Biomedical subjects
Publications and source records attributed to M Campanacci.
The key to the solution of this problem lies in the differential diagnosis. There are at least four dysplastic lesions of the congentially bowed tibia (with or without cysts) which have quite different behaviour patterns. There are also six different types of true congenital pseudarthrosis which exhibit similar differences in behaviour and response to treatment.
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 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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Osteofibrous dysplasia of the tibia and fibula is not a well recognized entity. We have seen thirty-five patients with the disease. Twenty-two comparable cases have been reported in the literature with such diagnoses as ossifying fibroma, congenital fibrous dysplasia, and congenital fibrous defect of the tibia. The main differential diagnosis is with fibrous dysplasia and with adamantinoma of a long bone. Twelve of our patients had long-term follow-up and some of the lesions regressed spontaneously. Osteofibrous dysplasia seldom has even a moderate tendency to progress during childhood, but it recurs frequently after curettage or subperiosteal resection. Such recurrences generally are moderately progressive or not progressive at all. Any progression of the lesion comes to an end after puberty. Attempts at radical surgery either primarily or after recurrence do not seem to be necessary. Surgery should be delayed as long as possible and should be restricted to extensive lesions. The results of surgical treatment usually are good even in patients with a recurrence, fracture, or pseudarthrosis.
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.
Twenty-nine cases of hemangioendothelioma (H.E.) of the bone have been studied. In addition to the clinical and radiologic features of the tumor, attention was principally paid to the relationships between a tentative histologic grading of malignancy and the clinical course and final outcome of the cases. Three histologic grades are identified: Grade I H.E., Grade II H.E., and Grade III H.E. (or hemangiosarcoma). Grades I and II H.E. are frequently multicentric in the same lower limb. Grade I H.E. has a constantly good prognosis. It may remain stationary for several years even without treatment and it may be cured even by curettage or radiation. Grade II H.E. often has a good prognosis. In one of our cases the initial biopsy was interpreted as being a low grade tumor but the subsequent histology of the local recurrence indicated a Grade III malignancy and the patient died with metastases. Another case was graded II and the patient died with metastases. There are three explanations for this discrepancy: (1) some tumors are malignant in spite of a seemingly low grade histology; (2) low grade and fully malignant areas are present in the same tumor; (3) malignancy may progress in some low grade tumors in the course of time. On the basis of our experience we lean towards the last two possibilities, and therefore recommend histological study of large and multiple sections. Grade III H.E. has a very bad prognosis. The study of H.E. of the bone presents several problems. More information from a larger series of cases is needed to define the value of the histological grading in determining a prognosis, therefore indicating treatment.
Fifty-five cases of osteosarcoma of the extremities were treated between 1972 and 1976 by combined surgery and chemotherapy (vincristine, adriamycin and methotrexate in medium doses) for 18 months. The follow-up ranges from 30 to 80 months (mean = 48 months). Twenty-six patients remained free from any evidence of disease, two had local recurrences but no metastases and 27 had metastases (four of these also had local recurrences). In 12 patients, the metastases appeared after the end of chemotherapy. Both metastases and local recurrences were more frequent in patients who had segmental bone resection (7/8) than in those treated by more radical surgery (22/47). Comparison with an "historical" group (94 osteosarcoma patients treated by operation alone in our Institute between 1960 and 1971) showed that the percentage of patients free from evidence of disease was higher in the group who receiving chemotherapy. In addition, the appearance of metastases in this group was delayed (mean = 16 months) as compared with the historical controls (mean = 8 months). On the other hand, after the same kind of operative treatment, the rate of local recurrences and the time of their appearance was almost identical in both groups.
Two hundred and forty-eight high-grade central osteosarcomata were treated by amputation or disarticulation; in 5.2 per cent the tumour recurred at the amputation site. The following causes may be responsible for local recurrence: the level of the amputation is too close to the tumour; there is an unrecognised intramedullary extension of the tumour; during a previous block resection tumour cells may have been seeded in the soft tissues; the primary tumour was too extensive even for radical surgery; "skip" metastases may have been present; iatrogenic tumour implantation may have occurred while a biopsy was being performed during the course of an amputation. Treatment of the primary osteosarcoma should take all these possibilities into account. In our experience adjuvant chemotherapy has not significantly changed the frequency of local recurrences which should be treated by radical operation or, if this is not possible, by irradiation; chemotherapy may be used as an adjuvant. The prognosis of local recurrences is bad.
The authors present a study of fifty-seven cases of pseudo-tumoral ossification of the muscles ("circumscribed ossifying myositis") and of the periosteum, supported by clinical, radiographic and histological evidence. This pathological condition is characterised by the formation of hyperplastic bone callus in muscle or periosteum, with a typical radiographic appearance, and with a relationship to trauma. We have excluded from our study pseudo-tumoral periosteal ossification in conditions of congenital origin (osteogenesis imperfecta, neurofibromatosis, Sane et al., 1971; Kullman et al., 1972), hyperplastic fracture callus in cranial trauma, ossification of muscles in limbs which are paretic or paralysed due to cerebral or cord lesions, and hyperplastic fracture callus in congenital syphilis. The form which is the subject of this study is absolutely benign, develops over a period of twelve to twenty-four months, with spontaneous maturation and partial regression. It can abe treated by radiotherapy to accelerate maturation, or by surgery when maturation has been completed.
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.
Fifty-five cases of osteosarcoma of the extremities were treated between 1972 and 1976 with combination surgery and polychemotherapy (vincristine, adriamycin and methotrexate at medium doses) for 18 months. Their follow-up presently ranges between 30 and 80 months (mean = 48 months). Twenty-six patients remained free from disease signs, 2 showed local recurrence but no metastases, and 27 exhibited metastases (4 of these also had local recurrences). In 12 patients, the metastases appeared after the end of chemotherapy. Both metastases and local recurrences were more frequent in those patients submitted to segmental bone resection (7/8) than in those treated by more radical surgery (22/47). Comparison with a historical group (94 osteosarcoma patients treated with surgery alone at our Institute between 1960 and 1971) revealed that, during the follow-up period considered, the percentage of patients free from disease signs was higher in the group that also received chemotherapy. In addition, in this group metastatic appearance was delayed (mean = 15 months) as compared to historical controls (mean = 8 months). On the other hand, after the same kind of surgery, the rate of local recurrences and the time of their appearance was practically the same in both groups.
Resection of the distal femur or proximal tibia en bloc has been performed on twenty-six patients with primary bone tumours. The gap was filled with autogenous bone grafts stabilised with a long intramedullary nail, thus arthrodesing the knee. In two cases temporary stabilisation with a Küntscher rod and acrylic cement was adopted because of adjuvant chemotherapy. Union was achieved in twenty-four cases (92 per cent). Infection was the main and practically the only major complication, occurring in five (19 per cent) of the cases: it healed with union in three, healed with non-union in one, and led to an above-knee amputation in the fifth case. Follow-up has been from one to eight years with an average of four years.
Resection of the distal end of the radius is indicated in the treatment of locally aggressive primary bone tumours, such as giant cell tumour. This requires reconstruction of the resected segment. Various techniques are examined and the one using an autoplastic fibular graft is described in detail. This method preserves good function of the wrist joint.
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.
The authors discuss their findings resulting from an analysis of a large series (195 cases) of Ewing's sarcoma. In particular they compare the clinical and radiographic pictures and prognosis prior to and after the introduction of adjuvant chemotherapy. This reveals such marked differences in the past and present features of this neoplasm that some of our previous criteria and attitudes to diagnosis and treatment have undergone radical reappraisal.
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.