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1/863. liver metastases from extremity soft tissue sarcoma.

    liver involvement by soft tissue sarcoma is an unfavorable prognostic factor for survival. Complete resection of liver metastases can bring improvement in selected patients, but chemotherapy remains the only palliative treatment option for most. Anecdotal long-term survival of patients with unresectable liver metastases treated with systemic chemotherapy has been reported, such as the patient presented here.
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ranking = 1
keywords = sarcoma
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2/863. Acral dermatofibrosarcoma protuberans with metastases.

    dermatofibrosarcoma protuberans (DFSP) is an uncommon cutaneous tumor that occurs rarely on the extremities. This tumor has a particular propensity for local recurrence following seemingly adequate excision. Metastases are rare, and usually follow repeated local recurrences. On rare occasions, fibrosarcomatous change may arise in a DFSP, and appears to affect adversely the prognosis. The adequacy of the initial resection is the most important prognostic factor; however, suggested margins vary between 1.5 and 5 cm. The ideal margin of resection on the foot is unknown. We report a patient with recurrent, acral DFSP with fibrosarcomatous change and pulmonary parenchymal metastases in a 48-year-old black male.
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ranking = 1.4
keywords = sarcoma
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3/863. Mixed-type liposarcoma of the oral cavity: a case with unusual features and a long survival.

    A case of mixed-type liposarcoma, which showed unusual dedifferentiation in the recurrence, is reported. The rapidly growing mass in the palate of a 60-year-old Japanese woman first revealed a combination of myxoid liposarcoma with features resembling storiform malignant fibrous histiocytoma. The recurrent neoplasm, showing an abrupt transition between myxoid and non-lipogenic parts, partially reverted to sclerosing well-differentiated liposarcoma. The patient died 10.1 years after the first operation.
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ranking = 1.4
keywords = sarcoma
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4/863. Extraneural metastasizing ependymoma of the spinal cord.

    This paper reports a case of the rare entity of an extraneural metastasizing ependymoma of the spinal cord. The tumor which arose in the conus medullaris and in the cauda equina was first diagnosed in 1956 when a thoracolumbar myeloresection was performed. At autopsy, 40 years after the primary diagnosis, a massive local tumor recurrence with extraneural metastases in the lungs, the pleura, the liver, and the thoracal and abdominal lymph nodes were found. Immunohistochemical stains of the extraneural metastases showed a strong cytoplasmatic expression of glial fibrillary acidic protein (GFAP). Neither the primary tumor nor its metastases showed any of the conventional morphological criteria of malignancy. Reviewing the literature we discuss the possible mechanism of extraneural tumor spread and the incidence of metastases with regard to the tumor type.
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ranking = 0.0033876867002955
keywords = ewing
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5/863. Peripheral primitive neuroectodermal tumors of bone. A review of three cases.

    The recently individualized and still incompletely understood family of peripheral neuroectodermal tumors encompasses several tumor types, of which some have a predilection for bone. Immunocytochemical studies are essential and usually provide the diagnosis. A t(11;22)(q24;q12) translocation is present in over 80% of cases. Ewing's sarcoma is now viewed as an undifferentiated form of peripheral neuroectodermal tumor, and both tumors require management with combination chemotherapy plus radiation therapy and/or surgery. Contradictory data have been reported regarding the comparative prognosis of peripheral neuroectodermal tumor and Ewing's sarcoma, indicating a need for further studies in large numbers of patients. We illustrate these points by three case-reports, two in girls diagnosed with a vertebral primary at five and nine years of age, respectively, and one in a man diagnosed with a pelvic primary at 29 years of age.
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ranking = 0.4
keywords = sarcoma
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6/863. Myxoid variant of follicular dendritic cell sarcoma arising in the breast.

    Follicular dendritic cell sarcoma is a malignant tumor of the follicular dendritic cell which can arise in extranodal sites. We present here a case arising as a mass in the breast of a 41-year-old woman. The tumor was composed of mildly pleomorphic spindly cells with pale ovoid nuclei and cell processes intimately admixed with mature lymphocytes. In much of the lesion the cells were dispersed in cords in a myxoid stroma, and elsewhere there were solid sheets. The neoplastic cells were immunoreactive for CD21, CD35, EMA, and S100 protein, but not for other lymphoid markers or cytokeratin. Electron microscopy showed interdigitating cytoplasmic processes with junctions but no external lamina. The differential diagnosis includes carcinoma, lymphomas, and a variety of myxoid sarcomas. The tumor recurred within a few months and displayed increased nuclear pleomorphism and lymphatic invasion but the patient appears free of disease 3 years after the further excision. This case extends the spectrum of follicular dendritic cell sarcoma in soft tissue sites.
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ranking = 1.4
keywords = sarcoma
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7/863. Soft tissue masses of the chest wall and axilla: has metastatic melanoma been considered?

    Isolated axillary and chest wall soft tissue masses are an uncommon presentation of metastatic cancer. The authors present three patients in whom malignant melanomas metastatic to these sites had been misdiagnosed, leading to inappropriate oncologic treatment planning in all three cases. The presumed diagnoses, even after fine-needle aspiration or trucut biopsies, were soft-tissue sarcoma (n = 2) and undifferentiated breast cancer (n = 1). The combination of taking a thorough history and performing proper immunohistochemical analysis of the biopsy material would have suggested the presence of malignant melanoma in all cases. As the disease appeared locoregionally limited in all patients, radical surgical resection with extended lymphadenectomy was performed without significant dysfunction of the upper extremity. One patient agreed to postoperative immunotherapy with interferon-alpha. Two patients are currently alive 17 and 14 months after operation. One patient was found to have systemic recurrence at 5 months, one experienced two isolated local recurrences in a prior operative site that were amenable to reresection and presently has no evidence of disease 12 months after resection, and one patient remains free of disease at 14 months. Clinical presentation, suggested diagnostic workup, and therapeutic implications are discussed to avoid misdiagnoses in this setting of possible clinical presentations of metastatic melanoma.
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ranking = 0.2
keywords = sarcoma
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8/863. Primary orbital leiomyoma and leiomyosarcoma.

    A case of an extremely rare primary orbital leiomyoma in a 25-year-old male patient is presented who had a lifelong history of deviation of the left eye globe with slight enophthalmos and reduced motility. Because of pain and increasing deviation of the eye the tumor was totally resected. On histologic examination the tumor showed ossification which is extremely rare so that a calcifying fibroma had to be ruled out. In immunohistochemistry, however, this tumor stained with smooth muscle antigen. Less than 2% of cells stained positive for Ki-S1, a proliferation marker. The second case is a rare primary orbital leiomyosarcoma in an 84-year-old female patient that showed massive growth. After exenteration histologic examination showed a dedifferentiated highly malignant soft tissue tumor which expressed desmin and smooth muscle actin but was negative for myoglobin, S-100 and HMB-45.
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ranking = 1
keywords = sarcoma
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9/863. Molecular assessment of clonality leads to the identification of a new germ line TP53 mutation associated with malignant cystosarcoma phyllodes and soft tissue sarcoma.

    Cystosarcoma phyllodes (CSP) is a rare breast neoplasm composed of stromal and epithelial elements. It usually runs a benign course but it may metastasize. In a 31-year-old patient with recurring CSP, a mesenchymal tumor in the leg developed. The question arose whether the latter tumor could be a metastasis from the CSP, which would have major treatment consequences. The problem was addressed using molecular methods, i.e., comparison of the pattern of polymorphic repeat markers on chromosome 17p as well as single strand conformation polymorphism analysis and sequencing of exons 5 to 8 of the TP53 gene in both tumor and normal tissue. An identical pattern of loss of heterozygosity in both breast tumors was demonstrated, but a different pattern was shown in the tumor in the leg. This led to the conclusion that the latter tumor had to be a new primary tumor. A mutation in codon 162 of the TP53 gene was found in the tumor tissue as well as in the normal tissue of this patient. This germ line mutation leads to the replacement of isoleucine by asparagine and most likely has functional consequences. In all four examined tumors of this patient, the normal TP53 allele was lost. This is strong evidence that this germ line TP53 mutation causes the genesis of these two rare primary mesenchymal tumors in this young patient. The current study exemplifies the power of molecular diagnostic methods in investigating the specific clinical problem of clonal relation between two separate tumors. The germ line mutation found in codon 162 of the TP53 gene and the association with cystosarcoma phyllodes have not been described previously.
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ranking = 2
keywords = sarcoma
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10/863. Infantile fibrosarcoma. Report of two cases.

    We present two cases of infantile fibrosarcoma, one of which was thought to be a congenital tumour on the thigh and was initially diagnosed as a haemangioma, and the other was a tumour on the trunk. Although both recurred locally after initial surgical treatment, wide local re-excision controlled the disease without adjuvant treatment.
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ranking = 1
keywords = sarcoma
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