1/41. Two cases of secondary angiosarcoma arising from fibrous dysplasia.Angiosarcoma associated with fibrous dysplasia is very rare. We have recently experienced two cases of angiosarcoma that secondarily arose from fibrous dysplasia. The first patient, a 55 year-old man, had noticed a deformity in the right upper arm since he was five years old. At the age of 25 years, polyostotic fibrous dysplasia was diagnosed by X-ray examination. The patient complained of swelling and pain around the left shoulder. The diaphysis of the humerus was mostly non-observable due to severe bone destruction by tumor invasion and there was a large soft tissue tumor. biopsy examination revealed grade II or III hemangioendothelioma with typical histologic findings of fibrous dysplasia. After interscapulothoracic amputation for wide tumor resection, he died of DIC. autopsy revealed multiple liver metastatic lesions of angiosarcoma. The second patient was a 66-year-old woman. She presented with a pathological fracture in the right tibia, due to an osteolytic lesion with cortical bone swelling and thinning. The histologic diagnosis was fibrous dysplasia without any sarcomatous changes. However, after a 3-time recurrence, angiosarcoma arose from the same lesion. She is now still alive with disease-free period of 6.5 years after amputation.- - - - - - - - - - ranking = 1keywords = sarcoma (Clic here for more details about this article) |
2/41. Fibrous dysplasia arising from the calcaneus.A case of an 18-year-old woman with fibrous dysplasia arising in the calcaneus, which is extremely rare, is reported, with the emphasis placed on differential diagnosis from low-grade central osteosarcoma. She had a severe pain in her left ankle after sprain. Plain radiographs showed a radiolucent lesion measuring 6.3 x 2.5 cm with a sclerotic margin in the left calcaneus. CT scans showed a well-defined lytic lesion with disruption of the lateral cortex and an ossification or calcification in its center. On MR imaging, the lesion had isointensities and high intensities on T1 and T2 weighted images, respectively, but its central portions showed lower intensities both on T1 and T2 weighted images. The lesion was enhanced with gadolinium except for the central portions. The specimen obtained by open biopsy consisted of fibrous tissue and foci of irregular woven bone. None of the nuclear atypia, mitoses, longitudinal stream of bone or invasive nature of growth was detected. The diagnosis of fibrous dysplasia was histologically made. The lesion was curetted and packed with autogenous bone chips. No evidence of recurrence was noted postoperatively.- - - - - - - - - - ranking = 14.951520315887keywords = osteosarcoma, sarcoma (Clic here for more details about this article) |
3/41. Repeated dedifferentiation of low-grade intraosseous osteosarcoma.Low-grade intraosseous osteosarcoma is an uncommon bone tumor that is characterized by minimum cytological atypism and a much better prognosis than conventional osteosarcoma. This report describes a patient who had a low-grade osteosarcoma that mimicked fibrous dysplasia (FD). The tumor had an area of high-grade sarcoma at the initial diagnosis. Ten years after incomplete resection of FD-like tumor, local recurrence with areas of high-grade tumor developed. This case illustrates the potential of dedifferentiation in low-grade intraosseous osteosarcoma.- - - - - - - - - - ranking = 119.72327363821keywords = osteosarcoma, sarcoma (Clic here for more details about this article) |
4/41. Establishment and characterization of a cell line from a malignant fibrous histiocytoma of bone developing in a patient with multiple fibrous dysplasia.PURPOSE AND methods: In order to provide material for genetic analysis of fibrous dysplasia (FD), a cell line designated GBS-1 was established from a secondary bone malignant fibrous histiocytoma (MFH) developing in a rib of a 44-year-old male polyostotic FD patient. RESULTS: The GBS-1 cells are characterized by a pleomorphic spindle cell morphology with abundant mucus production. On transplantation to nude mouse subcutis the cell line forms myxoid-spindle cell sarcomas with giant cells, the myxoid product being positive for periodic acid-Schiff (PAS) and alcian blue (Al-B) stains and completely digested by hyaluronidase, mimicking the original tumor. Chromosome and genetic analyses revealed multiple structural and numerical abnormalities of chromosomes with a large number of unidentifiable chromosomes and p53 mutation in exon 7 with LOH in the counterpart. CONCLUSIONS: Since cell lines for FD have hitherto not been available, the GBS-1 cells should prove useful for genetic analyses of FD and also MFH of bone origin.- - - - - - - - - - ranking = 0.11111111111111keywords = sarcoma (Clic here for more details about this article) |
5/41. Angiosarcoma of the chest wall in a patient with fibrous dysplasia.We have experienced a case of angiosarcoma of the chest wall in a patient with fibrous dysplasia. The patient was a 64-year-old man and had large bilateral thoracic masses. Each tumor, resected metachronously with the chest wall, was diagnosed as angiosarcoma. One of these tumors had a histological component of fibrous dysplasia. The patient died from brain metastasis 19 months after the first operation. Angiosarcoma associated with fibrous dysplasia of the chest wall is very rare.- - - - - - - - - - ranking = 0.77777777777778keywords = sarcoma (Clic here for more details about this article) |
6/41. Malignant fibrous histiocytoma and osteosarcoma in association with fibrous dysplasia of bone. Report of three cases.Three cases of bone sarcomas arising in fibrous dysplasia (FD) without prior radiation therapy were reported. One of the tumors was a conventional osteoblastic osteosarcoma (OS) and the other two were malignant fibrous histiocytomas (MFH). The lesion of FD was monostotic in two patients and polyostocic (monomelic) in one. Bone sarcomas and FD were detected simultaneously in all cases. One of the MFH cases died 6 months after diagnosis, and the other MFH case and the OS case are alive without disease 3 years and one and a half years after surgery, respectively. Conservative treatment has been selected in most of the patients of FD. We should closely follow up these patients, because bone sarcomas such as OS and MFH could arise at the site of FD. This is the first report of the cases of MFH of bone arising in FD.- - - - - - - - - - ranking = 75.09093491277keywords = osteosarcoma, sarcoma (Clic here for more details about this article) |
7/41. Fibrous dysplasia.The controversy is this case centers around the management of this lesion, not the differential diagnosis. All the consultants agreed that the history, physical, and CT findings were consistent with various fibroosseous lesions, the most likely being fibrous dysplasia. The need for additional tests varied with a bone scan (Dr. Kearns), a bone scan and CT scan (Dr. McGill), and MRI, MRI angiogram, bone survey, BUN, creatinine, calcium, and phosphorus (Dr. Potsic). Cosmetic and functional changes were considered priorities for the consultants, with orbital compression, malocclusion, tooth eruption, nasal obstruction, and sinusitis (Dr. Kearns), nasolacrimal duct obstruction and orbital compression (Drs. McGill and Potsic) being the concerns. Because this lesion is benign and slow-growing, the consensus is that surgery should be reserved for functional or cosmetic compromise. But how aggressive should one be and what approach should be used? The approaches varied with midface degloving or lateral rhinotomy (Dr. Kearns), midface degloving (Dr. McGill), or a Caldwell-luc and lateral rhinotomy (Dr. Potsic). Assessment of this tumor postoperatively should be with patient examinations and serial CT scans. None of the consultants worried about sarcomatous changes in this tumor.- - - - - - - - - - ranking = 0.11111111111111keywords = sarcoma (Clic here for more details about this article) |
8/41. osteosarcoma in a patient with McCune-Albright syndrome and Mazabraud's syndrome: a case report emphasizing the cytological and cytogenetic findings.Osteosarcomatous transformation in fibrous dysplasia is unusual. The incidence is increased in patients with concomitant Mazabraud's syndrome and McCune-Albright syndrome. We report the cytological, histological, and cytogenetic findings of this rare entity arising from a mass in the right elbow of a 44-year-old African-American woman. The fine-needle aspiration (FNA) findings were diagnostic of malignancy, with markedly atypical spindle and polygonal cells admixed with osteoid. The diagnosis of osteosarcoma by FNA was subsequently further confirmed by histological evaluation of an above-elbow amputation specimen. fluorescence in situ hybridization and comparative genomic hybridization demonstrated trisomies of chromosomes 5 and 7 in the fibrous dysplasia and osteosarcoma. In addition, multiple chromosomal abnormalities were also noted in the osteosarcoma. We are unaware of any previous reports of the cytogenetic findings in the tissue of this rare condition, and argue for the value of FNA in the evaluation of such patients under selected conditions.- - - - - - - - - - ranking = 45.410116503217keywords = osteosarcoma, sarcoma (Clic here for more details about this article) |
9/41. Synovial sarcoma associated with osteofibrous dysplasia. A case report and review of the literature.We report on a 14-year-old boy who demonstrated an unusual association between osteofibrous dysplasia and synovial sarcoma. This case suggests that a patient who presents with osteofibrous dysplasia of the tibia can eventually develop a malignant musculoskeletal tumor in the same anatomical location (same limb). This experience suggests that a patient who presents with osteofibrous dysplasia should be followed up for the possibility of a coexisting synovial sarcoma in the same leg.- - - - - - - - - - ranking = 0.66666666666667keywords = sarcoma (Clic here for more details about this article) |
10/41. Mazabraud's syndrome: intramuscular myxoma associated with fibrous dysplasia.The association of fibrous dysplasia and intramuscular myxoma is a rare disease known as Mazabraud's syndrome. Both lesions tend to occur in the same anatomical region. The relationship between fibrous dysplasia and myxoma remains unclear, where an underlying localized error in tissue metabolism has been proposed to explain this occasional coexistence. Another example of this syndrome in a 52 year-old woman is reported. The patient presented with a soft tissue mass at the anteromedial mid part of the left thigh. After excision of the mass, three separate bone lesions were detected in her control MRI. The soft tissue mass was misdiagnosed as liposarcoma in another center, and the bone lesions were interpreted as metastasis. The hypocellularity and the indistinct vascular pattern of the lesion were consistent with myxoma. The Jam-Shidi needle biopsies of the osseous lesions were diagnosed as fibrous dysplasia. The recognition of this entity is important for appropriate management of the patient. patients with soft tissue myxomas should be thoroughly examined for fibrous dysplasia. The greater risk of sarcomatous transformation in fibrous dysplasia with Mazabraud's syndrome should also be kept in mind.- - - - - - - - - - ranking = 0.22222222222222keywords = sarcoma (Clic here for more details about this article) |
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