1/40. 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 = antigen (Clic here for more details about this article) |
2/40. 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 = 1keywords = antigen (Clic here for more details about this article) |
3/40. 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 = 1keywords = antigen (Clic here for more details about this article) |
4/40. Capacity for epithelial differentiation in synovial sarcoma: analysis of a new human cell line.AIM: To analyse the capacity for epithelial differentiation in synovial sarcoma using a new human cell line. methods: A new human cell line, KU-SS-1, was established from a monophasic, spindle cell type of synovial sarcoma by grafting those cells on to severe combined immunodeficient (SCID) mice and then transferring them to in vitro culture systems. The KU-SS-1 cells were characterised by light and electron microscopy, and by immunohistochemical, flow cytometric, and cytogenetic analysis. RESULTS: Primary tumour and cultured cells at passage 20 showed a positive reaction for vimentin, which is a mesenchymal marker. After 40 passages, subcultured cells were injected into SCID mice to induce further tumours. These advanced subcultured cells and the tumour cells that they induced were positive for cytokeratin, an epithelial marker, and exhibited epithelial ultrastructural features such as intermediate junctions. Furthermore, two colour immunofluorescent analysis for proliferating nuclear cell antigen (PCNA) and intermediate filaments showed that a large number of PCNA expressing cells were positive for vimentin, and that part of this fraction also expressed cytokeratin. The existence of cells with reactivity for these three markers indicated that, in this cell line, a fraction with high proliferating capacity had both mesenchymal and epithelial markers. In addition, cytogenetically, this cell line expressed the SYT-SSX chimaeric transcript as a result of the t(X;18) (p11;q11) translocation. CONCLUSIONS: A human synovial sarcoma cell line was established and stably maintained in cell culture for more than 70 passages. In addition, this cell line showed epithelial differentiation, which supports the hypothesis that synovial sarcoma is a carcinosarcoma like tumour with true epithelial differentiation. This cell line will be a useful tool for investigating the nature of this tumour and will contribute to clinical studies.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/40. 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 = antigen (Clic here for more details about this article) |
6/40. Calcifying/ossifying synovial sarcoma shows t(X;18) with SSX2 involvement and mitochondrial calcifications.AIMS: Synovial sarcoma with extensive calcification and ossification is a rare variant, the ultrastructural, cytogenetic and molecular analysis of which has not been reported previously. methods AND RESULTS: A large mass in the shoulder of a 20-year-old male patient led to a deformity of the chest wall, thus supporting the hypothesis that this is a slowly growing variant of synovial sarcoma. Nevertheless, the patient developed metastatic lung disease 7 months after resection. On histology, the monophasic spindle cell proliferation was in several areas obscured by the massive calcification and ossification. immunohistochemistry showed keratin, epithelial membrane antigen, vimentin and CD99 expression. The cytogenetic analysis revealed a single t(X;18)(p11.2; q11.2), typical for synovial sarcoma. Additional fluorescence in-situ hybridization revealed SSX2 involvement. At the ultrastructural level, prominent needle-shaped intramitochondrial crystals were present, both in the cytoplasm and in the extracellular matrix. CONCLUSION: The presence of the t(X;18) with SSX2 involvement definitively characterizes this tumour as a variant of synovial sarcoma. In addition, the needle-like mitochondrial calcifications give a possible clue to the pathogenesis of the extensive metaplastic ossification and calcification.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/40. Primary synovial sarcoma of the kidney: Report of a case confirmed by molecular detection of the SYT-SSX2 fusion transcripts.We describe an unusual case of primary synovial sarcoma of the kidney. A 47-year-old woman had a tumor massively replacing the right kidney. There were no primary extrarenal neoplastic lesions. Microscopically, the tumor was composed of a cellular proliferation of relatively uniform spindle-shaped cells having atypical spindle or oval nuclei arranged in fascicles with tumor necrosis, without epithelial areas. Immunohistochemically, a small number of the tumor cells were positive for epithelial markers such as cytokeratin and epithelial membrane antigen. The SYT-SSX2 fusion transcripts were detected by a reverse transcription-polymerase chain reaction (RT-PCR) using rna extracted from formalin-fixed, paraffin-embedded tissue. ETV6-NTRK3 fusion gene transcripts that result from t(12; 15)(p13;q25), which is characteristic of cellular congenital mesoblastic nephroma, were not demonstrated. To our knowledge, this is the ninth case of primary renal synovial sarcoma. This case report indicates that synovial sarcoma should be taken into account for the differential diagnosis of renal spindle cell tumors and the molecular assay detecting the SYT-SSX fusion transcripts is useful for the final diagnosis of synovial sarcoma arising in an unusual location.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/40. Primary synovial sarcoma of the lung: a case report confirmed by molecular detection of SYT-SSX fusion gene transcripts.We report a rare case of primary synovial sarcoma of the lung. The patient was a 49-year-old woman who presented with a well-defined oval-shaped mass in the left upper lobe on a chest radiograph. A malignant pulmonary tumor was suspected and consequently a left upper lobectomy was performed. Grossly, the tumor measured 5 x 4 cm, was whitish-yellow in color and soft in consistency. Histologically, the tumor showed a dense proliferation of short spindle cells, partly arranged in interlacing fascicles. In some areas a hemangiopericytoma-like pattern, stromal myxoid change and necrosis of various sizes were noted. Numerous mitotic figures were also seen. Immunohistochemically, the tumor cells were positive for epithelial markers such as cytokeratin and epithelial membrane antigen. As these features suggested a monophasic fibrous type of synovial sarcoma, we examined for the presence of SYT-SSXfusion gene transcripts using rna samples from the frozen tumor tissue. A reverse transcription polymerase chain reaction amplified a single 583-base pair fragment characteristic of synovial sarcoma. As no other tumorous lesions were found during a follow-up period of 1 year, primary synovial sarcoma of the lung was our final diagnosis. This tumor should be considered in the differential diagnosis of round to short spindle cell tumors arising in the lung.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/40. Synovial sarcoma arising from the pleura: a case report with ultrastructural and immunohistological studies.Synovial sarcoma commonly occurs in the para-articular regions of the extremities, and rarely in the pleura. We report a 46-year-old woman with primary synovial sarcoma of the pleura. She was admitted with a complaint of left-sided chest pain and exertional dyspnea. She had previously undergone two operations for pleural neoplasm, at the ages of 33 and 36 years. A computed tomography scan revealed an expanded mass in the left thoracic cavity, involving the surrounding tissue. Macroscopic findings demonstrated a 25 x 25 x 15-cm grayish-white mass with hemorrhage beneath the pleura. Both epithelial and spindle cells were observed microscopically. Ultrastructural microscopy of the epithelioid cells demonstrated short, blunt microvilli on the luminal surface, and desmosomes between the neoplastic cells. Immunohistochemically, the tumor cells of the epithelial component were positive for embryonal membrane antigen (EMA), carcinoembryonic antigen (CEA), human mesothelial cells (HBME)-1, and cytokeratin, and the spindle cells were positive for vimentin. These findings led us to a diagnosis of primary synovial sarcoma of the pleura. She had no evidence of recurrence or metastasis after the third operation.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
10/40. radiation-associated synovial sarcoma: clinicopathologic and molecular analysis of two cases.Development of a soft-tissue sarcoma is an infrequent but well-known long-term complication of radiotherapy. Malignant fibrous histiocytomas, extraskeletal osteosarcomas, fibrosarcomas, malignant peripheral nerve sheath tumors, and angiosarcomas are most frequently encountered. radiation-associated synovial sarcomas are exceptional. We report the clinicopathologic, immunohistochemical, and molecular features of two radiation-associated synovial sarcomas. One tumor developed in a 42-year-old female 17 years after external irradiation was given for breast carcinoma; the other occurred in a 34-year-old female who was irradiated at the age of 7 years for a nonneoplastic condition of the left hand. Both lesions showed morphologic features of monophasic spindle cell synovial sarcoma, were immunoreactive for cytokeratins, epithelial membrane antigen, CD99, CD117 (c-kit), and bcl-2 and bore the t(X;18) (SYT-SSX1) translocation. We conclude that synovial sarcoma has to be added to the list of radiation-associated soft-tissue sarcomas.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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