Biomedical subjects
Publications and source records attributed to F Bertoni.
All cases of adamantinoma seen at the Istituto Ortopedico Rizzoli were retrospectively reviewed. Although this tumor is exceedingly rare, nine cases were collected. The tumor is composed of four histological patterns: spindle, basaloid, squamoid, and tubular. The prognosis of this tumor depends on the adequacy of therapy. Surgery that is expedient and adequate tends to offer the best prognosis.
The authors present ten cases of malignant neoplasm of the bones of the hand, four primary tumours and six solitary metastatic tumours, from the case material of the Rizzoli Institute. They stress the importance of differential diagnosis of these rare neoplasms, in particular from benign neoplastic or hyperplastic lesions, which are common in the hand.
The authors report twelve cases of soft tissue sarcoma distal to the flexor crease of the wrist. These were isolated from a total of 414 cases of soft tissue sarcoma treated at the Rizzoli Institute. This case material comprises three epitheloid sarcomas, four fibrosarcomas, two rhabdomyosarcomas, one synovial sarcoma, one myxoid malignant fibrous histiocytoma, and one myxoid chondrosarcoma. The authors deal in detail with the biological behaviour, the criteria of differential diagnosis, and the indications for treatment in each histological type of sarcoma.
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We retrospectively reviewed the records of 125 patients with chondrosarcoma seen at the Istituto Ortopedico Rizzoli. All of the patients had been followed for at least five years, and ninety-six patients had been followed for at least ten years. The requirements for the adequacy of treatment were carefully defined. Metastasis and survival were related to the histological grade of the tumor. Nine per cent of the grade-1 lesions and 44 per cent of the grade-3 lesions metastasized. Ninety-four per cent of the patients with grade-1 lesions survived for five years, compared with only 44 per cent of patients with grade-3 lesions. The ten-year survival rates were 87 per cent and 27 per cent, respectively. Adequacy of treatment had an important influence on the incidence of recurrence, length of survival, and length of disease-free survival. The incidence of recurrence in adequately treated patients was 6 per cent, but in inadequately treated patients it was 69 per cent. The five-year survival rates in these two groups were 81 per cent and 53 per cent, respectively. Seventy-eight per cent of the adequately treated patients were disease-free at follow-up (mean, 11.1 years) compared with only 6 per cent of the inadequately treated patients. We compared the results of this review with those of other reviews of chondrosarcoma.
The clinical study of encephalic ependymoma shows that cases present with polymorphous clinical pictures depending on intra and/or periventricular tumoural growth, generally such as to hinder precise diagnosis during life, unless under direct control, or, currently, using computerized axial tomography. The reported case, completed by macro and microscopic autoptic study, takes on particular importance because the neoplasia involved all encephalic ventricular cavities. This exceptional localisation and extension, only gave an aspecific clinical picture, dominated by the presence of a slight internal communicating type hydrocephalus and immediate general convulsive signs.
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Six cases of giant cell reaction of bone are presented. These are osteolytic lesions that affect the bones of the hand and foot. They are characterised by fibroblastic proliferation with the production of osteoid substance and bone, with abundant giant cells but no atypical cells. After describing the cases anatomically and clinically, the writers consider the differential diagnosis in bony lesions of the hand and foot containing giant cells. Giant cell reactions always heal after marginal excision. The most important differential diagnosis is with giant cell tumours and the bone lesions of primary hyperparathyroidism.
Three cases of clear cell chondrosarcoma are presented. This variety of chondrosarcoma should be differentiated most of all from chondroblastoma and osteoblastoma.
Three cases of the myxoid variety of malignant fibrous histiocytoma (I.F.M.) of the soft tissues are reported. This condition should be recognised as a separate entity differing from other benign pseudosarcomatous or malignant lesions of the soft tissues in that the myxoid component is present not as an occasional background, but as an integral constituent of the neoplasm. This indicates a more favourable prognosis as compared with other varieties of malignant fibrous histiocytoma.
Malignant degeneration of fibrous dysplasia is rare. It occurs with similar frequency at all ages and in both sexes. It is more frequent in cases of polyostotic than in monostotic fibrous dysplasia. In cases of fibrous dysplasia that do show malignant degeneration it is common to find that a high level of alkaline phosphatase persists in the serum, even in adults. Previous radiotherapeutic treatment appears to me a predisposing factor. Osteosarcoma is the most frequent neoplasm, followed at some distance by fibrosarcoma and chondrosarcoma. The tumour is most often localised in the femur; it is not unusual to find it in the tibia, maxilla and mandible. The treatment and prognosis are the same as those of the involved malignant neoplasm.
Two cases of histiocytosis X are reported. One affected a single bone, the other several bones. Both had the usual radiographic appearances, but a somewhat alarming cytological picture. The differential diagnosis between benign proliferative conditions and those possessing malignant or pseudo-malignant characteristics therefore demanded very careful consideration. After discussion of the radiological and histiocytological data, the diagnosis off histiocytosis X was arrived at, and in view of the favourable results, we are inclined to the view that these were atypical cases of eosinophilic granuloma.
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The authors report twenty five cases of dedifferentiated chondrosarcoma, illustrating the principal clinical, radiographic and histological features. The prognosis is particularly grave in this neoplasm: the only correct therapy is radical removal of the tumour by amputation or disarticulation.
Eleven cases of malignant fibrous histiocytoma of bone observed during recent years are reported. An analysis is presented with regard to sex, age, site, symptoms, radiography, macroscopic and microscopic aspects. The differential diagnosis is discussed on the basis of these criteria. Because of the small number of cases and limited follow up, it was not possible to come to any definite conclusions concerning prognosis and treatment. Treatment was surgical in almost all the cases.
A case of primary malignant mesenchymoma of bone is reported. It's a very rare kind of tumor of which only four cases were previously reported in the literature. It's a malignant neoplasm where histological aspects of both liposarcoma and osteosarcoma coexist. Prognosis appears to be similar to that of osteosarcoma.
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