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1/21. Dermatofibroma with diffuse eosinophilic infiltrate.

    Dermatofibroma, a common form of benign fibrous histiocytoma, is characterized by the presence of different cell types consisting of fibroblastic, histiocytic, and even multinucleated cells. Dermatofibromas are always accompanied by an inflammatory lymphoid cell reaction, including B cells and T cells at their border. To our knowledge, however, there is no report of an infiltration of eosinophils within this tumor. We describe a dermatofibroma on the pretibial region of an 8-year-old boy. Microscopic examination revealed a diffuse eosinophilic infiltration within the dermatofibroma, which was typically composed of histiocytic cells and mature collagen. The diagnosis of dermatofibroma was confirmed by demonstrating the immunoreactivity of tumor cells to an anti-factor XIIIa antibody.
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2/21. Malignant transformation of a giant cell tumor 25 years after initial treatment.

    The current authors report a patient in whom a malignant fibrous histiocytoma developed long after a benign giant cell tumor of bone was removed from the same site. Twenty-five years after a benign giant cell tumor of the lateral condyle of the proximal tibia had been treated by curettage and iliac bone grafting without radiotherapy, a 53-year-old woman noted progressive pain and an enlarging mass in the same area. Radiographs showed osteolytic change, whereas magnetic resonance imaging indicated a tumor arising at the site of the giant cell tumor and extending beyond the bone. Examination of an open biopsy specimen showed a high-grade malignant fibrous histiocytoma with some areas rich in giant cells. After five courses of caffeine assisted intraarterial chemotherapy, the tumor was resected with an adequate margin, and the defect was reconstructed with an implanted prosthesis of corresponding shape. The extensor mechanism of the knee was reinforced using an allograft of fascia from the tensor fascia lata muscle. The resected specimen showed a good histologic response (95% tumor necrosis) to preoperative chemotherapy. Excellent function in the knee has been regained with no evidence of disease recurrence. caffeine potentiated chemotherapy was effective in minimizing the extent of tumor excision, in this case of high-grade malignant fibrous histiocytoma representing transformation from a benign giant cell tumor.
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keywords = tibia
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3/21. De novo malignant transformation of giant cell tumor of bone.

    Two cases of malignant lesions are reported, both of which arose secondary to an originally benign giant cell tumor (GCT) of bone. The first case was a typical benign GCT, which occurred in the left proximal tibia of a 31-year-old woman. The tumor was treated by curettage and bone grafting. However, it recurred twice during 15 years of follow-up. The second recurrence showed that the lesion histologically had turned into malignant fibrous histiocytoma. The second case also started as an initially benign GCT that arose in the left distal femur of a 41-year-old man. The patient underwent curettage and bone grafting. The lesion recurred 13 years postoperatively. The histological appearance of the recurrent tumor showed it to be an osteogenic sarcoma. In both patients, radiation and never been given. Malignant transformation has rarely been reported in patients with GCT of bone who have not received radiation treatment.
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keywords = tibia
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4/21. Malignant fibrous histiocytoma associated with diaphyseal medullary stenosis.

    An unusual presentation of secondary malignant fibrous histiocytoma of the femur in a patient with diaphyseal medullary stenosis is described. The patient, a 42-year-old man, presented with a painful lump in the right knee. A radiograph of the right femur showed lytic destruction. Characteristic features of longitudinal linear striations in the metaphysis and medullary stenosis in the diaphysis were observed in radiographs of the long tubular bones. A radiograph of the pelvis showed bilateral acetabular bone scleroses. After chemotherapy, surgical resection was done. On light microscopic examination, the tumor had features characteristic of malignant fibrous histiocytoma. Specimens from the diaphyseal medullary stenosis from the femur and tibia showed typical features of bone infarction. Radiographs of other members of the patient's family showed similar features of linear striation, cortical bone thickening, and acetabular sclerosis, including wavy, open growth plate of the iliac crest. The patient's aunt had died of a bone sarcoma in the shoulder region. It is important to recognize this extremely rare clinicopathologic type of diaphyseal medullary stenosis that frequently is associated with secondary high-grade sarcomas.
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keywords = tibia
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5/21. Malignant fibrous histiocytoma arising within a bone infarct in a patient with sickle cell trait.

    Sarcoma associated with osteonecrosis or bone infarction is a rare but well-documented pathological event. In this report, a 69-year-old man with sickle cell trait presented with malignant fibrous histiocytoma (MFH) in his distal tibia. The resected tumor was found in association with a large medullary infarct that extended 10 cm proximal from the tumor site. Bone infarcts can be caused by a number of processes including corticosteroid overuse, alcoholism, dysbarism, and hemoglobinopathies such as sickle cell disease. patients with sickle cell anemia often develop osteonecrosis, but osteonecrosis has also been reported in people with sickle cell trait, albeit much more rarely. Our patient is only the third reported case of infarct-related bone sarcoma in a patient with sickle cell trait. Bone infarction may be a rare though serious consequence of sickle cell trait.
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keywords = tibia
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6/21. Postradiation malignant fibrous histiocytoma and osteosarcoma of a patient with high telomerase activities.

    BACKGROUND: An extremely rare case of postradiation malignant fibrous histiocytoma (MFH) and osteosarcoma (OS) secondary to radiation therapy for leukemia-related osteolytic lesions is presented. In addition, the telomere biology of these tumors was investigated. CASE REPORT: A 14-year-old boy was diagnosed with acute lymphocytic leukemia. The right tibia was irradiated at a total dose of 60 Gy, and the left tibia was irradiated at a total dose of 40 Gy. The left tibia developed MFH and the right tibia developed OS. RESULTS: telomere reduction (MFH 70.2, OS 70.0%) and high telomerase activities (MFH 12.1, OS 17.7 TPG) were observed. These results reflect an aggressive feature of postradiation sarcomas. CONCLUSION: prognosis for patients diagnosed with postradiation sarcoma is poor due to its aggressiveness. However, even if sarcoma occurs after irradiation in more than two fields in a single patient, improvements in prognosis are anticipated with appropriate chemotherapies and wide resection.
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keywords = tibia
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7/21. Benign fibrous histiocytoma of bone: a case report.

    Benign fibrous histiocytoma (BFH) occurs commonly in soft tissues with very few cases of BFH of bone on record. We report a case of BFH of tibia in fifty year old male who presented with supracondylar fracture femur due to trivial fall and a brief review of literature.
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keywords = tibia
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8/21. Malignant fibrous histiocytoma associated with bone infarcts: report of a case.

    A caisson worker with symmetrical bone infarcts in the tibiae demonstrated a malignant transformation of one of the bone infarcts with wide-spread metastases to the lungs and viscera. Histologically, the tumor was found to be a malignant fibrous histiocytoma and despite amputation, the patient died approximately one year following the diagnosis. This case lends credence to the concept of a cause and effect relationship existing between bone infarcts and the subsequent development of bone sarcoma.
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9/21. Malignant (fibrous) histiocytoma arising in association with a bone infarct in sicle-cell disease: coincidence or cause-and-effect?

    A patient with homozygous-S sickle-cell disease complicated by malignant (fibrous) histiocytoma arising within a tibial medullary infarct is reported. This is the ninth reported case of human sarcoma associated with bone infarct and the first associated with sickle-cell disease. Evidence for a causal relationship is presented.
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keywords = tibia
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10/21. Malignant fibrous histiocytoma and pleomorphic sarcoma in association with medullary bone infarcts.

    A malignant fibrous histiocytoma arose in the proimal tibia of a 40-year-old man who had multiple, symmetrically distributed, medullary bone infarcts of unknown etiology, involving the distal femora and the tibiae. Despite amputation and chemotherapy, widespread metastases developed and death occurred 19 months after surgery. A polemorphic sarcoma, probably representing an anaplastic malignant fibrous histiocytoma, arose in association with a single medullary infarct in the proximal humerus of a 33-year-old woman. She remains well without evidence of disease five years after treatment by radical radiotherapy followed by shoulder disarticulation. Sarcoma arising in association with bone infarction is a rare entity. Sixteen cases reported in the medical literature, including our own, are reviewed. The sarcomas arose in the tibia in nine cases, the femur in six cases and the humerus in one case. The patients were usually older individuals and 13 of the 16 were male. All but two had multiple bone infarctions. Four of the patients had caisson disease, three had what is probably an hereditary bone dysplasia, one had sickle cell disease and eight had infarcts of unknown etiology. Most patients have had a rapidly fatal outcome. Thirteen of the sarcomas have been fibrosarcomas or malignant fibrous histiocytomas, both of which are rare primary bone tumors. Analysis of the published cases of bone infarction-related sarcomas suggests that the risk of developing a sarcoma is greatest in infarcts with large medullary components.
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ranking = 3
keywords = tibia
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