1/399. Synovial sarcoma of the esophagus simulating achalasia. Synovial sarcoma is a rare malignancy occurring mainly in the extremities. Only seven cases have been described arising in the esophagus. All of them presented as a polypoid mass involving the upper third of the esophagus. A case of infiltrating synovial esophageal sarcoma simulating achalasia in a 63-year-old woman is reported. According to the literature, the location and the clinical pattern of this tumor are exceptional. The clinical features, pathologic findings, differential diagnosis, and management of this condition are discussed. ( info) |
2/399. Synovial sarcoma in the parapharyngeal space: case report and review of the literature. We encountered a rare case of synovial sarcoma in the parapharynx of a 47-year-old Japanese man. This patient presented with an enlarging tumor in the right side of his neck that had grown progressively over a 4-week period. Radiological examinations revealed that the tumor arose from the parapharyngeal space. The tumor could not be completely removed at surgery. Metastasis to the lumbar vertebra was detected postoperatively. The patient underwent three courses of chemotherapy and the delivery of palliative radiation to the lumbar vertebra without success. The patient died of lung metastasis 7 months after surgery. ( info) |
3/399. Primary pericardial synovial sarcoma: a case report and literature review. Primary synovial sarcoma of the heart is a rare tumor, with only six previous cases having been reported in the literature. Treatment has included surgery with or without chemotherapy. We present the first case of a documented synovial sarcoma arising from the pericardium in a 19-year-old man. Molecular analysis for t(X; 18) SYT-SSX gene fusion was positive. radiation treatment was given postoperatively to the entire heart with a boost to the area where the margins were positive. ( info) |
4/399. Synovial presentation of non-Hodgkin's lymphoma. The MRI appearances of synovial involvement from non-Hodgkin's lymphoma are described in a 61-year-old man. These appearances have not previously been described and the differential diagnosis is discussed. ( info) |
5/399. Primary synovial sarcoma of the lung: report of two cases confirmed by molecular detection of SYT-SSX fusion gene transcripts. AIMS: Primary pulmonary sarcoma is rare, and frequently creates diagnostic challenges. We describe two cases of primary pulmonary spindle cell sarcoma in which a molecular approach using archival paraffin-embedded tissue was proved to aid diagnosis. methods AND RESULTS: Both patients had huge masses replacing the upper and middle lobes of the lung, respectively, without any primary extrapulmonary neoplastic lesions. Microscopically, the lesions showed a solid hypercellular nodular or lobular growth of atypical short spindle cells in variably intersecting fascicles or in a haphazard fashion, together with focal areas displaying a prominent haemangiopericytoma-like pattern. Immunohistochemically, a small number of the tumour cells were positive for epithelial markers such as cytokeratin and epithelial membrane antigen. In both cases, a reverse transcription-polymerase chain reaction using rna extracted from formalin-fixed, paraffin-embedded tissues detected SYT-SSX fusion gene transcripts, which are characteristic of synovial sarcoma. CONCLUSION: On the basis of the morphological and molecular findings, these tumours are considered to be rare examples of monophasic synovial sarcoma of the lung. Our molecular assay detecting the SYT-SSX fusion transcripts is useful for the final diagnosis of synovial sarcoma arising at such an unusual anatomical site. ( info) |
6/399. Detection of a variant SYT-SSX1 fusion in a case of predominantly epithelioid synovial sarcoma. BACKGROUND: The translocation t(X;18)(p11.2;q11.2) characterizes synovial sarcoma, fusing the SYT gene at 18q11.2 to either SSX1 or SSX2 at Xp11.2. The usual chimeric product fuses SYT codon 379 to SSX1 or SSX2 codon 111. To date only three variant fusions have been identified. A predominantly epithelioid synovial sarcoma that expressed a novel variant of the SYT-SSX1 fusion is described. methods AND RESULTS: The current case was tested for the SYT-SSX fusion by reverse transcriptase polymerase chain reaction (PCR) followed by sequencing of the PCR product. Analysis revealed a 673 bp SYT-SSX1 chimeric product characterized by a novel junction of SYT codon 379 to SSX1 codon 83 with a 6 bp insertion at the fusion junction. CONCLUSION: As the number of reported variations of the SYT-SSX chimeric fusion increases in synovial sarcoma, the mechanics of the translocation machinery and the functional significance of these chimeric fusions will be better understood. ( info) |
7/399. A new human synovial sarcoma cell line, HS-SY-3, with a truncated form of hybrid SYT/SSX1 gene. Recent cytogenetical and molecular studies have indicated that synovial sarcoma harbors a t(X;18)(p11.2;q11.2) translocation, resulting in the formation of a hybrid SYT/SSX (SSX1 or SSX2) gene. We newly established a human cell line, HS-SY-3, from a synovial sarcoma. HS-SY-3 cells were shown to harbor the pathognomonic t(X;18)(p11.2;q11.2) translocation by chromosome analysis but not to exhibit the classical hybrid SYT/SSX transcripts induced by this translocation, using RT-PCR. To determine the reason for this discrepancy, we analyzed cDNA from HS-SY-3 cells, as well as the original sarcoma tissue by the rapid amplification of cDNA 3' end assay, and found that the chimaeric cDNA was 240 bp shorter than the previously established SYT/SSX1 cDNA due to truncation of the 3' side of SSX1. The HS-SY-3 cells should be useful for future functional studies of the SYT/SSX chimeric gene. ( info) |
| Intraneural synovial sarcomas are extremely rare in the main nerve trunks of the upper limb. We report on a 16-year-old youth who presented with a painless mass on the flexor aspect of the wrist with the clinical appearance of a ganglion. At operation there was a tumour of the median nerve that was shown on histology to be an intraneural synovial sarcoma. ( info) |
9/399. 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. ( info) |
10/399. Uptake of radiolabeled somatostatin analog is detectable in patients with metastatic foci of sarcoma. BACKGROUND: somatostatin receptors are present on many types of epithelial tumors, and ligands targeting these receptors are used to treat patients with neuroendocrine malignancies. Preclinical studies have demonstrated the presence of somatostatin receptors on a variety of mesenchymal tumors by in vitro receptor autoradiography. The use of radiolabeled somatostatin analogs to assess the presence of somatostatin receptors in vivo has been established, but use of this technique to evaluate human sarcomas has not been reported previously. methods: Seventeen patients (13 females and 4 males) with metastatic sarcoma underwent imaging via somatostatin-receptor scintigraphy. Scans were performed using indium -111 pentetreotide. Planar studies and single photon emission computed tomography imaging were performed at 4 and 24 hours, and results of scintigraphy were correlated with computed tomography findings. RESULTS: Twelve of 17 scans showed increased uptake in regions of known metastatic disease. There was no apparent correlation with scan positivity and patient age, histology, site of disease, or duration of diagnosis. CONCLUSIONS: Seventy-one percent of patients with advanced soft-tissue sarcomas had positive scintigraphy scans demonstrating tumor expression of somatostatin receptors subtype 2 in vivo. Imaging with indium-111 pentetreotide could be studied as an adjunct to conventional imaging modalities for assessment of sarcoma patients. Further research is needed to determine the prognostic implications of somatostatin receptor subtype 2 positivity, including larger studies to evaluate any potential correlation with metastatic behavior and other clinical outcomes. ( info) |