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PubMed1977

Intraosseous well-differentiated osteosarcoma.

A series of 27 patients with intramedullary osteogenic sarcomas that were so well-differentiated histologically as to be confused with benign lesions was studied. Of the 27 patients, 14 were females and 13 were males, and most were adults. The tibia and femur were involved commonly. Roentgenographically, the tumors showed a mixture of sclerosis and lysis with poor margination. However, they lacked the highly destructive appearance of high-grade osteosarcomas. Histologically, spindle cells with minimal cytologic atypia were arranged in interlacing bundles. Mitotic figures were scarce. The amount of osteoid was variable, commonly being abundant. With recurrence, three tumors became highly anaplastic. Excision of tumor almost invariably led to recurrences. Resection or amputation has led to good results in seven patients. Only three of the 27 patients died with metastatic tumor; one patient is alive with metastasis. Central low-grade osteosarcomas have been misdiagnosed frequently and hence treated inadequately. Careful roentgenographic and histologic correlation is required to make the diagnosis.

Adolescent↗
PubMed1979

Thorotrast-associated sarcoma of bone: A case report and review of the literature.

An osteosarcoma developed near the right lesser trochanter of a 55-year-old woman. The neoplasm fulfilled the generally accepted criteria for a Thorotrast related malignancy. Strengthening this relationship was the occurrence of the tumor in an unusual location and uncommon age group. Thorium was confirmed both in the tumor and in bone marrow histiocytes by its characteristic x-ray spectrum. Including this case only twelve Thorotrast-associated neoplasms of bone have been reported. The mean latency period after Thorotrast administration was 26 years. Regression analysis revealed that latency period and Thorotrast dose are inversely related. All tumors were reported to be sarcomas. Six osteosarcomas, one fibrosarcoma, one chondrosarcoma, and one undifferentiated sarcoma were proven histologically. All patients, for whom follow-up was given, had died of the tumor. The reasons behind the low frequency of reported Thorotrast-associated bone malignancies may be the low concentration of 232Th and radioactive daughters in bone, long latency period or the general lack of knowledge concerning the possible relationship between Thorotrast and bone tumors. Statistical association between Thorotrast injection and bone sarcoma is shown. The criteria for the diagnosis of Thorotrastosis are discussed as well as the long term deleterious effects. The distribution of Thorotrast within the body is discussed as well as the associated radiographic changes.

Adult↗
PubMed1985

Malignant fibrous histiocytoma of bone. A review of 13 cases and an ultrastructural study.

Observations on 13 patients with primary malignant fibrous histiocytoma of bone were reported. Included were nine male and four female patients, ranging in age from 6 to 81 years (mean, 44.8 years). Pain was the most common complaint. The interval from the first symptom to the initial treatment varied from 2 months to 20 years. Seven tumors arose in the knee region. Roentgenologically, most of the lesions presented with an osteolytic and destructive appearance. Histologically, highly variable morphologic features existed. The storiform-pleomorphic pattern was found in every tumor, although it was not necessarily pathognomonic for malignant fibrous histiocytoma of bone. Surgery, radical or incomplete, was the primary treatment for all but one patient. Lymph node metastasis was present in three. Five patients died of the disease from 3 to 79 months after the diagnosis (mean, 28 months), all exhibiting metastasis to the lung. Gaucher's body-like structure demonstrated in the electron microscopic study shows the histiocytic quality of malignant fibrous histiocytoma of bone.

Adult↗
PubMed1986

The use of an expandable and adjustable prosthesis in the treatment of childhood malignant bone tumors of the extremity.

Limb-sparing surgery for malignant bone tumors in the growing child usually has been avoided because of anticipated limb-length discrepancy, especially in the lower extremity. Therefore, amputation or modified amputation was often the treatment of choice. The purpose of this article is to introduce a new concept: an expandable and adjustable internal prosthesis that can be used in the growing child in the properly selected patient after removal of the malignant neoplasm. This procedure is an alternative to amputation.

Amputation, Surgical↗
PubMed1990

A common cytogenetic abnormality and DNA content alterations in dedifferentiated chondrosarcoma.

Dedifferentiated chondrosarcoma is an uncommon and aggressive variant of chondrosarcoma. The authors report the flow cytometric characteristics and cytogenetic findings in culture of two cases of dedifferentiated chondrosarcoma. The first case was DNA diploid by flow cytometry but had cytogenetic abnormalities consisting of breaks in the short arms of both chromosomes 1, resulting in deletion in one homolog and recombination in the other. In addition, cells from this tumor showed a balanced translocation between chromosomes 4 and 5, deletion of chromosome 9, and monosomy for chromosome 10. The second case was DNA aneuploid and more complex cytogenetically but had, in common with the first case, rearrangement and translocation at the same band on chromosome 1. These cytogenetic changes are compared with abnormalities previously reported for chondrosarcoma. Possible relationships between the nonrandom chromosomal abnormalities and subclassification among chondrosarcomas are discussed.

Aged↗
PubMed1992

An isolated nonosseous metastasis to the epidural space from an osteogenic sarcoma.

Metastatic disease from osteosarcoma most commonly occurs in the lung and bony sites. Both primary spinal osteosarcomas and spinal metastatic lesions are rare. A case is reported of a nonosseous epidural metastatic lesion from osteosarcoma. It was visualized best by metrizamide-enhanced computed tomographic scanning. The patient symptomatically improved with excision of the lesion although there was massive recurrence despite combined therapy.

Adolescent↗
PubMed1993

Nonepiphyseal giant cell tumor of the long bones. Clinical, radiologic, and pathologic study.

BACKGROUND: Giant cell tumors (GCT) usually involve the epiphyses of long bones and only rarely involve the metaphysis or diaphysis without epiphyseal extension. METHODS: This report presents the clinical and pathologic features of 14 patients with metaphyseal and diaphyseal GCT. Of these tumors, 10 were metaphyseal; 2, metadiaphyseal; and 2, diaphyseal. RESULTS: The sites of involvement included the proximal tibia in six patients, distal radius in three, proximal fibula in one, distal fibula in one, distal ulna in one, proximal humerus in one, and distal femur in one. Radiographically, the tumors were lucent, and the majority were sharply marginated without sclerosis. By contrast with conventional epiphyseal GCT, which generally appear in the mature skeleton, a large proportion (50%) of the GCT in this series were in patients who had open epiphyseal growth plates. Despite the unusual clinical presentation, the behavior of metaphyseal and diaphyseal GCT was similar to that of the typical epiphyseal GCT. There was a 43% recurrence rate after curettage in this series. CONCLUSIONS: It is important to distinguish this subset of GCT from other giant cell-rich lesions more common in these sites, including aneurysmal bone cyst, osteosarcoma, and nonossifying fibroma.

Adolescent↗
PubMed2000

Reconstruction after resection of tumors around the knee: role of the free vascularized fibular graft.

We present our experience with reconstruction after resection of tumors around the knee, using free vascularized fibular grafting. The study included 23 patients. The lower femur was involved in 17 cases, the upper tibia in 6. The cases included giant cell tumor of the lower femur (2 patients), giant cell tumor of the upper tibia (1 patient), malignant fibrous histiocytoma of the lower femur (1 patient), parosteal osteosarcoma (1 patient), and periosteal osteosarcoma (1 patient). The remaining patients suffered from conventional osteogenic sarcomas. The size of the defect ranged from 12 to 16 cm in length. Skin flap necrosis after tumor resection was the most common complication encountered. Other complications included peroneal nerve involvement in one case and rupture of the arterial anastomosis in another. All transferred fibulas progressed to union within 7-9 months. Union time of both upper and lower ends of the fibula and time of appearance of periosteal reaction were identical. In evaluating periosteal hypertrophy of the fibula, the hypertrophy (de Boer) index (de Boer HD, Wood MB, J Bone Joint Surg 1989;71B:374-378) proved unreliable. False positive results are obtained, when callus formation around the lower end of the femur is far more abundant than at the upper end of the fibula. For this reason, we introduced the graft index. The latter is the ratio between the diameter of the graft at its thinnest portion at latest follow-up to its diameter on the day of operation, as calculated on plain radiographs. Two of the viable fibulas developed stress fractures after plate removal. Functional and quality-of -life results were satisfactory. It is concluded that the free vascularized fibular graft remains a valuable reconstruction option after the resection of tumors around the knee, provided certain precautions are followed. First, before closure of the wound, the skin flaps should be assessed for their viability. Necrotic parts should be excised. Stable fixation is a necessary prerequisite at the time of operation. Removal of the fixation device should not be guided by union or periosteal hypertrophy, but by true widening of the medullary canal.

Adolescent↗
PubMed1989

Megaprostheses in the treatment of primary malignant and metastatic tumors in the hip region.

Twenty patients with malignant bone tumors of the hip region were treated surgically by resection and reconstruction with an endoprosthesis. Histologic types included five primary bone tumors and 15 metastatic lesions. At review four primary bone tumor patients are still alive without evidence of disease. The length of the observation period varied from 26 to 104 months. Eleven patients with metastatic bone disease died. The average postsurgical survival time was 23 months. All patients were able to walk with or without a cane. Failure of an endoprosthesis occurred in one case. According to the Enneking Evaluation System 11 patients had a good and 9 a fair result.

Adolescent↗
PubMed1993

Multifocal synchronous osteosarcoma: the Scottish Bone Tumour Registry experience.

We report 3 cases of multifocal synchronous osteosarcoma constituting 1% of osteosarcoma cases in the Scottish Bone Tumour Registry over 30 years. All were children and had a median age of 10 years. They all presented with multiple bony lesions in the absence of demonstrable pulmonary metastases. Treatment was with palliative radiotherapy in two and combination chemotherapy and radiotherapy in the most recent case. Survival was poor in all cases, with a median of 7 months. A proposal is made for genetic studies in future cases to help elucidate the origin of these tumours. The multicentric and metastatic hypotheses are discussed.

Adolescent↗