Cases reported "Sarcoma"

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1/139. MR imaging of complex tail-gut cysts.

    Retrorectal-cyst hamartomas (RCH) are rare developmental tail-gut cystic tumours of the retrorectal space, which occasionally undergo malignant transformation. We describe the magnetic resonance imaging (MRI) findings in two patients with RCH and in a third patient with unclassified sarcoma arising from a RCH. The RCH were hypointense or hyperintense on T1-weighted images and hyperintense on T2-weighted images; they did not enhance and they contained multiple septations. A solid component in the periphery of one cyst was markedly hypointense on T2-weighted images in keeping with fibrous material. The sarcoma arising from the wall of the RCH enhanced and was of intermediate signal intensity on all sequences. MR may help establish the diagnosis of RCH if an unenhanced cystic tumour is discovered in the retrorectal space and it can help detect those rare cases of malignant transformation of these developmental tumours.
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2/139. Histochemical investigation into the molecular mechanisms of malignant transformation in a benign glomus tumour.

    A glomangiosarcoma arose in a benign glomus tumour. The histological and immunohistochemical characteristics of the tumour were investigated. Apoptotic cells were identified by terminal deoxynucleotidyl transferase (TdT) mediated dUTP-biotin nick end labelling (TUNEL). The proportion of apoptotic cells was found to be low and TUNEL positive nuclei were present in the benign part of the tumour. Bcl-2 protein, an inhibitor of apoptosis, was strongly expressed in the glomangiosarcoma with only weak staining in the benign area. The proliferation index of the glomangiosarcoma was almost 10-fold higher than that of the benign glomus tumour. Numerous nuclei in the glomangiosarcoma were intensely stained for the tumour suppressor protein p53. The results of the this study may contribute to an understanding of the molecular basis of malignant transformation in benign glomus tumours.
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3/139. 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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4/139. Dendritic cell sarcoma of the thyroid.

    BACKGROUND: Follicular dendritic cell sarcoma (FDCS) arises from nonlymphatic antigen-presenting cells found in lymph node B-cell follicles. This extremely rare tumor, which usually arises in lymph nodes, does occur in extranodal head and neck sites such as the tonsil and soft palate. methods: A retrospective review of the patient followed at the University of Pittsburgh Medical Center from 1993 to the present was performed. CONCLUSIONS: This is the first reported case of an FDCS of the thyroid. A review of the literature provides useful information to aid in detection, treatment, and outcome of this unusual soft tissue malignancy.
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5/139. Tc-99m DTPA used as reference imaging to evaluate the distribution of other tumor-seeking tracers in tumors associated with neurofibromatosis.

    Two patients with multiple benign and malignant tumors associated with neurofibromatosis underwent radionuclide imaging with Tc-99m DTPA, Tl-201, and Ga-67. In these patients, Tc-99m DTPA accumulated intensively in both the benign and malignant tumors and localized and defined the extent of every tumor. In contrast, Ga-67 and Tl-201 uptake was seen only in focal areas of tumor where there was malignant transformation or at sites that showed progressive tumor growth. Tc-99m DTPA imaging accurately demonstrated areas of neoplastic involvement and identified the areas that would be seen with the other two tracers in individual tumors. Tc-99m DTPA may not always be used for the differential diagnosis of malignant and benign tumors of neurofibromatosis, but it can provide a reference pattern for imaging to evaluate accurately the distribution of Tl-201 and Ga-67 by mapping out the anatomic extent of these tumors.
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6/139. Popliteal vascular malformation simulating a soft tissue sarcoma.

    Differentiation of vascular abnormalities from soft tissue sarcomas may be difficult on clinical grounds, but is usually possible on imaging criteria. We report the MRI and digital subtraction angiography (DSA) findings in a patient presenting with a mass behind the knee. We discuss differentiating features and review the literature of similar cases.
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7/139. Soft tissue sarcomas after radiation treatment for breast cancer. Three case studies and review of literature.

    AIMS: By means of 3 cases with infield soft tissue carcinomas after radiotherapy for breast cancer, symptoms and therapy are described. Consequences for treatment planning and patient's information before radiotherapy for breast cancer are discussed. patients: Three of 1,025 patients with breast cancer irradiated from 1984 to 1997 suffered from infield secondary soft tissue sarcomas. The latency periods were 61, 49 and 59 months. Two patients had been treated with breast-conserving therapy (computerized planning, 50 Gy to reference point, 5 times 2 Gy/week, 5-MV photons), 1 patient received a local boost dose of 15 Gy (10-MeV electrons), patient 3 radiotherapy of the thoracic wall and regional lymph nodes after mastectomy using 12-MeV electrons (thoracic wall) and 5-MV photons (lymph node areas) to 50 Gy, 5 times 2 Gy/week. No adjuvant chemotherapy was given. All sarcomas were very extensive, all patients died from local progression and/or distant failure after 17, 13 and 12 months. RESULTS: The incidence of spontaneous sarcomas of the breast is about 0.06%, after operation and radiotherapy 0.09 to 0.45%. No correlations to radiotherapy technique and no risk factors were found. radiation dose could play a role, but there are very sparse data about this. CONCLUSIONS: Secondary soft tissue sarcomas are very rare, but familiar complications of radiotherapy. Only early diagnosis leads to a chance for cure. Because of unclear correlations to the treatment parameters and rareness of this event, in our opinion no regular information to the patient receiving radiotherapy for breast cancer is mandatory.
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8/139. Sarcomatous transformation of a prolactinoma associated with development of a fatal internal carotid artery pseudoaneurysm--case report.

    A 21-year-old female with a 5-year history of prolactinoma was referred to our hospital because of cerebrospinal fluid leakage and meningitis immediately following a third transsphenoidal operation for a rapidly growing tumor. Histological examination of the tumor removed at the second transsphenoidal operation found atypical epithelial and sarcomatous components. Administration of bromocriptine, radiotherapy, and chemotherapy were initiated. Emergent craniotomy was required for rapid regrowth of the tumor. Histological examination found predominant sarcomatous components. Tumor growth was difficult to control. The patient died of subarachnoid hemorrhage due to rupture of a pseudoaneurysm involving the C1 portion of the right internal carotid artery. Pituitary adenoma rarely shows malignant transformation. In this case, prolactinoma underwent malignant change to sarcoma. Development of the pseudoaneurysm may have resulted from surgical manipulation, radiotherapy, or tumor invasion.
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9/139. Diagnostic gold standard for soft tissue tumours: morphology or molecular genetics?

    The recognition of recurrent genetic alterations in specific tumour types has provided the basis for the reclassification of certain soft tissue neoplasms, and molecular analysis of patient material has the potential to provide both diagnostic and prognostic information. In this review, we evaluate the role of molecular genetic testing as the prospective 'gold standard' for sarcoma diagnosis. Molecular genetic testing, as with every new method, promises to improve accuracy and to be more sensitive and less subjective, claims that have been made previously by histochemistry, electron microscopy and immunohistochemistry. Technical limitations in molecular assays, as well as more general specificity issues, decrease the clinical usefulness of molecular pathological testing significantly and suggest that, at present, molecular evaluation is best considered an ancillary technique that neither supersedes other ancillary techniques nor eclipses traditional pathological examination.
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10/139. Malignant eccrine spiradenoma: a case report and review of the literature.

    BACKGROUND: Eccrine spiradenoma is a well-differentiated benign tumor of the sweat glands. Malignant change arising within eccrine spiradenoma is rare. OBJECTIVE: We describe a patient with malignant eccrine spiradenoma exhibiting both carcinomatous and sarcomatous differentiation. methods: Case report and literature review. RESULTS: A 37-year-old woman noted enlargement of a left axillary tumor that had been present for 20 years. The tumor was resected and the specimen, measuring 3.0 cm x 1.5 cm, revealed an encapsulated benign eccrine spiradenoma as well as an undifferentiated carcinoma possessing both carcinomatous and sarcomatous components. A transition zone was evident between the benign eccrine spiradenoma and the undifferentiated carcinoma, suggesting that the latter had arisen from the benign tumor. The malignant areas consisted principally of undifferentiated carcinoma (70%), although squamous cell carcinoma (10%), adenocarcinoma (10%), and chondrosarcomatous (10%) components were also present. Numerous mitotic figures were noted within the areas of malignant change, suggesting that the tumor was aggressive in nature. The patient died of systemic metastases 7 months after diagnosis. CONCLUSION: Although eccrine spiradenomas are usually benign, they can, on rare occasions, undergo malignant transformation. This case report describes one such occurrence of malignant transformation of a benign eccrine spiradenoma that unfortunately resulted in the patient's death from systemic metastases 7 months after diagnosis.
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