We describe 100 consecutive patients with osteoid osteoma. Of the 97 who had operations, 89 were treated by intralesional excision and eight by wide resection. The three remaining patients were not operated on because the osteoid osteoma was almost painless, or was found in the pedicle of the 12th thoracic vertebra at the site of entrance of the artery of Adamkjewicz. The diagnosis was confirmed histologically in all specimens. No local recurrences were observed at a minimum follow-up of one year. All except one patient were mobilised two to four days after surgery. A precise preoperative diagnosis of the lesion is mandatory, based on clinical findings, standard radiographs, thin-section CT and a bone scan. We compared our operative technique with 247 cases in which the percutaneous technique of removal or coagulation of the nidus had been performed. The latter procedure has a less constant rate of primary cure (83%
We report the use of the uncemented Kotz modular femur and tibia reconstruction system after 95 distal femoral resections performed from 1983 to 1989. The average follow-up was 51 months; 62 patients had at least 36 months' follow-up and 36 at least 60 months. Complications required reoperation in 55%. The postoperative infection rate was 5% for primary cases, 6% for revision cases, and 43% for revision of previously infected cases. The polyethylene bushes failed in 42% of cases at an average of 64 months postoperatively. Stem breakage occurred in 6% and was associated with the use of narrow stems and extensive quadriceps excision. The radiological results were excellent or good in most cases and were related to the initial screw fixation, but not to age, chemotherapy, length of resection or size of stem. The clinical results were excellent or good in 75%, failure usually being associated with a complication, especially infection.
Chondrosarcomas arising from soft tissues are rare. Two different varieties are described, myxoid and mesenchymal. We have collected nine cases of the tumour, five myxoid and four mesenchymal, from a review of 513 cases of chondrosarcoma seen between 1904 and 1988. We report the principal clinical, radiographical and histological differences between the two varieties and discuss their surgical treatment and prognosis.
The size and shape of the patella make it suitable for the partial replacement of a femoral or tibial condyle resected for tumour, or destroyed by trauma. It can provide a good articular surface and may give satisfactory knee function. Nineteen cases of patellar grafts are presented, with follow-up from two to nine years. Good consolidation of the graft and fair stability of the joint were obtained; the range of movement was 90 degrees or more in 79% of cases. In contrast with an allograft of a femoral or tibial condyle, the technique described does not need an allograft bank, has a lower risk of infection, and allows better and quicker consolidation and revascularisation of the grafts, as well as a better range of movement at the knee, probably because of the lack of fibrosis from immunological reaction. Merle d'Aubigne's technique, using a patellar graft with a vascular muscle pedicle, is useful only for some cases, requires a longer period of immobilisation and weakens the extensor apparatus. Our series shows that consolidation and revascularisation of a patellar graft can occur in the absence of a pedicle.
Forty-one cases of parosteal osteosarcoma were reviewed clinically, radiologically and pathologically. The fibrous and cartilaginous elements of each tumour were graded from I to IV for malignancy. Primary intramedullary involvement was found in one third of Grade I lesions, two-thirds of Grade II and nearly 90% of Grade III lesions. Thirty-five patients with adequate follow-up were also studied and evaluated as to the adequacy of surgical management in relation to the later development of local recurrence or metastasis or both. No metastases were seen from Grade I tumours despite a number of local recurrences. One third of patients with Grade II and half of those with Grade III tumours developed pulmonary metastases and died, all with involvement of the medullary cavity before distant spread. No patients with adequate surgical management developed local recurrence; in those with inadequate treatment there was an 88% local recurrence rate.
The clinical and pathological features of six cases of desmoplastic fibroma of bone are presented. Desmoplastic fibroma is rarely seen as a primary tumour of bone; when it does occur the sites of predilection are the long bones, but other sites such as the scapula and os calcis can be involved. Radiographically the lesion tends to expand the bone from within; it is well-demarcated and lytic, often with a trabeculated soap-bubble appearance. The cellular structure and the morphological arrangement are similar to those of aggressive fibromatosis of soft tissues. Differential diagnosis from malignant spindle-cell lesions of bone is important because the treatment of choice for desmoplastic fibroma of bone is simply excision with a thin layer of healthy tissue.
Ten patients with multiple non-ossifying fibromata are reported. All had associated extraskeletal congenital anomalies such as cafe-au-lait spots, mental retardation, hypogonadism or cryptorchidism, ocular anomalies or cardiovascular malformations. The radiographic picture and the distribution of the skeletal lesions are characteristic and constant. There are lucent areas in the shaft with a sclerotic margin; these areas narrow the medullary canal or may completely fill it. It is suggested that these features characterise a new malformation syndrome, possibly allied to neurofibromatosis. After skeletal maturation is complete, the skeletal lesions may (like non-ossifying fibromata) regress and undergo spontaneous healing.
This review of 27 cases serves to emphasis that periosteal chondrosarcoma and periosteal osteosarcoma are two distinct entities. Clinically, periosteal chondrosarcoma is less painful than periosteal osteosarcoma and runs a slower course. Radiographically, periosteal chondrosarcoma tends to affect the metaphysis and contains granular or "popcorn" opacities; while periosteal osteosarcoma more often affects the mid-diaphysis and shows lytic lesions with some spicules of reactive bone perpendicular to the underlying cortex. Histologically, periosteal chondrosarcoma shows lobular well-differentiated cartilage with Grade I or II (rarely Grade III) malignancy; periosteal osteosarcoma has a chondroid matrix with some osteoid component and Grade II or III malignancy. The prognosis in periosteal chondrosarcoma is good; conservative surgery is usually effective and metastases are very uncommon. In periosteal osteosarcoma the prognosis is less satisfactory but is better than that of other osteosarcomata; wide surgical excision is, however, needed and the incidence of metastases is about 15 per cent.
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.
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.
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 Kuntscher 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.
The results are presented of thirty-seven patients with Ewing's sarcoma; ten were treated by a combination of operation, radiotherapy and cyclic chemotherapy, the remainder by radiotherapy and chemotherapy but without operation. The drugs, vincristine, cyclophosphamide and adriamycin were used in combination and were continued for two years. The follow-up ranged from twelve to sixty-two months. The mortality rate and the incidence of metastases were both markedly lower than in a comparable previous series treated by radiotherapy alone, or by operation plus radiotherapy, but all without chemotherapy. The percentage of local recurrences and of metastases was much higher in the twenty-seven patients who had radiotherapy and adjuvant chemotherapy, than in the ten in whom operation was also performed. It is suggested that on the basis of these results (and on theoretical grounds) treatment should consist of radiotherapy combined with chemotherapy plus, whenever feasible, operative excision of the primary tumour.
