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Primary leiomyosarcoma of bone is a very rare tumor. Four cases are reported: 3 tumors were located in the femur and 1 in the talus. Clinical and imaging findings including radiographs, CT, and MRI are described. The final diagnosis was made by histopathological, immunohistochemical, and/or ultrastructural study after biopsy.
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Electron microscopy of a case of adamantinoma of the tibia shows features which support a mesenchymal angioblastic origin rather than epithelial. Comparison is made between tissue from this tumour and a squamous cell carcinoma of the femur arising in chronic osteomyelitis. In adamantinoma no desmosomes are found between tumour cells and their cytoplasmic ultrastructure shows features of mesenchymal cell type including evidence of pinocytic activity and bundles of filaments resembling hyperplastic endothelial cells. The stroma shows features similar to fibrous dysplasia of bone with fibroblasts, histiocytes and fibrolipoblastic lipid-laden mesenchymal cells. It is condluded that adamantinoma of the tibia should be considered to be an angioblastic tumour of bone.
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Fifteen cases of a recently described variety of osteosarcoma, periosteal osteosarcoma, were studied to define their radiographic characteristics. All the lesions were limited to the cortex that was always thickened but without invasion of the medulla cavity. The tumors presented as non-homogeneous masses of spiculated osteoid matrix progressively denser from the periphery to their cortical base. The average age was 28 and the most common location was the proximal third of the femur; all the lesions were diaphyseal in location. The spectrum of radiographic features and differential diagnosis are described in detail.
Experience with 56 cases of osteoarticular allograft transplantation of the extremities is presented. Cadaveric allograft including a joint surface and a variable amount of shaft is used in the treatment of aggressive benign and low-grade malignant tumors involving a long bone near a joint. The preoperative radiographic evaluation is outlined. Thirty-four patients had an uncomplicated course. Twenty-two patients had complications including aseptic fracture, infection, and tumor recurrence. The radiographic spectrum of both complicated and uncomplicated cases is illustrated.
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Scintigraphy in 3 patients with chondroblastoma showed that the tumors were hyperemic and avidly accumulated the radionuclide. These changes were also present in adjacent normal bone, but to a lesser degree. This suggest that radionuclide uptake in chondroblastoma is a function of the blood supply to the tumor rather than primary matrix extraction.
Desmoplastic fibroma (DF) of bone is a rare, nonmetastasizing but locally aggressive tumor that has been discussed infrequently in the radiology literature. The radiographs from 107 previously published cases of DF and seven cases from the authors' institution were analyzed to better understand and define its radiographic characteristics. DF was most common in the mandible, pelvis, and femur. A geographic pattern of bone destruction, with a narrow zone of transition and nonsclerotic margins, was seen in 80 (96%) patients with intraosseous DF for whom radiographs were available (83 patients). Internal pseudotrabeculation was seen in 76 (91%). Although widening of the host bone due to gradual apposition of periosteal new bone was common, occurring in 74 (89%) patients, distinct periosteal new bone occurred in only two (2%) patients with DF of intraosseous origin. The cortex was breached in 23 (28%) patients. Three cases of DF arising in the periosteum were identified and were differentiated radiographically from desmoid tumors of intraosseous or soft-tissue origin.
In 142 circular lung foci of 78 patients we determined the tumor doubling times (13 bronchial carcinomas, 7 benign pulmonary tumors, and 122 pulmonary metastases). They fluctuated between + 12 and + 8,863 days of tumor growth and a diminuation of -1,810 days of half-life. 44% of all pulmonary metastases doubled their volumes within 60 to 250 days. During the observation period the times of tumor growth varied not only in different pulmonary metastases of the same patient, but also in solitary circular lung foci of the same person considerably. There is no connexion between tumor doubling time and histological tumor type as well as the interval between surgery of the primary tumor and occurrence of pulmonary metastases. Though we found in patients of 10 to 30 years of age only quickly growing pulmonary metastases with a tumor doubling time of less than 60 days a corrlation between time of tumor growth and age could not be proved. By the determination of the tumor doubling time neither binding statements on the nature of a circular lung focus can be made nor clinical consequences for diagnostics or therapy be drawn.
We report on 3 cases of male late-adolescent/young-adult osteogenic sarcoma patients who refused complete chemical therapy according to the treatment plan "COSS 80". They all originated from disturbed family situations. Characteristic was their early independence and autonomy, dominance tendency, low level of self control, and high level of sociability. Primary motivation for refusal was the experienced intolerability of the chemotherapeutical side-effects and extreme fear of medical procedures (e.g. injections).
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Angiography was performed before a stabilizing operation in 4 patients with bone metastases to the femur or pelvis. In 2 patients the tumor was embolized with pieces of Spongostan before resection and in 2 the angiographic finding excluded resection and only nailing was performed. If resection is planned angiography and embolization should be performed in order to decrease blood loss during surgery.
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The usefulness of arteriography in the diagnosis and management of bone tumours has been evaluated in 78 cases (49 malignant neoplasms, 17 benign tumours and 12 inflammatory lesions). Using well-defined criteria, of which the presence or absence of tumour vessels is of decisive importance, arteriography represents - not as a routine procedure but in selected cases - a most useful guide to establishing the diagnosis, by adding important information to standard radiography, by defining the extent of the lesion and by assisting in the selection of the best site for biopsy.