Cases reported "Sarcoma, Synovial"

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1/87. Primary pericardial synovial sarcoma with detection of the chimeric transcript SYT-SSX.

    We report a case of a 19-year-old woman with a primary pericardial synovial sarcoma that extended from the right ventricular free wall to the posterior aspect of the left anterior thoracic wall. Synovial sarcoma was diagnosed by the detection of the chimeric transcript SYT-SSX using reverse transcriptase-polymerase chain reaction (RT-PCR). This transcript is generated by reciprocal translocation between chromosomes X and 18, and is specific to synovial sarcoma that usually occurs in the extremities of young adults. When pathological and immunohistochemical diagnosis of synovial sarcoma is difficult, the molecular biological technique using RT-PCR becomes a powerful method of confirmation of this neoplasm.
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keywords = neoplasm
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2/87. Tumors and tumorous masses presenting as temporomandibular joint syndrome.

    OBJECTIVE: neoplasms of the temporomandibular joint (TMJ) usually mimic common causes of TMJ syndrome, leading to delay in diagnosis. To increase awareness of TMJ neoplasms and establish guidelines for early intervention, we performed a retrospective analysis of a series of patients with neoplasms of the TMJ. STUDY DESIGN AND SETTING: A retrospective review of the records of patients with neoplasms of the TMJ from 1990 to 1997 was done. RESULTS: Six patients were identified. The neoplasms included benign and malignant neoplasms. The time from initial presentation to final diagnosis was in most cases prolonged, ranging from 3 months to 8 years. patients typically showed advanced lesions radiographically. All patients required surgical extirpation. Postoperative radiation therapy was used for malignant lesions. Patient outcomes were delineated. CONCLUSIONS AND SIGNIFICANCE: neoplasms, both benign and malignant, of the TMJ are rare but represent a challenging diagnostic problem. In patients with 1 month or more of recalcitrant TMJ swelling or pain, radiographic imaging may be considered to rule out these rare neoplasms. This may lead to earlier intervention and improved outcome.
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keywords = neoplasm
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3/87. Cystic synovial sarcoma.

    A case of synovial sarcoma of the paraspinal region showing massive cystic changes is described. The tumor presented in a 53-year-old white woman who noticed a lump on the upper aspect of her back. magnetic resonance imaging of the cervical spine showed a heterogeneous paraspinal mass with well-defined margins and a multilocular quality with foci of hemorrhage. Fine needle aspiration of the mass showed clusters of polygonal cells admixed with a scattered spindle cell component. Surgical excision of the mass showed a well-circumscribed but nonencapsulated tumor that showed multiple small cystic structures on cross-section. Histologic examination showed a biphasic neoplasm characterized by bland-appearing glandular elements embedded in a moderately cellular spindle cell stroma. The tumor contained multiple cysts of varying size. Immunohistochemical studies showed the glandular component to be positive for cytokeratin and epithelial membrane antigen. The spindle cell component was immunoreactive for cytokeratin, vimentin, bcl-2, and CD99. Stains for muscle-specific actin, smooth muscle actin, S-100 protein, and CD34 were negative. cytogenetic analysis showed a balanced reciprocal translocation involving chromosomes X and 18, in addition to other clonal abnormalities. Synovial sarcoma should be considered in the differential diagnosis of cystic lesions involving the soft tissues. magnetic resonance imaging is considered the procedure of choice for the evaluation of soft tissue tumors because of its superior soft tissue contrast and multiplanar imaging capability. While the imaging features of soft tissue tumors are often nonspecific, magnetic resonance imaging may provide helpful clues, thus narrowing the differential diagnosis. Immunohistochemical studies and cytogenetic analysis may be very helpful for establishing the correct diagnosis in cases with this unusual presentation. Ann Diagn Pathol 5:48-56, 2001.
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keywords = neoplasm
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4/87. Metastatic osteosarcoma to the liver after treatment for synovial sarcoma: a case report.

    Metastatic osteosarcoma most commonly affects the lungs and other bones. Hepatic metastasis at the time of diagnosis is extremely rare. A 14-year-old boy with synovial sarcoma of the left popliteal fossa was treated with surgical resection, radiotherapy for microscopic residual disease, and 1 year of chemotherapy (vincristine, cyclophosphamide, dactinomycin, and doxorubicin). Approximately 10 years after the initial diagnosis, a secondary osteosarcoma developed in the left proximal tibia. Computed tomography at presentation showed bilateral pulmonary metastases and large ossified nodules in the liver that demonstrated abnormal avidity on 99mTc MDP bone scan indicating hepatic metastasis. Despite chemotherapy (cisplatin, ifosfamide, high-dose methotrexate, and dacarbazine), the patient died of progressive disease 4 months after the diagnosis of the second cancer. Hepatic metastasis was found at the time of diagnosis of a secondary osteosarcoma and manifested as ossified nodules. The risk of radiation-induced osteosarcoma should always be considered in decisions about treatment for soft-tissue sarcoma.
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ranking = 0.052233548391421
keywords = cancer
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5/87. Poorly differentiated synovial sarcoma: a case report.

    Poorly differentiated synovial sarcoma is a rare soft tissue tumor. We studied a case arising in the pleural cavity of a young subject, characterised by the presence of spindle cell, small cell, and large epithelioid cell areas. We performed stains for mucosubstances and analysed the expression of cytokeratins 5/6, 7, 8, 18, 19, CEA, CD34, Ber-Ep4 and calretinin to characterize the phenotype of this neoplasm. We furthermore assessed immunohistochemically the presence of p53, Bcl-2, Bax and caspase 3, four apoptotic markers, to evaluate a relationship between apoptotic activity and the behaviour of this tumor. Our findings showed a strong presence of calretinin, p53 and Bcl-2 in all three areas. The possibility that poorly differentiated synovial sarcoma could be calretinin-positive was a new data, to our knowledge, and it could be of some importance in diagnostic pathology. Moreover, the negligible positivity for Bax and caspase 3 suggested that the minor role of programmed cell death could be one of the causes of the aggressive behaviour of this tumor. These data also suggest that the reduction of apoptotic phenomena in poorly differentiated synovial sarcoma could be considered one of the major mechanisms of tumoral growth.
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keywords = neoplasm
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6/87. Effect of myocutaneous inflammatory changes caused by intra-arterial chemotherapy on the outcome of patients who undergo limb-saving surgery.

    BACKGROUND: This study examined the effect of myocutaneous inflammatory changes caused by intra-arterial chemotherapy on the outcome of patients who undergo limb-saving surgery. methods: One hundred seven patients with malignant bone and soft tissue tumors were administered intra-arterial cisplatin and caffeine preoperatively with or without doxorubicin. Nine patients (8.4%) who had had myocutaneous inflammatory reactions were reviewed to determine the effects of this complication on limb-saving surgery. RESULTS: The patients complained of unbearable and continuous pain while undergoing intra-arterial infusion. gait disturbances and muscle weakness were caused by their severe regional pain, resulting in the use of crutches even before surgery. Extended areas of necrotic skin and muscle were resected simultaneously with limb-saving surgery on four patients. Myocutaneous necrosis in one patient, indurations in two patients, and flares in two patients were not treated surgically. Pain relief was provided to eight patients at some point. Four patients with extensive myocutaneous necrosis around the knee joint had restricted range of motion and poor functional results. radionuclide angiography using 99m-technetium-macroaggregated albumins was performed to evaluate the blood flow to the affected muscle and tumor. It showed marked increase of the radioisotope perfusion in the affected muscles but little uptake in the tumor. These results may suggest that the affected muscles diminish the effects of anticancer drugs on the tumors. CONCLUSIONS: Myocutaneous inflammatory reactions should be prevented if possible to obtain good limb function and chemotherapeutic effects in patients who undergo intra-arterial chemotherapy. Resection of necrotic tissue is mandatory to relieve pain.
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ranking = 0.052233548391421
keywords = cancer
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7/87. Mediastinal synovial sarcoma: report of two cases with molecular genetic analysis.

    Synovial sarcoma occurs predominantly in the paraarticular regions of the extremities. Synovial sarcoma of the mediastinum is an exceedingly rare neoplasm that has overlapping histologic and immunophenotypic features with other tumors in the differential diagnosis. We describe two cases. One is a 67-year-old patient who presented with chest pain and shortness of breath. diagnostic imaging revealed a mediastinal mass extending over the cardiac apex. Histopathology, immunohistochemistry, and molecular genetic analysis confirmed the diagnosis of synovial sarcoma. The patient underwent surgical resection and postoperative radiation therapy. He is alive and well 18 months after diagnosis. This case illustrates the importance of proper procurement of frozen tissue for molecular genetic analysis for the identification of the t(X;18), characteristic of synovial sarcoma. Detection of this translocation is of paramount importance to confirm this diagnosis, particularly when this neoplasm arises in atypical locations outside the extremities.
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ranking = 2
keywords = neoplasm
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8/87. Intrarenal solitary fibrous tumor of the kidney report of a case with emphasis on the differential diagnosis in the wide spectrum of monomorphous spindle cell tumors of the kidney.

    Solitary fibrous tumor (SFT) is a neoplasm that can occur in the urogenital tract, and is also reported occurring in the spermatic cord, seminal vesicles, urinary bladder, prostate, and kidney. Furthermore, it is most important to consider its existence in the kidney, because it is usually diagnosed as renal cell carcinoma pre-operatively. To our knowledge, only 10 cases of SFT have been reported in the kidney to date. We report the clinico-pathological features of an intrarenal SFT occurring in a 31-year-old woman. The tumor, measuring 8.6 cm in its greatest diameter, completely replaced the cortex and the medulla of the middle region of the right kidney, compressing the pelvis. Radiological imaging was consistent with a renal cell carcinoma. Histologically, the tumor was composed of a proliferation of bland-looking vimentin , CD34 , bcl2 and CD99 spindle cells exhibiting a haphazard to storiform growth pattern, pushing borders, and a low mitotic rate (2 mitoses x 10 HPF). We placed emphasis on the differential diagnostic problems, i.e., its differentiation from other primary monomorphous benign and malignant spindle cell tumors of the kidney, such as fibroma, benign fibrous histiocytoma, hemangiopericytoma, inflammatory myofibroblastic (pseudo-)tumor, leiomyoma, angiomyolipoma with predominant spindle cell smooth muscle component, benign peripheral nerve sheath tumors, renal mixed epithelial/stromal tumors, adult type mesoblastic nephroma, fibrous type monophasic synovial sarcoma, malignant peripheral nerve sheath tumors, fibrosarcoma, and low-grade fibromyxoid sarcoma.
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ranking = 1
keywords = neoplasm
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9/87. Monophasic synovial sarcoma of the neck in an 8-year-old girl resembling a thyroglossal duct cyst.

    Synovial sarcomas of the head and neck are rare. Typically, they are localized laterally in the parapharyngeal space. We report the case of an 8-year-old girl with a monophasic round cell synovial sarcoma of the anterior neck, clinically resembling a thyroglossal duct cyst. Histologic, immunohistochemic and cytogenetic findings are presented with a brief review of the literature. This case reaffirms the importance of considering malignant neoplasms in the differential diagnosis of pediatric neck masses.
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keywords = neoplasm
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10/87. immunotherapy with autologous dendritic cells and tumor-specific synthetic peptides for synovial sarcoma.

    Synovial sarcoma in an 11-year-old Japanese girl relapsed 5 months after autologous stem cell transplantation. Autologous dendritic cells (DCs) were generated from her peripheral blood mononuclear cells using granulocyte/macrophage colony-stimulating factor and IL-4. dendritic cells were pulsed with synthetic peptides containing a junctional region of SYT-SSX2 fusion protein generated by t(X;18) and were administered once per week. No side effects were observed. growth of metastatic nodules in the lung was temporally suppressed. The delayed-type hypersensitivity responses in skin were enhanced to tumor lysate but not to peripheral blood mononuclear cell lysate. The CD3 cells cultured with pulsed DCs lysed tumor cells in vitro. immunotherapy using DCs and tumor-specific peptides may be a safe approach in the treatment of childhood cancer.
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ranking = 0.052233548391421
keywords = cancer
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