Osteosarcoma: state of the art.
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Biomedical subjects
Publications and source records attributed to J W Beabout.
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Twenty-six patients who had a malignant giant-cell tumor of bone--a sarcoma either juxtaposed to a zone of typical benign giant-cell tumor or occurring at the site of a previously documented benign giant-cell tumor--have been seen at the Mayo Clinic. Of the twenty-six tumors, nineteen were secondary to a previous attempt at local control of a benign giant-cell tumor. All but one of these nineteen patients with a secondary tumor had received therapeutic irradiation four to thirty-nine years earlier. The nature and duration of the symptoms and the sites of predilection of the malignant giant-cell tumors were the same as for benign giant-cell tumor. Fibrosarcoma occurred three times as frequently as osteosarcoma. The best results of treatment of the secondary sarcoma were obtained with early ablation.
Although patients who have multiple myeloma usually have straightforward clinical symptoms and corroborative radiographs, in some instances, these patients will present atypically, with symptoms suggesting active disease but radiographs that are normal or nonspecific. We reviewed the records of 32 patients who had documented multiple myeloma and had undergone CT examinations, assessing the value of those examinations. Although CT is not indicated in all patients who have multiple myeloma, it is especially useful in patients who have bone pain and normal or nonspecific radiographs. CT provided confirmatory information in all cases in which lesions were seen on radiographs. CT also frequently demonstrated a greater extent of disease than could be appreciated on the radiographs.
Insufficiency stress fractures may occur in the sacrum after radiation therapy or secondary to postmenopausal or steroid-induced osteoporosis. These fractures are often either overlooked or confused both clinically and radiographically with metastatic disease. Findings on plain films and conventional tomograms are often subtle. Radionuclide bone scans show a characteristic distribution of increased uptake. Computed tomography is the definitive technique for demonstrating the fractures.
Insufficiency fractures in the supraacetabular region were identified in five women, aged 55-83 years. Factors contributing to the diminished resistance of their bones included postmenopausal osteoporosis, steroid therapy, radiation therapy, and rheumatoid arthritis. The supraacetabular fractures were seen on routine radiographs as hazy bands of sclerosis located immediately above and parallel to the acetabular roof. All five patients had additional fractures in the spine or pelvis. Supraacetabular insufficiency fractures may be an unsuspected cause of hip pain, especially in older women.
The use of computed tomography (CT) was evaluated in 25 patients with multiple myeloma, six with extramedullary plasmacytoma, and two with undiagnosed lesions. We found that CT was useful for (1) patients with multiple myeloma who had bone pain but normal roentgenograms, (2) patients with an M-protein, bone marrow plasmacytosis, and back pain from osteoporosis and compression fractures but an inconclusive diagnosis of multiple myeloma, (3) the determination of extent of tumor, and (4) guidance in needle biopsy.
In a review of cases of fibrous cartilaginous dysplasia of bone, five of fibrocartilaginous lesions were found to be different in clinical behavior and radiographic and morphologic features from the others. We have named these previously undescribed tumors "fibrocartilaginous mesenchymomas with low-grade malignancy in the fibrous elements."
Nine patients (seven males and two females) had high-grade, ordinary-type osteosarcomas that were juxtacortically located. The ages of these patients ranged from 9 to 62 years at the time of diagnosis. The roentgenographic appearance of these tumors occasionally is similar to that of periosteal osteosarcomas. Histologically, these tumors were indistinguishable from ordinary high-grade intramedullary osteosarcomas. None of the nine tumors had histologic features of parosteal or periosteal osteosarcomas. All nine patients were treated by ablative surgery. Seven patients died of tumor. Because of its poorer prognosis, this tumor should be differentiated from other juxtacortical osteosarcomas.
Clear cell chondrosarcoma of bone, a low-grade malignant tumor, frequently presents diagnostic difficulties. Its clinical, roentgenographic, and pathologic characteristics separate it from conventional chondrosarcoma and, more importantly, from several benign bone tumors with which it is often confused. This report analyzes 47 cases seen at the Mayo Clinic and in consultation. The lesion is more common in males than females (2.6:1) and has a predilection for the end of long bones, particularly the proximal femur. The age range is wide; most patients are in the third and fourth decades of life. Roentgenographically, the lesion most often is purely lytic and slightly expansile, with a sharp margin between the tumor and the adjacent normal bone. Pathologically, clear cells arranged in an indistinct lobular pattern characterize the tumor. Frequently, areas mimicking other primary bone tumors, benign and malignant, are present, often to such an extent as to obscure the true identity of the process. The overall mortality in the present series was 15%. En bloc resection, including a margin of normal bone and soft tissue, appears to be the treatment of choice.
Benign giant cell tumor of bone may be implanted into the surrounding soft tissues at the time of surgery or pathologic fracture. These soft-tissue implants may produce ossification that is radiographically visible. A review of the available radiographs of 400 cases of giant cell tumor from the Mayo Clinic and 700 cases seen in consultation yielded 17 examples of this phenomenon. In all but one case, the ossification was found at the periphery of the implant. The radiographic appearance of these implants is characteristic. Recognition of this appearance is necessary so that the tumor may be totally eradicated.
Parosteal osteosarcoma is an uncommon malignant tumor of bone, and in a review of Mayo Clinic records we identified eleven cases of so-called dedifferentiated parosteal osteosarcoma. Ten of the eleven patients had had a long history of treatment for multiple recurrences of the tumor as a low-grade parosteal osteosarcoma and then for a definite recurrence as a high-grade undifferentiated osteosarcoma. The prognosis in this group of patients was similar to that in patients with conventional osteosarcoma.
We report 35 cases of peripheral skeletal osteochondromatous tumefactions which are both histologically and radiologically distinctive. The ages of the patients ranged from 14 to 74 years, with no sex predilection. Symptoms were related to the tumefaction. None of the patients had antecedent physical trauma or radiation. All lesions occurred on proximal phalanges, metatarsals, or metacarpals. Primary treatment was usually excision, and the gross appearance was typically that of a small osteochondroma. Histologically, the lesion exhibited marked proliferative activity, irregular bony-cartilaginous interfaces, and enlarged, bizarre, and binucleate chondrocytes, mimicking chondrosarcoma. Radiologically, the proliferations lacked both central continuity of the tumor with the underlying osseous medulla and "flaring" of the adjacent cortices. In 18 cases, the lesions recurred after primary excision, and at least eight lesions recurred twice. In spite of the rate of recurrence and the disturbing histologic appearance of these proliferations, behavior as a malignant tumor is either very rare or nonexistent.
The most serious complication of Paget disease is the development of sarcoma. Forty-one cases of bone sarcoma in Paget disease, which represented our institution's entire experience, were reviewed. The relative frequency of sarcomatous degeneration was 0.9%. Histologically, 35 of 41 lesions were osteogenic sarcomas, and six were fibrosarcomas. The distribution of lesions was similar to that expected with uncomplicated Paget disease, except for a disproportionately high number of lesions in the humerus; only one lesion involved the vertebrae. The tumors were categorized radiographically as lytic, mixed, and sclerotic, in descending order of frequency. The vast majority of lesions occurred in the pelvis, humerus, and femur. Overall prognosis for these patients was poor, with a five-year survival of 8%. Finding areas of cortical destruction is particularly useful in the detection of malignant degeneration. The radiologist must maintain a high index of suspicion when evaluating radiographs of patients with Paget disease, especially of those who present with pain or a palpable mass.
Some patients with radiologic findings of neurogenic arthropathy or multiple fractures do not exhibit overt neurologic signs. Results of nerve conduction velocity, computer-assisted sensory examination, periosteal nociception, and morphometric and graded teased-fiber evaluation of cutaneous nerves allowed us to recognize a mild neuropathic abnormality. Neurogenic arthropathy and subclinical neuropathy were also found in relatives. In three kinships, the underlying disorder was probably hereditary sensory neuropathy type 1 and in several others, it was recessively inherited sensory neuropathy. These arthropathies were often painful, and overt loss of superficial and deep pain sensation was not a prominent or necessary condition. An interplay of multiple factors including insensitivity, trauma, obesity, activity, abuse, personality, mental subnormality, and metabolic joint and bone disease are probably involved in the development of the bony lesions and thus provide further evidence that environmental factors affect expression of human mutant genes for inherited neuropathy.
Hemangioendothelial sarcoma of bone is a rare tumor. A series of 112 cases was studied to define the clinical and pathologic features of this disease. Of the various histologic features evaluated to define the ones most significant prognostically, the histologic grade of the lesions was the single feature that correlated best with disease-free survival. In this series, patients with multi-focal tumors did not have a better prognosis than patients with unicentric disease. Surgery remains the primary treatment for these patients. Radiotherapy may be useful for surgically inaccessible lesions of the axial skeleton. Too few patients have received adjuvant chemotherapy to evaluate its usefulness in this setting.
Langerhans' cell granulomatosis (LCG) (histiocytosis X) of bone is generally considered a childhood disease. A series of 61 adult patients who presented with histologically confirmed skeletal disease was studied. The male-to-female ratio was 2.1, and 36 patients were more than 30 years old, the oldest being 71 years. Pain was the most frequent symptom. Forty-six patients has solitary lesions, six had two or three, and nine had more than three. The time interval between the development of lesions, although usually less than 2 years, ranged up to 32 years. Rib, skull, and pelvis were the most frequently involved sites. Eight patients had extraskeletal disease. Radiologically, a well-defined lytic lesion, either with no surrounding reaction or with sclerotic margins, was seen most often. The rare, poorly defined lesions tended to occur in the clavicle. Microscopically, typical Langerhans' cells were present in large numbers. Eosinophils were usually prominent. Benign giant cells, mitotic figures, and necrosis also were observed. There was no histologic difference between solitary and multiple lesions. Surgery, radiation therapy, or a combination of both usually provided adequate treatment. Only two of the solitary lesions recurred. Eleven patients suffered from chronic, progressive disease, which inconsistently responded to treatment. None of the patients in our series died of the disease.