Biomedical subjects
Publications and source records attributed to D C Dahlin.
The clinical and pathologic features of five cases of adenoacanthoma of the colon are presented. Although the lesion is rare, both the pathologist and the surgeon should be aware of this unusual neoplasm in order to avoid error in diagnosis, especially when metastatic lesions are being evaluated. In our experience, these rare colonic lesions are more aggressive than ordinary carcinomas of the colon.
The ultrastructural features of a malignant histiocytoma of the acromial process of the scapula were studied. Material was obtained from two surgical biopsy specimens and an amputation specimen from the tumor. Cells possessing characteristics of histiocytes, fibroblasts, xanthoma cells, and multinucleated giant cells were present throughout the tumor. Smaller numbers of undifferentiated cells and lymphocytes were also observed. Intimate cytoplasmic interdigitations between adjacent tumor cells were found, and instances of degenerating intracytoplasmic cells, possibly representing phagocytosis, were observed. Specimens stained with periodic acid-Schiff reagent with and without exposure to diastase, examined by light microscopy, showed that numerous cells contained phagocytized material consisting of degenerating cells rather than cytoplasmic glycogen. Intraumor lymphocytes apparently represented an inflammatory reaction to the tumor. The tumor giant cells and xanthoma cells were probably modified histiocytes. Results of the study were compared with previous reports of ultrastructural studies of malignant histiocytoma of soft tissues. Fundamental similarities between such studies and this one suggested that the progenitor cell is a histiocyte, whether arising in bone or in soft tissues, and that the progenitor cell is capable of differentiation in both histiocytic and fibroblastic directions.
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Survival of patients with primary osteosarcoma who received traditional surgical management has been very poor. Several studies have reported the percentage of patients alive 3 years after first treatment to be between 20 and 25%. In a study of such patients first treated at the Mayo Clinic between Jan. 1, 1963, and July 1, 1974, the survival noted in the first few years was typical of that of other reports, about 25%. However, by 1972 through mid-1974, the 3-year survival had become 50%. This improvement was not the result of adjuvant treatment or any basic change in type of patient treated, yet it is similar to that reported in connection with some of the new adjuvant therapies used in trials depending on historical controls. Our results cast doubt on findings made in the absence of concurrent, randomly selected controls.
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There appears to be a total of five cases of chondrosarcoma of the hyoid bone (not all associated with Gardner's syndrome). The case reported herein is one that was first reported in the literature on Gardner's syndrome and the second reported instance of a patient with Gardner's syndrome who had a malignant bone tumor.
A study of 31 cases of giant-cell tumor of vertebrae above the sacrum indicates that this unusual diagnosis is sometimes appropriate. Several entities, especially aneurysmal bone cyst and osteoblastoma, should be considered in the diffferential diagnosis. Evidence indicates that excision, sometimes with ancillary irradiation, provides a better chance for cure than would be expected with giant-cell tumor in other sites--sites where recurrent, generally large tumors develop in nearly half of the cases. Giant-cell tumors of vertebrae may affect vertebral bodies or arches and may cause irreversible damage to the spinal cord or to the emerging roots.
Malignant (fibrous) histiocytoma is currently defined as a malignant primary bone tumor that contains a mixture of fibrogenic cells and cells that are histologically similar but which appear to be histiocytic. In this type of histiocytoma the nuclei are often indented; cytoplasm is usually abundant and may be slightly foamy; nucleoli are often large; and multinucleated malignant cells are usually a prominent feature. Many dedifferentiated chondrosarcomas, osteosarcomas, and fibrosarcomas of bone contain areas that resemble what we regard as malignant (fibrous) histiocytoma. When the entirety of a malignant tumor of bone fits the outlined histologic pattern, the designation of malignant (fibrous) histiocytoma seems appropriate. From 158 fibrosarcomas of bone and 962 osteosarcomas of bone in our files, 35 tumors were segregated because they appeared to be properly designated as malignant (fibrous) histiocytoma. A wide age range was represented by affected patients, and a large variety of bones harbored these tumors. Approximately one-third of patients eligible for 5-year follow-up were long-term, symptom-free survivors. Four deaths occurred from the tumor after more than 5 years, and one patient had radiographic evidence of pulmonary metastasis 7 years after amputation. Radiation therapy has been curative in at least two cases. The correct designation for these tumors in the light of current knowledge is malignant tumor, consistent with malignant (fibrous) histiocytoma.
A series of 27 patients with intramedullary osteogenic sarcomas that were so well-differentiated histologically as to be confused with benign lesions was studied. Of the 27 patients, 14 were females and 13 were males, and most were adults. The tibia and femur were involved commonly. Roentgenographically, the tumors showed a mixture of sclerosis and lysis with poor margination. However, they lacked the highly destructive appearance of high-grade osteosarcomas. Histologically, spindle cells with minimal cytologic atypia were arranged in interlacing bundles. Mitotic figures were scarce. The amount of osteoid was variable, commonly being abundant. With recurrence, three tumors became highly anaplastic. Excision of tumor almost invariably led to recurrences. Resection or amputation has led to good results in seven patients. Only three of the 27 patients died with metastatic tumor; one patient is alive with metastasis. Central low-grade osteosarcomas have been misdiagnosed frequently and hence treated inadequately. Careful roentgenographic and histologic correlation is required to make the diagnosis.
Osteosarcoma of bone is a recognizable entity if the histopathologist designates tumors as such when their malignatn cells produce osteoid substance even if only in small foci. Such definition distinguishes this lesion from other sarcomas that arise in bone, especially chondrosarcoma and fibrosarcoma. There is a general tendency to consider that osteosarcomas represent a stereotyped form of disease for which new modalities of treatment can be applied and assessed. The question of whether a given osseous lesion is actually malignant and not a benign neoplasm or even a reactive non-neoplastic condition simulating a malignant tumor may be difficult for the histopathologist. Pathologists without considerable experience in the diagnosis of bone tumors find this question especially vexing. The establishment of a valid diagnosis of osteosarcoma introduces the additional problem that the 11 varieties considered in this paper may pose significant recognizable variations in the clinical capability of the disease. It is apparent that the physician must recognize the known clinicopathologic and prognostic factors of these subtypes in his assessment of the overall problem.
The presence of more than one distinct giant-cell tumor in the same patient is rare; seven cases have been reported in the literature. The present series involves eleven patients with thirty-five individual tumors. Multifocal giant-cell tumors may pose a diagnostic problem. It is difficult to distinguish a multicentric giant-cell tumor from a primary giant-cell tumor with metastatic spread. This problem is magnified when the second lesion is not noted at the onset or is metaphyseal in location. Moreover, these lesions present a therapeutic challenge to the surgeon. Multiple lesions tend to exhibit the same aggressive clinical behavior as a solitary giant-cell tumor. A high recurrence rate after curettage indicates that aggressive surgical management is necessary.
Three cases of myxomas of bone are presented. No similar tumor, except in bones of the jaw, was found in a study of more than 6,000 bone tumors in the files of the Mayo Clinic to Jan. 1, 1976. These three tumors were found in a series of about 5,000 lesions of bone in patients sent to the Mayo Clinic for consultation. All three of these tumors affected the femur. Care was taken to exclude lesions such as chondrosarcoma, fibrosarcoma, chondromyxoid fibroma, and ganglia of bone, all of which may contain prominently myxoid zones.
Four patients with extra-articular synovial chondromatosis and one patient with combined intra-articular and extra-articular involvement are described. In all five patients the lesion was anomalously located (knee, shoulder, hip, and ankle). The lesion usually occurs in the hands and feet. Accurate diagnosis is important, because if the synovial origin of the cartilaginous proliferations is ignored, the evidence of cellular activity may lead to an erroneous diagnosis of chondrosarcoma. All five patients had a benign clinical course.
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A clinicopathologic study of 79 patients with parosteal osteogenic sarcoma revealed that more females than males were affected and that most of the patients were in the second to the fourth decades of life. Sixty-eight percent of the patients had involvement of the posterior aspect of the lower femoral shaft. The roentgenogram characteristically showed a large, dense lobulated mass attached by a broad base to the underlying bone but with no involvement of bone itself. Microscopically, the tumor presented as well-formed bands of osteoid within a hypocellular spindle cell stroma. Seven of the lesions had foci of high-grade osteogenic sarcoma within an otherwise typical parosteal osteogenic sarcoma. Six of the 79 lesions showed involvement of the medullary cavity at surgery. Thirty-one patients had excision as their initial treatment; four of the 31 required nv further therapy. Ten patients had resection initially; three of the ten had recurrence. Only four of 27 patients who underwent amputation initially developed pulmonary metastasis. The presence of histologically "active" tumor and medullary involvement seemed to affect the prognosis adversely. Our data indicate that complete radical removal of the tumor is the treatment of choice, with resection when feasible and amputation when necessary.
A study of 102 osteogenic sarcomas of the bone surface revealed that 79 were parosteal osteogenic sarcomas. Roentgenographically, these 79 were dense, lobulated lesions attached by a broad base to underlying bone, usually the lower femoral shaft. Histologically, they were low-grade osteosarcomas. The other 23 lesions (periosteal osteogenic sarcoma) usually involved the upper tibial shaft and presented as small radiolucent lesions on the surface, with formation of spicules of bone perpendicular to the bone shaft. Histologically, these 23 were relatively high-grade, predominantly chondroblastic osteogenic sarcomas. Thirteen of the 23 patients were males, and most were in the second decade of life. Of five patients who had excision of the tumor, two had recurrence. Seven of 13 patients who underwent amputation initially were alive without disease at last follow-up. Only 4 of the 23 patients have dies of metastatic disease.
Of approximately 1,999 cases of osteogenic sarcomas at the Mayo Clinic, 25 were diagnosed as telangiectatic osteogenic sarcomas. Of the 25 patients involved, 16 were males and 9 were females, and their ages ranged from 6 to 49 years. Six patients had had pathologic fracture. The lesions were typically located centrally and usually in the distal femur or proximal humerus and roentgenographically were large and purely lytic with destruction of cortex. Grossly, the lesions were cystic and contained clotted blood. Histologically, cystic spaces that contained blood were lined with anaplastic spindle cells and benign giant cells; sometimes, there were so few malignant cells that diagnosis was difficult. Usually, fine, lacelike osteoid was present. Of the 25 patients, 23 have died of metastatic disease, and another has developed pulmonary metastasis 11 months after amputation. Only one patient has survived for more than five years; however, he has developed pneumothorax. Data from this series suggest that the outlook in telangiectatic osteogenic sarcoma is more bleak than in conventional osteosarcoma.
Benign osteoblastoma is a rare tumor, more frequent in males, usually occurring in patients under the age of 30 years, and is seen most frequently in the spine, femur, tibia, and mandible. Its varied roentgenographic appearance may suggest a large osteoid osteoma or an aneurysmal bone cyst, but about one-fourth of cases present a picture consistent with a malignant neoplasm. The roentgen changes in the spine are frequently subtle and require care for identification. Differentiation from osteogenic sarcoma is sometimes difficult even with histological material, because some low grade osteogenic sarcomas contain areas resembling osteoblastoma. Conservative surgery is the treatment of choice for osteoblastoma.