1/169. 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.- - - - - - - - - - ranking = 1keywords = spindle cell, spindle, cell (Clic here for more details about this article) |
2/169. 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.- - - - - - - - - - ranking = 0.028814686911295keywords = cell (Clic here for more details about this article) |
3/169. Synovial sarcoma, histologically mimicking primitive neuroectodermal tumor/Ewing's sarcoma at distant sites.We report a case of synovial sarcoma (SS) showing unusual histology at distant sites. A 47-year-old man was aware of a tumor on the sole of his left foot. After preoperative chemotherapy with a diagnosis of SS, wide excision was performed. During postoperative chemotherapy, multiple tumorous lesions developed in the bone (including the whole spine) and both lungs. The patient died 1 year later. Histologically, the excised tumor of the foot showed a biphasic cellular pattern typical of SS, whereas at autopsy the bone and lung lesions were composed only of undifferentiated small round cells with cytoplasmic fibrillar processes. Homer-Wright rosettes were also observed. Immunohistochemically, 80% of the bone and lung tumor cells expressed MIC2 protein homogeneously. To clarify whether the bone and lung round cell tumors were metastatic lesions or second malignancies, especially primary primitive neuroectodermal tumor (PNET)/Ewing's sarcoma (ES), we performed reverse transcription-polymerase chain reaction (RT-PCR) analysis of tumor type-specific fusion gene transcripts. The SYT/SSX fusion transcript was identified in both the foot and lung lesions, whereas the EWS/FLI1 transcript was not detected in either lesion. Therefore, we concluded that the multiple bone and lung tumors were poorly differentiated metastatic tumors, which arose from the SS of the foot. We also conclude that the identification of chimeric fusion transcripts can be successfully applied to poorly differentiated sarcomas and will help in the differential diagnosis of tumors that cannot be distinguished by conventional morphological examinations. Also, it should be remembered that cytoplasmic staining for MIC2 protein may occur in sarcomas other than PNET/ES.- - - - - - - - - - ranking = 0.014407343455647keywords = cell (Clic here for more details about this article) |
4/169. Primary synovial sarcoma of the penis--case report and literature review.Urogenital synovial sarcoma is an exceedingly rare lesion that can be clinically confused with chronic inflammatory masses and granulomas, squamous cell carcinoma, malignant melanoma or metastatic tumours. We present a case of primary synovial sarcoma of the penis in a 29-year-old Egyptian male. This case report is, to the best of our knowledge, the first report in the medical literature. A review of other penile sarcomas is included with a description and discussion of their pathological and clinical manifestations.- - - - - - - - - - ranking = 0.0036018358639118keywords = cell (Clic here for more details about this article) |
5/169. Synovial sarcoma of the upper digestive tract: a report of two cases with demonstration of the X;18 translocation by fluorescence in situ hybridization.Two cases of synovial sarcoma that arose in the upper digestive tract are reported. One case was a polypoid mass that arose at the gastroesophageal junction; the other was a large intramural mass that arose in the wall of the stomach. Both cases had a classic biphasic pattern. In the stomach tumor, the biphasic morphology was focal and there was an abrupt transition to poorly differentiated synovial sarcoma. The tumors had immunohistochemical features that were consistent with synovial sarcoma. Ultrastructural evaluation of the gastroesophageal tumor supported the diagnosis. The diagnostic X;18 translocation was demonstrated by fluorescence in situ hybridization on sections from paraffin-embedded tissue in 86% and 50% of interphase nuclei from the gastroesophageal and gastric tumor, respectively. The translocation was present in equal frequency in the epithelial and spindle cells in the biphasic areas and the poorly differentiated areas of the gastric tumor, indicating that the development of the more aggressive subclone was probably due to genetic mutations not encompassing the SYT-SSX gene fusion product. We are aware of only five reported cases of synovial sarcoma arising in the digestive tract, all in the proximal esophagus. These cases are the first reported arising in the gastroesophageal junction and stomach and the only cases of synovial sarcoma of the digestive tract in which the diagnostic translocation was demonstrated. Sarcomatoid carcinoma (carcinosarcoma) and gastrointestinal stromal tumor are the main differential diagnoses for synovial sarcoma in this site. Synovial sarcoma of the digestive tract may be underdiagnosed, and its recognition may have important clinical implications. fluorescence in situ hybridization is helpful in making this distinction.- - - - - - - - - - ranking = 0.49639816413609keywords = spindle cell, spindle, cell (Clic here for more details about this article) |
6/169. Molecularly confirmed primary prostatic synovial sarcoma.We report the second molecularly-confirmed primary prostatic synovial sarcoma. The diagnosis was particularly elusive at the light microscopic level in that the tumor failed to show epithelial differentiation, but it did show combined spindle-cell and poorly differentiated (round-cell) morphologies. The immunohistochemical staining profile was nonspecific and potentially misleading. Only by demonstration of the characteristic SYT-SSX gene fusion of synovial sarcoma by reverse transcriptase polymerase chain reaction (RT-PCR) analysis of rna extracted from archival material could the diagnosis be confirmed. This case further illustrates the use of this technique in diagnostic pathology.- - - - - - - - - - ranking = 0.13144849445301keywords = spindle, cell (Clic here for more details about this article) |
7/169. Successful restoration of the trapezius muscle using pedicle latissimus dorsi. A case report.Reconstruction of the trapezius muscle using a pedicle latissimus dorsi flap was performed in a 27-year-old man with a large synovial sarcoma in his shoulder girdle. Size and location of the tumor required combined resection of surrounding muscles, including the trapezius, levator scapulae, and rhomboid major and minor. Thus, an extensive defect of the suspending muscles of the scapula was created after accomplishing an adequate resection of the tumor. The flap was performed to restore the trapezius functionally because there were no adjacent muscles available. The transferred muscle compensated for loss of the trapezius, thereby recovering excellent shoulder function. Although an opportunity of its application is thought to occur infrequently, the pedicle latissimus dorsi can activate scapular motion successfully in the absence of the levator scapulae. The technique may be extended to salvage failed conventional reconstruction after spinal accessory nerve palsy.- - - - - - - - - - ranking = 0.0036018358639118keywords = cell (Clic here for more details about this article) |
8/169. Malignant peripheral nerve sheath tumor with a t(X;18).We describe an ankle tumor arising in a 16-year-old girl. The tumor demonstrated histology typical of a malignant peripheral nerve sheath tumor (MPNST), but exhibited a variant form of the (X;18) translocation associated with synovial sarcoma. Immunohistochemical stains were positive for vimentin, CD57, collagen type iv, and Bcl-2. Routine and molecular cytogenetic studies showed an unbalanced 3-way chromosomal translocation that involved chromosomes X, 18, and 1. Electron microscopic findings were noncontributory. This unusual tumor raises the following questions and possibilities: (1) As the t(X;18) suggests, could this tumor be a monophasic synovial sarcoma with the histologic features of an MPNST? (2) Or, as the histology suggests, is this tumor an MPNST that has a t(X;18)? (3) Finally, could MPNST histology, a t(X;18), and no defining immunohistochemical or electron microscopic features represent an as yet unrecognized part of a spectrum that spans from synovial sarcoma to MPNST or other spindle cell tumors?- - - - - - - - - - ranking = 0.49639816413609keywords = spindle cell, spindle, cell (Clic here for more details about this article) |
9/169. Small-cell variant of synovial sarcoma: fine-needle aspiration with ancillary features and potential diagnostic pitfalls.We report a small-cell variant of synovial sarcoma examined by fine-needle aspiration (FNA) biopsy. The patient is a 23-yr-old female who had a synovial sarcoma involving the left infratemporal region, diagnosed at 7 yr of age, followed by a metastatic lesion involving the lung and chest wall 16 yr later. The chest wall metastases was sampled by FNA biopsy. The aspirate consisted of numerous, small, round cells with very high nuclear-to-cytoplasmic ratios. The cytomorphologic features could potentially be confused with other pediatric small round cell tumors. Ancillary studies demonstrated positive staining of the neoplastic cells for cytokeratin, epithelial membrane antigen (EMA), and CD99. The differential diagnosis of other small round cell tumors that may be mistaken for the small-cell variant of synovial sarcoma are presented. We believe that this is the first FNA report detailing the cytologic and ancillary features of the small-cell variant of synovial sarcoma.- - - - - - - - - - ranking = 0.03962019450303keywords = cell (Clic here for more details about this article) |
10/169. Primary synovial sarcoma of the kidney.The authors present two cases of primary synovial sarcoma of the kidney. Both patients had a mass in the upper part of the right kidney without any primary extrarenal neoplastic lesions. Grossly, the tumors were soft to rubbery masses measuring 5.5 cm and 5 cm in diameter, respectively. Histologically, both tumors were poorly differentiated synovial sarcoma. The lesions exhibited a hypercellular solid or lobular growth of round, oval, or short spindle cells in variably solid sheets, in intersecting fascicles, or in a haphazard fashion. Areas of solid aggregation or fascicles of the tumor cells alternating with hypocellular myxoid tissues, together with areas displaying a prominent hemangiopericytoma-like pattern, were found. Immunohistochemically, vimentin was diffusely positive and a few tumor cells were positive for cytokeratin, epithelial membrane antigen, and neurofilament. The tumor cells were negative for S- 100 protein, CD34, smooth muscle actin, and desmin, whereas CD56 and CD99 were positive. In both cases, reverse transcription-polymerase chain reaction using ribonucleic acid extracted from formalin-fixed, paraffin-embedded tissues detected SYT-SSX2 fusion gene transcripts, which are characteristic molecular findings of synovial sarcoma. One patient died 10 months after diagnosis. These tumors are unique cases of primary synovial sarcoma of the kidney confirmed by molecular study.- - - - - - - - - - ranking = 0.51440734345565keywords = spindle cell, spindle, cell (Clic here for more details about this article) |
| | Next -> |