Michael Bonfiglio

Giant-cell tumor of bone. An analysis of two hundred and eighteen cases.

Two hundred and twenty-two tumors in 218 patients were collected from different institutions and doctors. Eight patients had died less than eighteen months after the diagnosis was made, one of an unrelated disease, the others as a result of their tumor. The remaining 210 patients with 214 tumors were followed for an average of 9.9 years, the range being two to thirty-four years. In each case, skeletal maturity, location of the lesion, roentgenographic findings, histological characteristics, and treatment were analyzed. Skeletal maturity, as evidenced by closure of the epiphyses, was present in all patients. This observation may explain the predominance of females (27:11) in the patients less than twenty years old. More than half (55 per cent) of the lesions were located in the lower end of the femur, the upper part of the tibia, and the lower end of the radius. Other sites were the sacrum, pelvis, proximal end of the femur and fibula, and the small bones of the hands and feet. The roentgenographic findings were sufficiently characteristic to be helpful but not diagnostic. They were influenced by previous treatment of fracture. The histological features permitted grading as suggested by Jaffe, Lichtenstein, and Portis30. However, the grading was not of prognostic value although it did serve to alert the surgeon to the possibility that the tumor was malignant. A consistently accurate prediction as to local recurrence or pulmonary metastasis could not be made on the basis of the histological grade. The treatment of the tumors in this series was performed by many surgeons and varied considerably. All patients had a preliminary biopsy. The primary surgical treatment ranged from curettage or excision, with or without bone-grafting, to amputation. The success of surgical treatment, whether by curettage or resection, appeared to depend on the completeness with which the tumor was removed. After primary curettage, thirty-five of forty-five tumors recurred; whereas, after primary curettage combined with bone-grafting, twenty-two of the ninety-one tumors recurred. After primary resection, ten of the forty-four tumors recurred, whereas, after primary resection combined with bone-grafting, four of twenty-two tumors recurred. Primary amputation was performed for ten tumors none of which recurred. After primary irradiation, six of ten tumors recurred. Ninety-seven per cent of the recurrences occurred within two years of the time that the initial diagnosis was made. At the time of this study, 189 of the 218 patients were alive and well, and twenty-nine had died. Of the 189 who were alive and well, 121 had had no recurrence after primary treatment and sixty-eight had required secondary procedures. Of the twenty-nine patients who had died, fourteen had died of their tumor, three of postirradiation sarcoma, and twelve of unrelated disease. Eighteen of the twenty-nine dead patients had recurrent lesions. In the whole series, 180 secondary procedures were performed, 140 on sixty-eight patients who survived and forty on the twenty-nine patients who died. Of the 140 procedures on surviving patients, eighteen were for infection, five for lung metastases, four for fracture or non-union of a bone graft, and 113 for fifty-nine recurrences. The forty procedures done on the patients who died included one for infection, one for fracture of the bone graft, two for lung metastases, and thirty-six for recurrences. Of the seventy-seven patients who had recurrent lesions, fifty-six had one recurrence; sixteen, two recurrences; and four, three recurrences. Success in the treatment of recurrences was observed after a second curettage with or without bone-grafting in nine of sixteen patients, after secondary resection with or without bone-grafting in sixteen of thirty-five patients, after secondary irradiation in five of thirty-six patients, and after amputation in thirty-nine of forty-six patients. Irradiation, used for ten primary and thirty-six secondary tumors, was clearly beneficial in eight, possibly helpful in nine, and clearly ineffective in twenty-nine. Three of the patients so treated had postirradiation sarcoma. The average follow-up of patients who had radiotherapy was 9.2 years (the range four months to thirty-four years). In five of the six patients with pulmonary metastases, lobectomy was successful.

Melorheostosis: A Report Of The Clinical, Roentgenographic, And Pathological Findings In Fourteen Cases

1. Fourteen previously unreported cases of melorheostosis are presented. The disease progressed rapidly in the children and slowly in the adults. Contracture and deformity were the earliest findings in childhood and pain and stiffness in affected joints was the most common complaint in adult life. Soft-tissue contractures and deformities, especially in the hands and feet, were usually found and treated before roentgenograms were made which showed the characteristic hyperostosis. 2. Roentgenograms made in infancy or early childhood usually showed a faint linear hyperostosis which was difficult to diagnose. This rapidly increased in the immature skeleton and more slowly in the adult skeleton, eventually producing the appearance of bone flowing along the cortex. 3. Abnormality of the soft tissues of mesodermal origin, as manifested by contractures, was usually discovered earlier than the underlying hyperostosis, often leading to incorrect diagnoses. They usually caused greater deformity and disability than the disease in bone. 4. The pattern of involvement suggests that this disorder is a congenital abnormality akin to paraxial hemimelia and that it is initiated early in embryonic life prior to the formation of the limb buds.

Lipofibromatous Hamartoma of the Median Nerve

We have described one patient with lipofibromatous hamartoma of the median nerve in the forearm, wrist, and hand and reviewed thirteen reported cases. This uncommon entity, of probable congenital origin, occurs more often in females than in males. It presents as a mass in the volar aspect of the forearm, wrist, or palm, usually during childhood. Treatment should be transection of the volar carpal ligament with careful removal of any extraneural lipofibromatous material. Although the follow-up of cases reported in the literature has been brief, apparently such treatment produces symptomatic improvement. Excision of all or part of the mass involving the median nerve certainly produces a significant neurological deficit in the hand and is therefore contraindicat-ed.

Dysplasia Epiphysealis Hemimelica: A Report Of Fifteen Cases And A Review Of The Literature

Fifteen new cases of dysplasia epiphysealis hemimelica are reported and the literature is reviewed. The cartilage overgrowth was usually found to be limited to one extremity and to half of the affected epiphysis. The microscopic pathological changes are indistinguishable from those of an osteocartilaginous exostosis. Excision of the lesion is indicated when it produces deformity or interferes with function. Other procedures are seldom required. These lesions retain the potential for cartilage proliferation in adult life and, we presume, for malignant degeneration. They should be closely followed in the adult and be removed if they increase in size.

Non-traumatic Necrosis of the Femoral Head: Part I. Relation Of Altered Hemostasis To Etiology

Fifty patients (forty-four male and six female) with non-traumatic necrosis of the femoral head underwent evaluation at the University of Iowa Hospitals between 1951 and 1968. Their average age was forty-six years. There was bilateral involvement in thirty-six patients (72 per cent). Associated diseases of statistical significance included heavy alcohol consumption in thirty-seven patients, gout in ten, and hyperuricemia in ten. These and other associated diseases are often accompanied by disturbances in clotting mechanisms. The association constitutes indirect evidence for the existensce of a bleeding or clotting defect in forty-eight of the fifty patients. Direct evidence for a clotting defect was found in twenty-six patients. It included observations of petechiae, easy bruising, hemorrhage, and abnormal platelet counts with significant thrombocythemia and thrombocytopensia. In thirty patients a battery of studies of coagulation revealed significant abnormalities in nine patients. Based on this direct and indirect evidence we regard femoral-head necrosis as a skeletal expression of systemic disease which by a constellations of events alters coagulation homeostasis resulting in sludging, thrombosis, or hemorrhage in an area of susceptible blood supply.

Aseptic Necrosis of the Femoral Head and Non-Union of the Femoral Neck

A long-term evaluation has been made of the effect of drilling and bone-grafting for aseptic necrosis of the femoral head with or without union of the fractured femoral neck, idiopathic aseptic necrosis of the femoral head, and aseptic necrosis of the femoral head caused by traumatic dislocation. One hundred and twelve patients were followed for from two to fifteen years. The results show that the procedure is effective in restoring viability to the necrotic femoral head and continuity to the ununited femoral-neck fracture in a sufficient number of patients (78.4 per cent) to warrant its continued use in the management of these complications.

Treatment by Bone-grafting of Aseptic Necrosis of the Femoral Head and Non-union of the Femoral Neck (phemister Technique)

An evaluation has been made of the effect of drilling and bone-grafting for non-union of the fractured femoral neck or aseptic necrosis of the femoral head, or both, in fifty-three patients. The results show that the procedure is effective in restoring continuity to the ununited femoral-neck fracture, and viability to the necrotic femoral head in a sufficient number of instances to warrant its consideration in the management of these complications.

Synovial sarcoma: an electron microscopic study of monophasic and biphasic forms.

Seven cases of synovial sarcoma were studied by electron microscopy. The 4 cases of the monophasic spindle type contained elongated cells arranged in sheets and cords; ultrastructurally the cells contained many cytoplasmic processes and were scattered in an amorphous matrix. The monophasic spindle type demonstrates ultrastructural characteristics that are unique and serve to differentiate it from the biphasic pattern and from fibrosarcoma. Three cases of the biphasic variety demonstrated cytologic characteristics of a true glandular epithelium and a malignant spindle cell stroma. The epithelial nature of the biphasic variety is a feature that raises doubt whether synovium is the stem cell.

Non-traumatic Necrosis of the Femoral Head: Part Ii. Experiences In Treatment

The results of treatment by drilling and bone grafting in fifty-five hips indicate that the procedure can restore viability to the non-traumatic necrotic femoral head in a majority of instances (79 per cent). Mold arthroplasty, when warranted, may also result in a satisfactory hip.

Synovial sarcoma: a review of 33 cases.

Thirty-three patients with synovial sarcoma were studied with respect to age, sex, site, clinical presentation, pathologic findings, and primary surgical treatment. Seventy percent of the patients were in the first three decades of life, and there was a 2:1 male predominance; the overall survival rate was 36%. There was a high local recurrence rate (64%) after simple local excision, and poor survival (27%). While wide local resection gave the best results (50% survival), it also carried a risk of high recurrence. Patients treated by amputation had a 42% survival rate. Thus, it can be concluded that adequate surgical treatment improved survival and reduced the recurrence rates. The lungs were the most common metastatic site, but lymph node metastasis occurred in 27% of the patients, often after tumor recurrence. Favorable prognostic factors included youth (patients younger than 20) and biphasic or epitheloid histologic types.