Cases reported "Fibrosarcoma"

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1/45. Transformation of ameloblastic fibroma to fibrosarcoma.

    The direct transformation of an ameloblastic fibroma into a fibrosarcoma in a 16-year-old Caucasian male is reported. Although no ameloblastic epithelium was found in the recurrent tumor, the odontogenic origin of the fibrosarcoma was evident. The ameloblastic fibrosarcoma and the fibrosarcoma of identical odontogenic origin represent an entity which should be distinguished from conventional fibrosarcoma as these tumors demonstrate different clinical behaviors.
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2/45. Low-grade fibrosarcoma with palisaded granulomalike bodies (giant rosettes): report of a case that metastasized.

    "Hyalinizing spindle cell tumor with giant rosettes" is a tumor recently described by Lane et al. and thought by them possibly to represent a form of low-grade fibromyxoid sarcoma. Proof of a metastatic potential was lacking. We report an example of this tumor on the arm of a 28-year-old woman. The ultrastructural study of the tumor confirmed the fibroblastic nature of the lesion, which subsequently metastasized to the lung. Histologically, the giant rosettes simulated palisaded granulomas. Our findings warrant classifying the tumor as a sarcoma, and we suggest the designation low-grade fibrosarcoma with palisaded granulomalike bodies (giant rosettes).
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ranking = 42717.211556588
keywords = rosette
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3/45. A rare case of fibrosarcoma of the inferior vena cava expanding into the right atrium.

    The authors report in detail a case of fibrosarcoma of the inferior vena cava. A 53-year-old female presented with echographic and computed tomographic evidence of a solid formation in the porta hepatis that fused with the inferior vena cava. Because of appearance and rapid development of symptoms of right cardiac failure, the patient was transferred to the Clinic of Cardiac Surgery and underwent an emergency operation. A solid formation arising from the inferior vena cava and expanding into the right atrium was found during the operation. The operation performed was not a radical one. fibrosarcoma of the inferior vena cava was proved on histological examination. A single therapeutic course with vincristine and farmorubicin was administered. The patient died from recurrence of the malignancy 26 months after the operation. The epidemiologic, diagnostic, and therapeutic aspects of the case are discussed in relation with the literature data.
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keywords = formation
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4/45. Pseudosarcoma in Paget's disease.

    The authors describe the case of a male patient who had had Paget's disease since 1973 which led to the diffuse swelling of the proximal femur in 1993. The elevated alkaline phosphatase level and the destructive lytic lesions on the X-ray of the femur raised the possibility of malignancy. The bone scan showed increased accumulation in the proximal part of the left femur. On the MR the low T1 and the elevated T2 signal intensity suggested malignity despite that in some regions signals typical of adipose tissue could also be seen, which is usually interpreted as a sign of benignity. In the histological specimens no signs of malignant tumour cells were identified. The patient received bisphosphonate treatment which decreased his pain and swelling and also the alkaline phosphatase level. The patient died because of a rapidly progressing retroperitoneal fibrosarcoma and lung metastasis in 1996. The autopsy did not prove any malignant transformation of Paget's disease of bone in the proximal femur.
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5/45. Low grade fibromyxoid sarcoma. a further low-grade soft tissue malignancy characterized by a ring chromosome.

    Supernumerary rings in the context of a simple karyotype characterize several low-grade malignant tumors of soft tissue and bone. Low-grade fibromyxoid sarcoma is an uncommon low-grade sarcoma, the cytogenetics of which has not yet been reported. Here we describe the first molecular-cytogenetic characterization of a pulmonary metastasis of low-grade fibromyxoid sarcoma. The histology of the primary and recurrent tumors was consistent with the diagnosis of low-grade fibromyxoid sarcoma of the usual type, whereas the pulmonary metastasis was of the "giant rosettes" variant. cytogenetic analysis revealed a ring chromosome. Because gain of material of chromosomes 7 and 16 was detected by CGH, the ring chromosome is assumed to be composed of material from these respective chromosomes.
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ranking = 6102.4587937982
keywords = rosette
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6/45. Myxofibrosarcoma with an infiltrative growth pattern: a case report.

    Myxofibrosarcoma, also known as a myxoid variant of malignant fibrous histiocytoma, is one of the most common sarcomas in the extremities of elderly people and is characterized by a high frequency of local recurrence. We report a case of myxofibrosarcoma, intermediate grade, involving the thigh along the fascial plane and between the muscles without the formation of an apparent nodular lesion. On microscopic examination, the tumor lacked areas of necrosis and pronounced cellular pleomorphism, but it was highly cellular with proliferation of spindle cells which contained large elongated, hyperchromatic and irregularly shaped nuclei, slightly eosinophilic cytoplasm and indistinct cell margins, arranged in both interlacing fascicles and a storiform pattern. Immunohistochemically, many of the tumor cells showed intense reactivity to vimentin and CD34. More than 20% of the cells were positive for p53 protein and the MIB-1 labeling index was approximately 30%. desmin, alpha-smooth muscle actin, muscle-specific actin, S-100 protein, cytokeratin, epithelial membrane antigen, bcl-2 protein and neurofilament were negative. The absence of a discrete mass lesion and diffuse infiltrative nature precluded early recognition of tumor. Seven years after hindquarter amputation, the patient has been alive without evidence of local recurrence or distant metastasis. This case indicates that myxofibrosarcoma can demonstrate a highly infiltrative growth pattern. It is possible that this infiltrative nature is associated with a high rate of local recurrence of the tumor. A careful radiological examination of the extension of the tumor prior to surgery is mandatory considering the infiltrative nature of myxofibrosarcoma.
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7/45. Hyalinising spindle cell tumour with giant rosettes: report of a case with unusual features including original histological and ultrastructural observations.

    Hyalinising spindle cell tumour with giant rosettes (HSCTGR) is an uncommon, recently described low-grade sarcoma which shares many histological features with low-grade fibromyxoid sarcoma (LGFMS). We report a case of HSCTGR occurring in the deep soft tissues of the thigh of a 46-year-old woman, that presented as a slowly growing, painless mass. Microscopically the tumour was composed of spindled stromal cells amongst which were scattered so-called collagen rosettes. The distinctive feature of this case was the previously unreported finding of lymphoid cells of T-cell phenotype admixed with fibrohistioctyic cells in the cellular cuff surrounding the collagenous core of the rosettes. The case was further unusual in that it included focal areas of increased cellularity with a mitotic count of up to three per 10 high-power fields. While the latter feature has been associated with increased recurrences and metastases in LGFMS, it is not known whether the significance is similar in HSCTGR. The spindled stromal cells showed ultrastructural features of poorly differentiated fibroblasts, while those at the edges of the rosettes showed features of altered fibroblasts, some with a fibrohistiocytic appearance. These findings support the interpretation that HSCTGR forms part of the spectrum of sarcomas showing fibroblastic differentiation.
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ranking = 48819.670350386
keywords = rosette
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8/45. fibrosarcoma in bizarre parosteal osteochondromatous proliferation.

    Bizarre parosteal osteochondromatous proliferation (BPOP) is a rare benign lesion predominantly involving the small bones of the hands and feet. Malignant transformation in BPOP has not been documented in the English literature. This report presents the coexistence of fibrosarcoma with BPOP in the right distal fibula of an 18-year-old woman.
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9/45. Hyalinizing spindle cell tumor with giant rosettes: report of a case showing remarkable myofibroblastic differentiation.

    We examined the proliferative activity and the differentiation line of tumor cells in a case of "hyalinizing spindle cell tumor with giant rosettes" (HSCGR). A 6 cm tumor within the right deltoid muscle of a 58-year-old female was found by physical and radiographical examinations. A biopsy revealed the histological features of a spindle cell tumor with rosette-like structures. Wide excision was done under the diagnosis of HSCGR. The tumor presented as a gray-whitish, solid mass with focal pseudocystic degeneration. Immunohistochemically, the tumor cells were diffusely positive for vimentin and were also focally positive for S-100, but negative for desmin and alpha-smooth muscle actin. The cells stained positively for Ki-67 with even distribution, there being a correlation with the cellularity of the areas, with a labeling index ranging from 0.3 to 0.5%. In addition, flow cytometry revealed an almost normal diploid dna pattern and 5.8% S-phase fraction, indicating low proliferative activity. Ultrastructurally, many tumor cells displayed discontinuous basal lamina, pinocytotic vesicles, dilated rough endoplasmic reticulum, and microfilaments with focal dense bodies. The main component of the rosette was collagenous fibrils with normal diameter and normal periodic banding. We interpreted this case of HSCGR as a low grade fibrosarcoma with remarkable differentiation of myofibroblastic lineage, and with focally accumulated, morphologically normal collagenous fibrils.
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ranking = 42717.211556588
keywords = rosette
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10/45. Anal fibrosarcoma: report of a case and review of literature.

    A unique case of anal fibrosarcoma is reported. review of the literature in the past half century revealed only 13 cases of rectal fibrosarcoma, and no case of anal fibrosarcoma. Abdominoperineal resection is the usual treatment if the disease is confined to the rectum or anus. An extended resection may be indicated in those patients who have more extensive disease. Long-term follow-up information was not available in the literature. The prognosis is presumed to be the same as if not worse than for fibrosarcomas in other parts of the body. Reporting of this rare tumor when found in unusual locations is encouraged.
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