1/99. 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.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/99. 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 = 1.049033435061keywords = undifferentiated (Clic here for more details about this article) |
3/99. 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 = 0.81148666892994keywords = carcinoma (Clic here for more details about this article) |
4/99. 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 = 0.81148666892994keywords = carcinoma (Clic here for more details about this article) |
5/99. 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 = 10.796428244924keywords = spindle-cell (Clic here for more details about this article) |
6/99. 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.- - - - - - - - - - ranking = 6keywords = neoplasm (Clic here for more details about this article) |
7/99. 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.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/99. 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.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/99. 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.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
10/99. 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.- - - - - - - - - - ranking = 2.6229733378599keywords = neoplasm, carcinoma (Clic here for more details about this article) |
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