Cases reported "Soft Tissue Neoplasms"

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1/1030. 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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2/1030. Translocation (11;22)(q24;q12) in a small cell tumor of the thigh in a 2-year-old boy: immunohistology, cytogenetics, molecular genetics, and review of the literature.

    A case of a 2-year-old boy with a palpable mass in the left thigh is presented. Incisional biopsy was performed and subsequent histopathological examination revealed an infiltrative tumor composed of relatively large cells. The tumor cells were immunoreactive for vimentin and keratin, but not for desmin or smooth muscle actin. cytogenetic analysis showed a 46,XY,t(11;22)(q24;q12) karyotype. The translocation (11;22)(q24;q12) is said to be characteristic for the family of Ewing's sarcoma and related tumors. As a result of the t(11;22)(q24;q12) the EWS gene on chromosome 22q12 joins the 3' part of FLI-1 gene on chromosome 11q24, which encodes a member of the ets family of transcriptional regulators. Using reverse transcription polymerase chain reaction (RT-PCR) a corresponding EWS-FLI-1 fusion product was detected. Additional immunohistological staining for p30/p32MIC2, which is suggestive, but not specific for Ewing's sarcoma, appeared to be weakly positive. In the current case a diagnosis of Ewing's sarcoma was considered unlikely, because of the location of the tumor and the immunohistological profile. Nevertheless it was decided to treat the patient according to a Ewing's sarcoma protocol based on the genotype of the tumor. The findings were compared with other extraosseous pediatric small cell tumors showing the t(11;22)(q24;q12) described in the literature.
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ranking = 0.8
keywords = sarcoma
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3/1030. lymphoma presenting as a soft tissue mass. A soft tissue sarcoma simulator.

    lymphoma presenting as a soft tissue mass is rare and thus may be confused with the more common soft tissue sarcoma. No previous analysis of the clinical and radiologic features of lymphomas presenting as soft tissue masses is available because most of the cases reviewed are from the pathology literature. Four patients with diagnoses of extranodal lymphomas of the soft tissues were reviewed retrospectively with respect to their clinical features, primary tumor characteristics, stage, radiographic characteristics, treatment, and followup. Mean age was 72.5 years (range, 52-85 years). The soft tissue mass occurred in the thigh (three cases) and shoulder (one case). The median size of the soft tissue mass was 6.7 cm (range, 2-15 cm) in the largest dimension, as measured on magnetic resonance imaging. These patients each had evidence of lymphadenopathy at the time of diagnosis. Lactate dehydrogenase was increased significantly in two cases and increased slightly in two other cases. One case was Stage II(E) at presentation, one was Stage III(E), and two were Stage IV. All were B cell immunophenotype. All patients died between 2 and 24 months after diagnosis, despite the use of Cytoxan, vincristine, adriamycin, and prednisone chemotherapy in each case. Clinical and radiographic features that favor extranodal soft tissue lymphoma over sarcoma include pain and tenderness, lymphadenopathy (particularly when confluent radiologically), ipsilateral extremity swelling, and elevated lactate dehydrogenase.
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ranking = 1.2
keywords = sarcoma
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4/1030. 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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5/1030. 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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6/1030. 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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7/1030. Papillary clear cell adenocarcinoma of the groin arising from endometriosis.

    A 67-year-old woman presented with rapidly enlarging right anterior thigh mass. Clinical impression was of a primary sarcoma, but the histologic analysis of an incisional biopsy specimen showed adenocarcinoma with clear cell differentiation. Initially thought to be a metastasis, the malignant tumor appears to have arisen from extraabdominal endometriosis of the right groin. This case shows clinical, radiographic, and histologic findings that may help the orthopaedic surgeon recognize an unusual complication of extrapelvic endometriosis.
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ranking = 0.2
keywords = sarcoma
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8/1030. Ewing's sarcoma presenting as a posterior mediastinal mass: a lesson learned.

    Thoracic vertebral body hemicorpectomy and chest wall resection was performed in a 17-year-old male patient with a posterior mediastinal tumor thought to be neurogenic in origin. No preoperative tissue diagnostic endeavor was made. Final pathologic diagnosis showed this tumor to be Ewing's sarcoma. This communication alerts the thoracic surgeon to the need for definitive diagnosis of posterior mediastinal masses with vertebral body involvement, particularly in children. induction chemotherapy is the accepted standard of management of these sarcomas.
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ranking = 1.2
keywords = sarcoma
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9/1030. Low grade fibromyxoid sarcoma: fine-needle aspiration cytology with histologic, cytogenetic, immunohistochemical, and ultrastructural correlation.

    BACKGROUND: Although the histologic features of the recently described low grade fibromyxoid sarcoma are well established, to the authors' knowledge there are no reports in the literature describing the cytologic features of this tumor by fine-needle aspiration. Recognition of this lesion is important because of its indolent but metastasizing nature. methods: The authors retrospectively reviewed their surgical pathology files for cases of low grade fibromyxoid sarcoma with a preoperative fine-needle aspiration biopsy (FNAB); three such cases were found. Immunohistochemical studies were performed in all three tumors, ultrastructural examination was performed in two tumors, and fresh tissue for cytogenetic analysis was obtained in one tumor. RESULTS: All FNABs showed similar features. The aspirates were relatively hypocellular with an abundant myxoid background; the neoplastic cells contained oval to spindle shaped nuclei with minimal pleomorphism. No capillaries or areas of fibrous tissue were identified. Cytogenetic study of one case revealed no chromosomal abnormalities. The histologic findings were characteristic for this lesion. By immunohistochemistry the tumor cells showed diffuse and strong reactivity for vimentin only; at the ultrastructural level the neoplastic spindle cells had characteristics of fibroblasts. CONCLUSIONS: The cytologic features of low grade fibromyxoid sarcoma are not specific enough for a definitive diagnosis based on FNAB alone; however, correlating the cytologic and clinical findings can narrow the range of diagnosis. The differential diagnosis includes other myxoid lesions, in particular superficial or intramuscular myxoma and myxofibrosarcoma. In addition, the immunohistochemical and ultrastructural findings support a fibroblastic origin for this neoplasm.
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ranking = 1.6
keywords = sarcoma
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10/1030. Ewing's sarcoma of the soft tissues? Case report.

    A case is reported of a malignant tumour of the soft tissues of the leg, with histological and pathological features comparable with those of Ewing's sarcoma of bone. This extension of the term Ewing's sarcoma to the soft tissues is proposed and the differential diagnosis is discussed.
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ranking = 1.2
keywords = sarcoma
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