1/144. fibrosarcoma of the trachea in a child: case report and review of the literature.We present a case of nearly total obstruction of the trachea in a 15-month-old boy suffering from tracheal fibrosarcoma. The diagnosis was confirmed on histological examination of a removed part of the tumor, with subsequent successful complete resection of tracheal fibrosarcoma. Follow-up to the age of 13 years showed complete recovery with normalization of respiratory functions. The diagnostic and therapeutic aspects of this case are discussed.- - - - - - - - - - ranking = 1keywords = ring (Clic here for more details about this article) |
2/144. 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.- - - - - - - - - - ranking = 1keywords = ring (Clic here for more details about this article) |
3/144. Alterations of the 11p15 imprinted region and the IGFs system in a case of recurrent non-islet-cell tumour hypoglycaemia (NICTH).BACKGROUND: AND OBJECTIVE: Non-islet-cell tumour hypoglycaemia (NICTH) is a rare disorder and has been explained by oversecretion of non mature IGF-II and dysregulation of the IGFs sytem. The mechanisms responsible for tumoural IGF-II overexpression in NICTH have been rarely studied. We report an extensive study of IGF-II and IGFBPs as well as chromosome 11p15 gene expression regulation in a case of a pleural fibrosarcoma in a 63-year-old patient presenting with NICTH. methods AND RESULTS: Abnormal high molecular weight precusor forms of IGF-II were present in the patient's serum and were associated with dramatic alterations in the circulating levels of IGF-I, IGF-II and their binding proteins, as well as an inhibition of the somatotroph axis. These alterations returned to normal following complete surgical removal of the tumour. No structural change in chromosome 11p15 region was apparent in the tumour. However, dysregulation of this imprinted region was demonstrated, leading to the loss of imprinting of the IGF-II gene associated with high IGF-II expression, and by contrast decreased level of expression of H19 and p57KIP2 genes. recurrence of the tumour four years latter was not associated with hypoglycaemia or changes in the levels of circulating IGFs or IGFBPs, despite IGF-II overexpression by the tumour. This suggests that a large tumour volume is required to reach high enough levels to cause changes in the levels of circulating IGFs and IGFBPs, and to cause hypoglycaemia. CONCLUSION: These results suggest that a dysregulation of gene expression and imprinting of chromosome 11p15 region is associated with tumour growth and IGF-II overexpression in non-islet-cell tumour hypoglycaemia.- - - - - - - - - - ranking = 33.704912514387keywords = chromosome (Clic here for more details about this article) |
4/144. Fibrous tumors in children - a morphologic and interphase cytogenetic analysis of problematic cases.We describe and discuss the findings by fluorescent in situ hybridization (FISH) for detection of non-random chromosomal gains, in a group of unusual fibrous lesions in children. Nuclear disaggregation was used to prepare slides from eight cases which were hybridized using alpha-satellite enumeration probes for chromosomes 8, 11 and 17. trisomy 8 and 11 were detected in a high percentage of nuclei in cases of congenital/infantile fibrosarcomas (ranging from 45 to 80%), and in a low grade fibrosarcoma in an older child (23%). Only gains of chromosome 17 were detected in a case of infantile fibromatosis (22%). In this study we have found that given the unconventional histopathologic features, the detection of more than one non-random chromosomal gains by FISH, may aid in further defining fibrous tumors in children, and may be useful as an ancillary diagnostic test in the future.- - - - - - - - - - ranking = 22.469941676258keywords = chromosome (Clic here for more details about this article) |
5/144. 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.- - - - - - - - - - ranking = 654.30865318106keywords = ring chromosome, chromosome, ring (Clic here for more details about this article) |
6/144. Intracranial fibrosarcoma arising 5 years after chemotherapy alone for glioblastoma multiforme in a child.We present a child diagnosed with glioblastoma multiforme during infancy and successfully treated with the 'eight-in-one' chemotherapy regimen, who developed an intracranial fibrosarcoma 5 years later. After resection of the fibrosarcoma, she received cranial radiation therapy and high dose chemotherapy with bone marrow transplant. She remains alive and recurrence-free 7 years following the diagnosis of her second intracranial malignancy.- - - - - - - - - - ranking = 1keywords = ring (Clic here for more details about this article) |
7/144. A novel reciprocal (10;17)(p11.2;q23) in myxoid fibrosarcoma.Recurring reciprocal translocations have been identified as the primary chromosome aberrations in a number of neoplasms. These aberrations are often closely associated with particular morphologic or phenotypic subtypes of tumors and in some cases have prognostic implications. We have identified a novel reciprocal t(10;17)(p11.2;q23) in a case of low-grade myxoid fibrosarcoma, which may prove to be a new tumor specific chromosome aberration.- - - - - - - - - - ranking = 23.469941676258keywords = chromosome, ring (Clic here for more details about this article) |
8/144. 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.- - - - - - - - - - ranking = 1keywords = ring (Clic here for more details about this article) |
9/144. 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.- - - - - - - - - - ranking = 1keywords = ring (Clic here for more details about this article) |
10/144. Acral myxoinflammatory fibroblastic sarcoma with unique clonal chromosomal changes.Acral myxoinflammatory fibroblastic sarcoma is a rare tumor of the distal extremities. We present the hitherto unreported karyotypic abnormalities of this new entity. The tumor presented as a mass in the dorsum of the foot in a 53-year-old woman and showed the typical virocyte-like and lipoblast-like cells in a myxoid and inflammatory background. cytogenetic analysis revealed a complex karyotype with a reciprocal translocation t(1;10) (p22;q24) in addition to the loss of chromosomes 3 and 13. fluorescence in situ hybridization with the 769E11YAC and BAC 31L5 and 2H23 probes showed the breakpoint to be located proximally to BCL10 and distally to GOT1 genes on chromosomes 1p22 and 10q24, respectively. The presence of these clonal chromosomal changes supports the neoplastic nature of acral myxoinflammatory fibroblastic sarcoma and underscores that it represents a separate entity.- - - - - - - - - - ranking = 22.469941676258keywords = chromosome (Clic here for more details about this article) |
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