Secondary Chondrosarcoma Research Articles

Secondary chondrosarcoma in osteochondromas. Medullary extension in 15 of 45 cases.

We reviewed 45 secondary chondrosarcomas: 16 arising in solitary osteochondromas and 29 in 27 patients with multiple osteochondromas. Less than radical or wide primary surgery resulted in local recurrence in 8 of 14 lesions: 2 of 2 intralesional excisions, 5 of 11 marginal excisions, and in one case with radiotherapy alone. No local recurrence was found after a primary wide surgical procedure in 30 lesions and a radical procedure in 1 lesion after a mean of 8 (0.2-22) years' follow-up. 5 patients had died: 3 of pulmonary metastases (2 stage IB, 1 stage IIB), and 2 of local tumor invasion, both in the spine. Medullary invasion occurred in 15 of 45 lesions, this was often than reported in secondary chondrosarcoma. Medullary invasion was not always detected preoperatively, even when MR or CT examinations had been performed. Therefore, we recommend that a secondary chondrosarcoma should be removed with a wide surgical resection, including a part of the underlying bone, to keep the local recurrence risk low. There were no clinical or prognostic differences between tumors that had arisen from solitary or from multiple osteochondromas.

Metastasis of chondrosarcoma

This study was undertaken to analyse metastases of patients with intermediate- or high-grade chondrosarcomas. Out of 24 intermediate-grade tumours, 5 (21%) developed metastases, as did 6 of 10 high-grade tumours (60%) (P = 0.04). Four patients developed pulmonary metastasis only, 5 developed both pulmonary metastases and metastases of the other sites. Two patients showed a rare metastatic pattern: bone metastases only. The metastasis rate in the primary chondrosarcoma (42%) was higher than that in the secondary chondrosarcomas (0%) (P = 0.03). The metastasis rate was higher in patients with local recurrence (86%) than in those without local recurrence (19%) (P = 0.01). In 5 of 6 patients who had a local relapse and metastasis, the interval between the two relapses was a few months.

Oral presentation of secondary chondrosarcoma arising in osteochondroma of the nasal septum

A case is described in which a midline palatal swelling, on incisional biopsy, showed an ossifying, chondroid lesion with the histologic features of a lowgrade chondrosarcoma. Complete excision revealed that the tumour had originated in a benig osteochondroma of the nasal septum.

A practical classification system for multiple cartilaginous exostosis in children.

Forty-one patients with multiple cartilaginous exostosis (MCE) were classified into three groups: Group I, no involvement of the distal forearm (n = 8); Group II, involvement of the distal forearm without shortening of the radius or ulna (n = 11); Group III, involvement of the distal forearm with shortening of the radius or ulna (n = 22). Groups were compared with regard to number of lesions, regional morbidity rate, age of onset, height, and presence of valgus deformity of the ankle. From these results, children in Group I were mildly affected. Further, those in Group III were severely affected, and all children in Group III had lesions of the innominate bone or proximal femur, where secondary chondrosarcoma occurs most frequently. This classification should prove useful in estimating severity and identifying cases at high risk for malignant transformation. MCE seemed to be an aberrant enchondral ossification and its mode of development to be reflected in the growth potential of each bone. Dislocation of the radial head occurred in three cases and was the initial symptom in two of these. Dislocation of the radial head may occur earlier than previously thought, hindering its prevention.

Immunohistochemical localization of collagenous proteins in cartilaginous tumors: characteristic distribution of type IX collagen.

The distinctive tissue localization of collagen types, particularly of type IX collagen in human cartilaginous tumors (10 cases of enchondroma and 15 cases of chondrosarcoma including 3 cases of secondary chondrosarcoma) was examined immunohistochemically using affinity-purified antibodies against types I, II, III, V, VI, and IX collagen, in comparison with that in human fetal cartilage. In fetal cartilage matrix, types II and IX collagen were diffusely distributed, while types I, III, and V collagens were not present. In the matrices of enchondromas and primary chondrosarcomas, types II and IX collagens were also diffusely distributed, but with some areas of irregular type IX collagen deposits. The secondary chondrosarcoma simulated normal fetal cartilage in the distribution pattern of types II and IX collagen, unlike the pattern in primary chondrosarcoma, where types II and IX collagen were decreased and poorly immunostained, whereas non-cartilaginous interstitial collagens (I, III, and V) appeared diffusely in the matrix, increasing with the grade of malignancy. These findings suggest that neoplastic cartilage is characterized initially by an uneven distribution of type IX collagen, prior to any alteration of other types of collagen; the diverse expressions of intercellular components in cartilaginous tumors may be one indicator for malignancy.

広範切除術を行った軟骨肉腫の２例

We experienced 2 cases of chondrosarcoma treated by wide local resection. Case 1, a 49 year-old woman, had a mass with a history of tumor formation for 5 or 6 years in the right 2nd and 3rd intermetacarpal region, which had been resected 20 years previously. This case was a very rare secondary chondrosarcoma (Grade 1 in Evans' classification) originating from the 3rd metacarpal bone. She survives relapse-free without any limitation of ADL except for inability to use chopsticks at 30 months after wide local resection with a marginal margin.Case 2, a 46 year-old woman, had noticed tumor formation in her right thigh for 2 or 3 years. Roentgenographically the tumor was located from the femoral neck through to the mid-distal one third of the femoral shaft, and showed a ground glass appearance with a partial central radiolucent area and a thicker cortex. This case was diagnosed as pimary central chondrosarcoma (Grade 2 in Evans' classification). She has survived relapse-free, graded by Enneking's modified functional evaluation method as “Good” (25points) in the knee joint and “Fair” (20points) in hip joint 20months after wide local resection with a wide margin followed by total femoral replacement with a Kotz modular femur and tibia reconstruction system.Although she developed an allergic skin reaction to metal around the surgical scar at the knee joint 13 months after surgery, this completely disappeared with steroid ointment. No loosening has been seen.

Ollier's disease with secondary chondrosarcoma associated with ovarian tumour

The association of an ovarian tumour with Ollier's disease or the Maffucci Syndrome is rare. We report what we believe to be the first patient with Ollier's disease associated with an ovarian tumour who later developed a secondary chondrosarcoma.

The malignant potential of enchondromatosis.

In a tri-institutional, retrospective study with long-term follow-up, forty-four patients who had multiple enchondromas were identified. Thirty-seven patients did not have hemangiomas (Ollier disease) and seven did (Maffucci syndrome). Of the thirty-seven patients who had Ollier disease, a low-grade chondrosarcoma developed in four; an astrocytoma, in one; and a granulosa-cell ovarian tumor, in one. In four of the seven patients who had Maffucci syndrome, there were six low-grade chondrosarcomas, one high-grade osteosarcoma, one pancreatic adenocarcinoma, one biliary adenocarcinoma, and one astrocytoma. None of the patients in either group died of the skeletal sarcoma, but four of five patients who had a non-skeletal malignant lesion died. From life-table analyses of these patients, we estimated that the incidence of secondary chondrosarcoma in patients who have Ollier disease is about 25 per cent at the age of forty years, and that malignant degeneration is almost a certainty in patients who have Maffucci syndrome. We concluded that periodic surveillance of the brain and abdomen for occult malignant lesions is indicated in patients who have enchondromatosis.

肩甲骨に発生した２次性軟骨肉腫の１例

A rare case of chondrosarcoma at the left scapula was reported. About 13cm×12cm size mass was palpable in his lateral side of the scapula, however, the range of motion of the left shoulder was not restricted. Roentgenorams showed the huge mass of the scapula and the partial calcification of the surface was noted. To confirm the diagnosis, complete excision of the mass was performed, and histological findings revealed secondary chondrosarcoma arising in osteochondroma of the scapula.

遺伝性多発性軟骨性外骨腫より生じた２次性軟骨肉腫の４例

This paper describes 4 cases of secondary chondrosarcoma in hereditary multiple exostosis. The incidence of malignant change in hereditary multiple exostosis was estimated to be about 8.7%. These tumors arised from the pelvis and the proximal end of the femur. All of these tumors were giant but microscopic findings were those of well differentiated chondrosarcoma.

A New Histologic Approach to the Differentiation of Enchondroma and Chondrosarcoma of the Bones

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.

Chondrosarcoma in Children and Adolescents

Chondrosarcoma is rare in children. Only 12 patients with a diagnosis of chondrosarcoma were treated at Children's Hospital Medical Center in Boston during the period from 1957 to 1980. Pediatric chondrosarcoma has a relatively rapid onset, manifested by pain, a palpable mass and neurological symptoms with the lesion localized in the spinal column. The pelvis was the most frequent location. Ten patients had primary and two patients had secondary chondrosarcoma. Of the latter two, one developed chondrosarcoma in a preexisting osteochondroma and the other had irradiation for neuroblastoma. The poorest outcomes were in patients who had involvement of the pelvic girdle and spinal column. Grade 2 and 3 lesions had the worst prognoses with metastases occurring within 12 months. Eight patients died at the time of study, and the longest survival time was 80 months. If surgically feasible, the treatment is radical excision. Radiation therapy and chemotherapy should be reserved for recurrences or distant metastasis.

Chondrosarcoma arising in osteochondroma.

Secondary chondrosarcomas are rare. An analysis of 75 cases of chondrosarcomas secondary to osteochondroma (42 lesions were from the Mayo Clinic files and 33 were from consultation files) revealed that 40 of the patients had single exostosis and 35 had multiple lesions. More males than females were affected, and most of the patients were age 20 to 40 years. The tumors involved various bones. Malignant change was manifested radiologically by fuzzy margins of the cartilage cap and by the presence of lucent zones within the lesion. Grossly, the surface of the thickened cartilage cap is irregular. Microscopically, most of the tumors were well-differentiated (Grade 1). Treatment was surgical. Simple excision of the tumor resulted in a large recurrence rate. Resection and amputation were generally curative. Only 12 of the patients have died of their tumor. Most patients died of local recurrence. Only two of the 75 patients had definite evidence of metastatic disease.

Chondrosarcoma of the first metatarsal?Primary or secondary to enchondroma?

Enchondromas are said to be extremely rare precursors of secondary chondrosarcomas. Peripheral enchondromas of the hand and foot may be considered as benign, even when the histomorphological study reveals pleomorphic features with atypical nuclei. Our case deals with a 56 year old woman who had an enchondroma of the first metatarsal. Its distinct histopathological signs of focal malignant transformation were only seen in later review. Nine months after curettage and autologous bone-grafting a chondrosarcoma was diagnosed. The enbloc-resection of the medial foot provided adequate surgical treatment of the chondrosarcoma.

Intracytoplasmic collagenesis in chondrosarcoma

A secondary chondrosarcoma was removed from the pelvis of a 32-year-old woman. Electron microscopic observation showed that the vacuoles near the Golgi apparatus contained characteristic cross-banded fibrils. These fibrils had an axial period of about 2500 A and an asymmetrical banded structure. They were essentially identical with segment long spacing fibrils produced in vitro.

A Case of Chondrosarcoma Arising in the Exostosis of the Femur

The patient is 25 year old, female. From childfood she had hereditary multiple exostosis. 3 years ago the right thigh started to enlarge gradually and recently she has pain in the same region. From the clinical and X-ray points of view, the diagnosis was made as secondary chondrosarcoma in the upper part of the femur arising in cartilagenous exostosis. The mass was completly removed en-bloc and metal prosthesis was inserted. The tumor weighed 1250g. The histological diagnosis was osteochondroma. 8 months after the operation, there is no evidence of reccurence or metastasis.

Fibrosarcomatous Dedifferentiation of Primary and Secondary Chondrosarcoma

Five of twenty-seven chondrosarcomas seen at the Hospital for Special Surgery from 1952 to 1972 showed fibrosarcomatous dedifferentiation. One was primary and four were secondary, three in patients with enchondromas and one in a patient with Ollier's disease. The development of purely fibrosarcomatous metastases in less than one year in four of the five patients emphasizes the malignant nature of this change.

Radical local excision in the treatment of malignant tumors of the shoulder girdle (the Tikhov-Linberg operation): report of a case.

AbstractA patient with secondary chondrosarcoma of the scapula complicating diaphyseal aclasia treated by resection of the shoulder girdle by the method of Tikhov‐Linberg is reported. Good function in the dependent arm and hand were preserved. This technique of radical local excision for tumors of low grade malignancy about the shoulder girdle is occasionally a desirable alternative to an interscapulothoracic amputation.

Observations on the Bone Tumors at the Saga Prefectural Hospital

Among 844 out-patients visiting the Saga prefectural Hospital for the past six months (from Oct. 16th, 1968 to Apr. 15th, 1969), 25 cases of bone tumors were diagnosed radiologically and cyto-histologically.25 cases of bone tumors consists of 11 cases of carcinoma metastatic to bone, 2 cases of reticulosarcoma metastatic to bone, 7 cases of cartilaginous exostosis including 1 case of secondary chondrosarcoma, 2 cases of multiple myeloma, 1 case of chondroblastic osteogenic sarcoma, 1 case of monostotic fibrous dysplasia and 1 case of leukemia?, and so on.Chiefly, our observations centered around the diagnostic study of those bone tumors.It is suggested that the cytological examination through the bone marrow aspiration is very valuable for the early diagnosis of the malignant bone tumors.

Sarcomata of the Osteogenic Series (Osteosarcoma, Fibrosarcoma, Chondrosarcoma, Parosteal Osteogenic Sarcoma, and Sarcomata Arising in Abnormal Bone)

Osteogenic sarcoma is a convenient and generically correct designation for all skeletal conective-tissue neoplasms of mesenchymal origin including: osteosarcoma, chondrosarcoma, fibrosarcoma, parosteal osteogenic sarcoma, and osteogenic sarcomata arising in Paget's disease, irradiated bone, Osteochondroma, fibrous dysplasia, or other bone disease. This report is summary of the clinical, histopathological, and roentgenographic findings, and of the treatment and end-results of treatment in 552 cases of osteogenic sarcoma. Factors which have no influence on prognosis are age, sex, bone of origin, trauma, and the occurrence of fracture. Factors which do influence prognosis include: histological type of sarcoma, tumor size, preoperative and postoperative serum alkaline Phosphatase elevations, lymph-node involvement, and treatment. Absolute five-year survival figures varied from 0.0 per cent for osteogenic sarcoma arising in Paget's disease to 82 per cent for parosteal osteogenic sarcoma; the figures for the other types were as follows: osteosarcoma, 13.2 per cent; chondrosarcoma, 28.1 per cent; fibrosarcoma, 31.7 per cent; and secondary chondrosarcoma, 67 per cent. Only in chondrosarcoma did grading correlate with prognosis; the patients with low-grade and medium-grade chondrosarcoma having considerably better survival rates than those with high-grade tumors. The incidence of late failures (patients dying of sarcoma more than five years after diagnosis) showed an inverse relationship to five-year survival. Prognosis varied directly with tumnor size, and no patient with an osteosarcoma or fibrosarcoma greater than 15.0 centimeters in greatest dimension was cured. When examination of the surgical specimen revealed lymph-node metastases, the outcome was always fatal. Radical ablative surgery is the only curative therapy for osteogenic sarcoma. Irradiation as the sole therapy did not cure a single patient. Preoperative irradiation followed by radical ablative surgical therapy was attended by a worse prognosis than surgery alone. Two patients with unilateral lung metastasis were cured by lobectomy. Adjuvant chemotherapy by perfusion administered prior to radical ablative surgery might be considered for future investigative protocols.