Biomedical subjects
Publications and source records attributed to M Campanacci.
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."
Local injection of radiopaque medium demonstrated the presence of intracystic fibrous septa in 13 patients with solitary bone cyst. Contrast examination was helpful in predicting the response of solitary bone cysts to treatment by injection of methylprednisolone-acetate (MPA). As the number of septa increased, an increased difficulty in obtaining an equal distribution of MPA inside the cyst and a higher incidence of incomplete healing of the cyst was encountered.
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.
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.
Eighty cases of primary fibrosarcoma of bone from the Rizzoli Institute are reviewed. There was a slight male predominance with a wide patient age distribution. Roentgenologically, low grade fibrosarcomas had generally well-defined margins and a "soap-bubble" appearance. High grade tumors appeared permeative and more aggressive. Histologically, the majority of tumors were high grade (3 and 4). Prognosis correlated well with the grade of the tumor. The 10-year survival rate was 83% for low grade sarcomas and 34% for high grade tumors. Local recurrence was a bad prognostic sign.
Mesenchymal chondrosarcoma of bone and soft tissues treated at the Istituto Ortopedico Rizzoli are reviewed. The skeletal locations were prominent in five cases. Only two cases were in the soft tissues. Radiographic picture in the bone shows an aggressive osteolysis, with soft tissues invasion. Histologic picture is the same in bone and soft tissues, and is highly distinctive: islands of well-differentiated chondrosarcoma embedded in undifferentiated mesenchymal cells with high malignancy characteristics. The cases showed a poor prognosis, and no patient survived more than four years.
Ten patients with multiple non-ossifying fibromata are reported. All had associated extraskeletal congenital anomalies such as café-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.
We are reporting on the cases of twenty patients with periosteal chondroma to stress the importance of a proper clinical and radiographic diagnosis of this lesion. Awareness of the features of the lesion helps to prevent overtreatment of this benign condition, because the cytological findings may be ominous. Even the radiographic pattern may be suggestive of malignant disease. Periosteal chondromas apparently arise from under the periosteum of the diaphysis or metaphysis in adolescents and young adults. Close cooperation between the surgeon, the radiologist, and the pathologist is necessary to achieve proper diagnosis and treatment. Marginal excision is usually effective treatment.
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Eighty consecutive patients with localized Ewing's tumor treated with adjuvant chemotherapy for two years plus local tumor control were retrospectively reviewed. Adjuvant chemotherapy protocol was constant for all patients, but local treatment consisted of amputation, resection (complete or incomplete) plus radiotherapy, or radiotherapy alone. The follow-up ranged from 21--101 months (mean = 50 months). The mortality rate and incidence of metastasis were significantly lower than in a comparable group of 83 patients previously treated by radiotherapy, or surgery plus radiotherapy but without chemotherapy. The percentage of local recurrence was not significantly different between the two groups. Of the patients treated with adjuvant chemotherapy, the percentage of local recurrence and metastases was much lower when the primary lesion was located in the extremities and when this lesion was treated by conservative surgery followed by irradiation. This was also true when the resection was not complete. Little discrepancy in functional results was observed using either resection plus radiotherapy or radiotherapy alone. The above data suggest that in treating Ewing's sarcoma, conservative surgery, even incomplete, can play an important role when associated with radiotherapy and adjuvant chemotherapy.
A homogeneous group of 53 Caucasian subjects with high-grade osteosarcoma (OS) was typed for HLA-A and B locus antigens. Although no significant differences in the distribution of these antigens were found in comparison with 425 local controls, a trend towards an increase of HLA-B18 and decrease of HLA-B12 was observed. All the patients underwent amputation plus adjuvant chemotherapy and among the 29 patients with a follow-up longer than one year, 9 out of 10 subjects with HLA-A3 antigens developed metastases within a few months. None of the OS patients had the HLA-A3, B7 haplotype which is present in linkage-disequilibrium in the control population.
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.
The writers describe the technique of posterior hemiresection in parosteal osteosarcoma of the distal femur by means of two incisions, medial and lateral. In not previously operated cases, this operation permits wide excision and offers a good guarantee of cure. The stability and mobility of the knee remain intact. The strength of the remaining bone can be increased by autoplastic and homoplastic bone grafts.
Ninety-five consecutive cases of unicameral bone cyst treated at the Istituto Ortopedico Rizzoli by intracystic injections of methylprednisolone acetate were retrospectively reviewed. Eighty per cent of the patients had a satisfactory result. Of the 20% failures, 13.5% had a recurrence of the cyst. Recurrence after methylprednisolone acetate treatment is a previously unrecognized entity, with recurrence risk factors associated with multiloculation, cyst size and age of the patient.
The authors report on the results obtained in 15 patients with primary non-Hodgkin's lymphoma (NHL) of bone treated by radiotherapy and polychemotherapy; 86% of the patients (13 of 15) were continuously disease-free at a median follow-up of 70 months (42-104). No local recurrence was observed. The necessity of clearly distinguishing primary non-Hodgkin's lymphoma of bone from Ewing's sarcoma is discussed.
A new type of endoprosthesis of the humerus is presented. It is indicated in resection of the proximal humerus for primary and secondary neoplasms. After describing its construction and the technique for fitting it, the writers analyse the clinical results in thirteen cases. They emphasize the advantages over prostheses of other kinds: simplicity of fitting, wide possibilities of adaptation to individual cases, and the very satisfactory aesthetic and functional aspect.
Twenty year's (1959-1979) experience in the treatment of osteosarcoma at the Bone Tumor Center of the Istituto Ortopedico Rizzoli is presented. During this period 433 cases were recorded, but only 266 were considered. All the patients underwent surgery but after 1970 whole-lung irradiation (1971), immunotherapy (1971), and chemotherapy (1972 onward) were added as adjuvant therapies on a nonrandomized basis. In the group treated with surgery alone the prognosis was very poor: 10% survived nine years or more after the diagnosis, an average disease-free interval of 7.7 months and an average survival time of 13 months. Monolateral whole-lung irradiation had negative results and was abandoned after six cases. Adjuvant immunotherapy with irradiated autologous tumor cells gave moderately positive results in 16 patients, but only by delaying the appearance of first metastases, therefore increasing the time of survival. Adjuvant chemotherapy was performed with three different protocols: one protocol with ADM only and two protocols using VCR + MTX (at medium dose) + ADM, administered according to two different schedules. Superimposable results were obtained with these three regimens. With equal follow-up, the percentage of continuously disease-free patients treated with adjuvant chemotherapy was significantly higher than that of patients treated with surgery alone (P less than 0.001). The patients in the chemotherapy group who had relapses showed a prolonged time (mean = 12.3 months) to the onset of the first metastasis. Adjuvant chemotherapy caused virtually no morbidity and no deaths. Reference is made to the advantages of a large and homogeneous caseload deriving from a single institution to avoid preselection bias and evaluate the effectiveness of new therapeutic approaches when patient randomization has not been employed.