone-dependent serum factors in one tumor and by growth hormone-independent serum factors in two tumors. Two tumors were more responsive to serum from growth hormone-treated hypophysectomized rats than to serum from intact rats, and one tumor was unresponsive to serum stimulation. The data indicate that: (a) prostaglandin A1 is a very potent inhibitor of RNA synthesis in human chondrosarcomas; (b) N6-monobutyryladenosine 3',5'-cyclic monophosphate affects human chondrosarcomas differently than it does normal cartilage; and (c) responses of human chondrosarcomas to serum growth factors vary among individual tumors.

Bucladesine↗

Multiple cutaneous metastases of a scapular chondrosarcoma.

The onset of metastatic chondrosarcoma in a young woman was heralded by the appearance of multiple firm, tender, cutaneous nodules two years after the primary tumor in the left scapula had been resected. Chondrosarcoma rarely metastasizes to the skin, but when it does, it may have an identical histological appearance to a cartilaginous tumor of the skin, an entity that is generally considered clinically benign. Although it is very unlikely that an occult chondrosarcoma will be first manifested clinically by a solitary cutaneous metastasis, the abrupt appearance of multiple cutaneous cartilaginous lesions would seem to warrant an investigation for primary chondrosarcoma.

Adult↗

Fine needle aspiration cytology of extraskeletal chondrosarcoma.

The cytologic presentation of a case of extraskeletal chondrosarcoma diagnosed by fine needle aspiration in a 57-year-old asymptomatic female is described. A mass detected on routine chest X ray and defined by CAT scan was subjected to a preoperative percutaneous fine needle aspiration under fluoroscopic guidance; a core biopsy was also obtained. Cytologic findings included pleomorphic malignant cells, with occasional spindle-shaped forms and binucleated and multinucleated cells having various degrees of nuclear atypia. The sarcomatous nature of this neoplasm was readily recognized in the cytologic material, although histologic and ultrastructural studies, which are also illustrated, were necessary to establish its specific histologic type. The biopsy was interpreted as a probable chondrosarcoma, and an exploratory laparotomy revealed a soft tissue tumor arising in the retroperitoneum. A diagnosis of soft tissue chondrosarcoma was rendered. In retrospect, the distinctive cytologic findings in the aspirated material suggest that extraskeletal chondrosarcoma should be considered in the differential diagnosis of soft tissue tumors.

Chondrosarcoma↗

[Clear-cell chondrosarcoma of the bone].

Data on the macroscopic features and histological structure of clear-cell chondrosarcoma of the bone obtained in studies on 15 tumors are presented. The most typical element of the histological picture consists of peculiar "clear" cells containing glycogen inclusions. The tumor cells produce interstitial cartilage substance and osteoid as well as primitive slightly atypical bone structures. Electron microscopic examinations performed in 5 tumors revealed "clear" cells, the cells of the type occurring in chondrosarcomas and chondroblastomas, and multinuclear giant cells. Morphological features of the clear-cell chondrosarcomas allow them to be considered as a special type of malignant bone tumors. They differ from the known kinds of chondrosarcomas and somewhat resemble chondroblastomas and chondroblastic osteogenic sarcomas. The possibility of secondary origin of the tumor on the basis of malignization of chondroblastoma is discussed.

Adolescent↗

Characteristics of the newly derived human chondrosarcoma cell line OSHU-1: observations on osteoinduction.

A cell line designated OSHU-1 was produced by cell culture of a grade II chondrosarcoma excised as a whole from the public bone of a 59-year-old man. The OSHU-1 chondrosarcoma cell line was maintained in vitro for over 10 months and was invariably tumorigenic when injected in inbred BALB/c athymic nude mice. The karyotype was grossly abnormal and suggestive of a malignant growth. Scanning and transmission electron microscopy showed features commonly associated with malignant transformation. Transplants of the isolated tumor cells reproduced many of the microscopic, histochemical, and biosynthetic features of the original chondrosarcoma. Hydroxyproline and uronic acid rapidly accumulated between 2 and 3 weeks after transplantation. 35S uptake increased about 5,5-fold within 7 days after transplantation. After 4-6 weeks in athymic mice, the rate of growth of the chondrosarcoma reached a plateau. The interior of the tumor calcified, while on the surface the tumor induced the formation of new bone and bone marrow, apparently of host origin.

Animals↗

Mesenchymal chondrosarcoma: a report of 17 cases.

Mesenchymal chondrosarcoma is a rare, almost uniformally lethal variant of chondrosarcoma which has been regarded as resistant to chemotherapy and radiotherapy. Seventeen cases are reported; 14 are dead of tumor or still alive with disease; eight of the 14 died less than one year after treatment, predominately of distant metastatic disease. This confirms previous reports of the highly aggressive nature of mesenchymal chondrosarcoma. Although surgery is regarded as the major method of treating the primary disease, it has limitations because many tumors cannot be adequately removed owing to location or extension to unresectable sites. It was found that such tumors respond to irradiation and that a combination of chemotherapy and radiotherapy should be used in unresectable tumors or those resected with inadequate margins. Adjuvant chemotherapy is advised in all cases treated with radical surgical excision. Unfavorable prognostic factors in mesenchymal chondrosarcoma are pain as a presenting symptom and a central rather than a peripheral limb location.

Adult↗

The management of chondrosarcoma of bone.

Chondrosarcomas of bone are among the most difficult problems in diagnosis and management in the field of orthopaedic oncology. In addition to the variability of presentation of the "standard" hyaline, myxoid or fibrous types of lesion, three unusual forms of the tumor are now recognized--mesenchymal, clear cell and dedifferentiated--which show differences in their biologic behavior. Chondrosarcomas, with only rare exception, are radio-resistant and show only a limited response to adjunctive chemotherapy. The optimal therapy at present is surgical, but clearly the type of surgical procedure selected should vary with the malignity and extent of the tumor in order to avoid either undertreatment (resulting in a high rate of local recurrence and/or distant metastases) or excessive and unnecessary sacrifice of normal tissue. Patients suspected of having chondrosarcomas should be thoroughly evaluated by clinical examination, radiographic and special studies to define as fully as possible the site and extent of the local tumor, the presence or absence of a soft-tissue mass and the existence of distant metastases. A biopsy should be performed through an incision which can be subsequently excised during the definition procedure. The tissue obtained should be graded histologically and the clinical and radiologic data added to define whether the lesion is benign or a low-grade or high-grade chondrosarcoma. Using a special system recently described by Enneking and Spanier, the lesions should be evaluated on the basis of the grade (G1 or G2); the site of the lesion in relation to the anatomic compartments (T1 or T2) and the presence or absence of distant metastasis (M0 or M1). Based on a scheme presented in this article, the appropriate one of four grades of surgical procedures should be chosen. For benign tumors, Grade I procedures (intralesional curettage or excision) are generally adequate. For Stage IA tumors, occasionally a Grade II procedure, marginal excision may be sufficient, but more frequently Grade III procedures (wide intracompartmental resections) are indicated. Stage IIA and IIB lesions should be treated with Grade III or Grade IV (radical resection) surgical procedures. The treatment of inoperable or Stage IV tumors (those with metastasis) is unsatisfactory and consists of palliative resections, radiation therapy and chemotherapy.

Adult↗

Selective increase in expression of isoform PP1 gamma 1 of type-1 protein phosphatase in chondrosarcoma cells.

The expression of the two catalytic subunits of protein phosphatase (PP) type 1 PP1 gamma 1 and PP1 delta was examined in 4 cases of osteochondroma and 4 cases of enchondroma as a benign cartilaginous tumor, and 4 cases of chondrosarcoma as a malignant cartilaginous tumor using immunohistochemical analysis. The percentage of tumor cells stained positively with antiserum against PP1 catalytic subunit isoform PP1 gamma 1 were significantly higher in chondrosarcoma than in osteochondroma and enchondroma. Furthermore, chondrosarcoma showed markedly high S-phase fraction in the cell cycle of tumor cells, as compared to osteochondroma and enchondroma. These results suggest that PP1 gamma 1 is involved in the accelerated growth of malignant cells in chondrosarcoma.

Adolescent↗

Chondrosarcoma of the larynx. A diagnostic and therapeutic dilemma.

Chondrosarcoma of the larynx. A diagnostic and therapeutic dilemma. Chondromas and chondrosarcomas of the larynx are very rare. Differential diagnosis between benign chondromas and the different degrees of malignancy in chondrosarcomas is difficult. Behaviour of chondromatous tumors of the larynx differs from those of the overall head and neck region. A case of chondrosarcoma of the larynx is described. A literature review regarding chondro(sarco)mas of the larynx and the head and neck region is given presented.

Aged↗

[Overexpression of c-erbB-2 proto-oncogene product in chondrosarcomas].

The c-erbB-2 proto-oncogene plays an important role in the regulation of cellular proliferation and differentiation. Overexpression of c-erbB-2 proto-oncogene product in 40 cases of chondrosarcomas was investigated using ABC immunoperoxidase method. Overexpression of c-erbB-2 product was present in 36/40 (90%) chondrosarcomas and in 1/9 (11%) benign cartilage tumors. Normal cartilage tissue gave negative results. These findings indicate that c-erbB-2 proto-oncogene product has practical value in diagnosing chondrosarcomas. c-erbB-2 positive grading showed a significant inverse correlation with the histopathological grading. Chondrosarcomas with high histological grade and poor differentiation showed a bias toward a decline in expression of c-erbB-2 product. The c-erbB-2 proto-oncogene product may be used as a differentiation marker.

Adult↗

[Treatment of chondrosarcoma. A retrospective study at the Norwegian Radium Hospital].

The authors present a retrospective analysis of 59 chondrosarcoma patients treated at the Norwegian Radium Hospital during the period 1981 to 1993. 31 patients were admitted with untouched tumour, seven after fine needle cytology and 20 after open biopsy or partial excision. One patient had recurrent local disease. Only 20% of the tumours were of high grade malignancy. 51 patients were treated by surgery. Reconstructions were performed in 16 patients, using allografts or endoprostheses. Amputations were performed in six cases and wide excision in 12 cases. In these 18 patients local recurrence appeared in one case, and two developed lung metastases. Only one of the 18 patients operated by amputation or wide excision has since died from chondrosarcoma. Marginal excisions were performed in 26 cases. Nine of these patients developed a local recurrence, five developed metastases and three have died. Six patients had partial excisions. Postoperative radiotherapy was given to one patient only. Five of the six are alive. In one case, the quality of the margins could not be evaluated. A total of 45 of the 51 patients treated for the primary tumour by surgery are alive. The median observation time is four years. Treatment of nonmetastatic chondrosarcoma should be surgical. Chondrosarcoma patients show wider variations in age, localization of tumour and tumour growth rate than patients with other bone sarcomas. Although wide excisions provide the best local control of any grade of malignancy, the mutilation or risk involved may be so great that some patients may benefit from marginal or even partial excision.

Adult↗

Possible association of p53 overexpression and mutation with high-grade chondrosarcoma.

Overexpression and point mutation of the p53 protein/gene was investigated in a series of chondrosarcoma by an immunohistochemical approach, and direct sequencing of the genomic DNA, respectively. In 2 of the 16 cases studied, both of which were high grade chondrosarcomas (grade III), immunodetectable p53 was identified. Histologically, one was ordinary type and the other a clear cell variant. However, no positivity was observed in the other cases including nine of low grade, ordinary type, three of low grade, clear cell type, and two of extraskeletal myxoid chondrosarcoma. Direct sequencing, following polymerase chain reaction amplification of exons 5-9 of the p53 gene in 14 cases, in which fresh materials were available, successfully demonstrated base substitution mutations in only two cases with detectable p53 overexpression on immunohistochemistry. Their details were GTC (valine) to TTC (phenylalanine) at codon 157 in exon 5, and CGT (arginine) to CAT (histidine) at codon 273 in exon 8. No mutation was detected in the other 12 cases which were negative for p53 immunostaining. These findings strongly suggest that p53 mutation plays a crucial role in the biologically aggressive subtype, and possibly in the process of tumor progression in human chondrosarcoma.

Adult↗

Intradural recurrence with chondrosarcoma of the spine. A case report and review of the literature.

Known for its resistance to chemotherapy and radiation therapy, chondrosarcoma remains largely a surgically managed tumor predisposed to local recurrence and late distant metastasis. A 42-year-old man with known thoracic chondrosarcoma developed progressive low back pain, lower extremity weakness, and urinary incontinence. Magnetic resonance imaging and computed tomographic myelography demonstrated multiple filling defects throughout the lumbosacral region. Surgical excision of these lesions disclosed intradural, extramedullary drop metastases of the chondrosarcoma. The spread of neoplasms within the dural space is uncommon, accounting for less than 5% of spinal metastases, and has not previously been reported for chondrosarcoma.

Adult↗

Parosteal (juxtacortical) chondrosarcoma of the humerus associated with regional lymph node metastasis. A case report.

Recurring parosteal (juxtacortical) chondrosarcoma of the humerus occurred in a 79-year-old man. Roentgenograms of the left humerus showed minimal cortical irregularity and a large soft tissue mass without calcification or periosteal reaction. Magnetic resonance imaging (MRI) showed a multilobular tumor of high intensity (T2-weighted imaging) without marrow involvement. During the left shoulder disarticulation an enlarged axillary lymph nodes was discovered and removed. Macroscopic examination of the disarticulated humerus showed a large yellow parosteal mass, which looked like cartilage, completely surrounding the humeral shaft. The marrow cavity was almost normal except for a small erosion at the site of the olecranon fossa. The histologic diagnosis of the mass was Grade 2 chondrosarcoma of parosteal (juxtacortical or periosteal) origin associated with regional lymph node metastasis. The features that made this case unique were a long history of the tumor (for 32 years), minimal cortical destruction of the bone by the tumor, and regional lymph node metastasis. This case illustrates the differential diagnosis of the parosteal chondrosarcoma from periosteal osteosarcoma and extraskeletal myxoid chondrosarcoma.

Aged↗