Case Of Primary Synovial Sarcoma Research Articles

Primary synovial sarcoma arising from a crossed ectopic kidney with fusion

Crossed renal ectopia with fusion is a rare anomaly of the kidney. The present case report describes a 67-year-old man with renal tumor who had been diagnosed as having a crossed ectopic kidney with fusion for 25 years. The pathological diagnosis of the primary tumor specimen was Wilms' tumor with favorable histology. Upon tumor recurrence, molecular detection of SYT-SSX2 fusion transcripts lead to the diagnosis of synovial sarcoma of the kidney. To our knowledge, this is the first case of primary synovial sarcoma arising from a crossed ectopic kidney with fusion.

A Case of Primary Synovial Sarcoma of the Thorax With a Variant SYT-SSX1 Fusion Transcript

With synovial sarcoma (SS) of the thorax, being exceptionally rare, its definite diagnosis is difficult, and the optimal therapy has not yet been established. An examination of our patient, a 64-year-old man with SS using a chest roentgenogram showed a large mass with homogeneous density in the lower two-thirds of the left hemithorax. A computed tomographic image of the chest revealed a large, heterogeneous, enhanced mass in the left hemithorax. Histologic examination of the resected tumor tissues suggested monophasic fibrous SS. A fragment of the SYT-SSX1 fusion transcript, which was smaller than the control, was amplified with reverse transcriptase polymerase chain reaction. Direct sequence analyses revealed the fusion between exon 9 of SYT and exon 5 of SSX1 instead of fusion between exon 10 of SYT and exon 6 of SSX1, which is found in most cases. Although the biological and clinical significance of this rare variant is not yet known, our data present another example of the usefulness of molecular analyses for making a definite diagnosis of SS in unusual sites.

Synovial sarcoma of the sellar region

Primary sarcomas of the sellar region are uncommon, although a wide variety have been reported. To date, no cases of primary synovial sarcoma have been described as occurring at this site. We report an immunohistochemically and molecular genetically confirmed primary synovial sarcoma involving the sellar/parasellar region and cavernous sinus in an adult male. Subtotal resection and radiosurgery proved to be efficacious. The spectrum of primary sellar region sarcomas is summarized.

Primary Synovial Sarcoma of the Thyroid Gland

Synovial sarcoma is a rare but distinct soft tissue neoplasm, most commonly occurring in para-articular regions of the extremities of young adults and also occurring in the head and neck region. To the best of our knowledge, only one case of primary synovial sarcoma of the thyroid has been previously reported. Here, we report a 15-yr-old man who had a chief complaint of a palpable neck mass. The neck computed tomography revealed a relatively well-demarcated solid mass in the left thyroid gland. After fine needle aspiration cytology, total thyroidectomy and lymph node dissection were performed. Grossly, the mass was covered by the same capsule as the thyroid gland, measuring 6×5×5 cm in dimensions and weighing 78 gm. The cut surface showed a well demarcated, lobulated, grayish tan, and rubbery solid tumor. Histologically, this tumor was a biphasic synovial sarcoma. Immunohistochemical, ultrastructural, genetic studies, and cytologic findings were all consistent with synovial sarcoma. When synovial sarcomas arise in this unusual site, recognition and differential diagnosis become more difficult. The differential diagnosis of a spindle epithelial tumor with thymus-like differentiation is very difficult due to their similar clinical, histological, and immunohistochemical features. Ultrastructural and cytogenetic studies for synovial sarcoma are necessary to establish a definitive diagnosis.

Primary Pulmonary Synovial Sarcoma: A Case Report

We report a rare case of primary synovial sarcoma of the lung. A57-year-old man had a well-defined tumor in the right middle lobe seen on chest computed tomography, and underwent lobectomy. Grossly, the nonencapsulated tumor measured 4.5 cm in greatest diameter, with a solid and tan-white cut surface. Histologically, the tumor was mainly composed of a dense proliferation of spindle cells. Immunohistochemical studies were focally positive for epithelial membrane antigen, and diffusely positive for CD99 and Bcl-2. Cytokeratin, S-100 protein, desmin, smooth muscle act in, and CD34 were absent. SYT-SSX1 gene fusion transcript was detected by reverse transcription-polymerase chain reaction, which is diagnostic of primary synovial sarcoma of the lung. We also review the literature with regard to the clinicopathologic, immunohistochemical, and molecular studies of primary pulmonary synovial sarcoma.

Primary Synovial Sarcoma

To describe the clinicopathologic and immunohistochemical features and treatment of a rare case of primary synovial sarcoma of the orbit. Retrospective interventional case report. A 14-year-old young man with histologically proven synovial sarcoma of the orbit. The diagnosis was confirmed by demonstration of a specific chromosomal translocation by polymerase chain reaction studies. The patient was treated with orbital exenteration and followed up for 18 months at regular intervals. The tumor was excised completely, and the patient has done well during the initial 1-year follow-up with no sign of recurrence. We report the fourth case of synovial sarcoma of the orbit and, at 14 years of age, the youngest patient reported.

Thoracic-Abdominal Approach in Primary Pulmonary Synovial Sarcoma

We report a case of primary synovial sarcoma of the lung. The patient was a 32-year-old male who presented with a mass in the right hemithorax invading the peritoneal cavity. The neoplasm was resected through a thoracic-abdominal approach. The patient is doing well 21 months after surgery. Within the last decade thoracic localizations of synovial sarcoma are an emerging histopathological entity thanks to the molecular analysis of the SYT-SSX fusion gene transcript.

Synovial sarcoma in older patients: clinicopathological analysis of 32 cases with emphasis on unusual histological features.

To analyse the clinicopathological features of synovial sarcoma presenting in patients over 60 years of age, an uncommon subset which have not been specifically studied. Thirty-two cases of primary synovial sarcoma in patients aged > or =60 years were retrieved from the authors' consultation files. These were analysed histologically and immunohistochemically and clinical follow-up was obtained in 26 cases (median duration 41 months). Mean age at diagnosis was 71.6 years (range 60-84) with 19 females and 13 males. Anatomical sites were lower limb (n = 13), upper limb (n = 5), lung/pleura (n = 5), trunk (n = 4), head/neck (n = 3), mediastinum (n = 1) and scrotum (n = 1). Histologically, 23 were monophasic and nine were biphasic; 14 were poorly differentiated, of which five showed focally marked pleomorphism. Unusual features in two cases each included organoid nodules, granular cell change, squamous metaplasia and papillary architecture. Ten patients developed local recurrence and 11 developed metastases, of whom seven died. Large tumour size, poorly differentiated morphology and high mitotic rate correlated with poor outcome. Less than 10% of synovial sarcomas occur in patients over 60, in which age group this diagnosis is often not considered. Despite inevitable bias in consultation material, it seems that these cases, when compared with younger age groups, more often show poorly differentiated histology and more often develop at unusual locations.

Low expression of p27Kip1, a cyclin‐dependent kinase inhibitor, is a marker of poor prognosis in synovial sarcoma

BACKGROUND Low expression of p27kip1, a dominant cyclin-dependent kinase inhibitor involved in G1-S transition of the cell cycle, recently has been reported to be associated with aggressive tumor growth. It has been shown that active cell proliferation alludes to poor prognosis in patients with synovial sarcoma. However, to the authors' knowledge, little is known about the clinicopathologic significance of p27kip1 in synovial sarcoma. METHODS p27kip1 expression was examined immunohistochemically in 55 cases of primary synovial sarcoma, and the relations between p27kip1 expression and several cell proliferation markers, i.e., mitotic index (MI), Ki-67 labeling index (Ki-67 LI), and clinicopathologic parameters related to poor prognosis, were determined. Univariate and multivariate survival analyses were performed to evaluate the prognostic significance of p27kip1 expression in synovial sarcomas. RESULTS p27kip1 labeling index (p27kip1 LI) correlated inversely with MI (r = −0.44, P = 0.0007) and Ki-67 LI (r = −0.63, P < 0.0001). Of the clinicopathologic parameters examined, tumor necrosis (P = 0.019) and American Joint Committee on Cancer (AJCC) stage (P = 0.021) correlated significantly with p27kip1 LI. Survival analysis showed that p27kip1 LI was an independent prognostic factor for overall survival in patients with synovial sarcoma (P = 0.0031). CONCLUSIONS The study results suggested that low expression of p27kip1 may be useful as a marker of poor-prognosis synovial sarcoma. Cancer 2001;91:1005–12. © 2001 American Cancer Society.

Low expression of p27Kip1, a cyclin-dependent kinase inhibitor, is a marker of poor prognosis in synovial sarcoma

Low expression of p27(kip1), a dominant cyclin-dependent kinase inhibitor involved in G1-S transition of the cell cycle, recently has been reported to be associated with aggressive tumor growth. It has been shown that active cell proliferation alludes to poor prognosis in patients with synovial sarcoma. However, to the authors' knowledge, little is known about the clinicopathologic significance of p27(kip1) in synovial sarcoma. p27(kip1) expression was examined immunohistochemically in 55 cases of primary synovial sarcoma, and the relations between p27(kip1) expression and several cell proliferation markers, i.e., mitotic index (MI), Ki-67 labeling index (Ki-67 LI), and clinicopathologic parameters related to poor prognosis, were determined. Univariate and multivariate survival analyses were performed to evaluate the prognostic significance of p27(kip1) expression in synovial sarcomas. p27(kip1) labeling index (p27(kip1) LI) correlated inversely with MI (r = -0.44, P = 0.0007) and Ki-67 LI (r = -0.63, P < 0.0001). Of the clinicopathologic parameters examined, tumor necrosis (P = 0.019) and American Joint Committee on Cancer (AJCC) stage (P = 0.021) correlated significantly with p27(kip1) LI. Survival analysis showed that p27(kip1) LI was an independent prognostic factor for overall survival in patients with synovial sarcoma (P = 0.0031). The study results suggested that low expression of p27(kip1) may be useful as a marker of poor-prognosis synovial sarcoma.

Prognostic Significance of Apoptosis in Synovial Sarcoma: Correlation with Clinicopathologic Parameters, Cell Proliferative Activity, and Expression of Apoptosis-Related Proteins

bcl-2 overexpression in synovial sarcomas has been recently reported. Although it is widely known that bcl-2 suppresses apoptosis in various cells, there are no studies that have examined the significance of apoptosis in synovial sarcoma. In the present study, we visualized apoptotic tumor cells by the terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate in situ nick end-labeling (TUNEL) method in 49 cases of primary synovial sarcoma. The degree of apoptosis was analyzed in relation to several clinicopathologic parameters, cell proliferative activity, and immunohistochemical expression of apoptosis-related proteins, including bcl-2, bax, bcl-x, bak, p53, p21 (WAF1/CIP1), Fas, and Fas ligand. TUNEL index (TUNEL-I) significantly correlated with the mitotic index (MI) (ñ = 0.60, P < .0001) and Ki-67 labeling index (MIB1-I) (ñ = 0.52, P = 0.0005). There was a highly significant association between high TUNEL-I value (>.8%) and poor prognosis (log-rank test; P < .0001). Many synovial sarcomas were diffusely positive for bcl-2 family proteins (bcl-2, bax, bcl-x, and bak) and were negative or only sporadically positive for Fas, Fas ligand, p53, and p21 (WAF1/CIP1) proteins. The results indicated that increased rate of apoptosis in primary synovial sarcoma was considered to be an indicator of poor prognosis. In addition, apoptosis in synovial sarcoma may be controlled by multiple apoptosis-regulating mechanisms, including the bcl-2 family.

Primary synovial sarcoma of the gastrocolic ligament.

A case of primary synovial sarcoma of the gastrocolic ligament is reported in a 37-year-old woman. CT showed an encapsulated intraabdominal cystic tumour with an amorphous solid component. Barium studies showed a mass at the gastrocolic ligament which elevated the gastric antrum and displaced the transverse colon downwards. This is the first report of synovial sarcoma formation in the gastrocolic ligament. This tumour may have originated from pluripotential mesenchyme.

A Case of Primary Pulmonary Sarcoma with Morphologic Features of Biphasic Synovial Sarcoma

Synovial sarcoma is a malignant soft tissue neoplasm that occurs frequently in the extremities of young adults, near large joints. The lung is a common site of metastasis but an extremely unusual primary site for synovial sarcoma. We report an unusual case of primary synovial sarcoma that arose in the lung of a 59-year-old woman. The tumor had histologic and immunophenotypic features consistent with biphasic synovial sarcoma These features included of an intimate admixture of cytokeratin and epithelial membrane antigen(EMA)-positive neoplastic epithelial cells and vimentin-positive fibroblast-like spindle cells. The patient had a closed thoracomy drainage and doxycycline pleurodesis for malignant loculated effusion and showed tumor extension in the left whole lung 4 months after pleurodesis. This case is an usual addition to the small number of published reports on primary pulmonary synovial sarcoma The distintive features of this neoplasm allow it to be distinguished from a variety of primary and metastatic malignancies in the lung.

Synovial sarcoma: immunohistochemical expression of p-glycoprotein and glutathione s-transferase-pi and clinical drug resistance

Our purpose was to study the role of the expression of P-glycoprotein (Pgp) and glutathione S transferase-pi (GST-pi) in predicting the response to chemotherapy, relapse-free interval, and survival of patients with synovial sarcoma (SS). Thirty-seven cases of primary SS, without regional lymph node or distant metastases, were studied. There were 17 females and 20 males, ranging in age from 7 to 81 years (median, 31 years) with tumors located in the lower extremity (n = 24) upper extremity (n = 5) and trunchus (n = 8). The cases were retrospectively studied without knowledge of clinical course to compare the immunohistochemical expression of Pgp and GST-pi, flow cytometry parameters (ploidy and % of cells in S+G2 phases), and PCNA and Ki-67 labeling of primary tumors before any therapy, with that observed in local recurrences and metastases after chemotherapy. The relationship of the aforementioned parameters with clinicopathological features (gender, age, and histo-blood group of the patients, size, location, histological subtype. TNM stage, and clinical response to chemotherapy of the tumors) was also evaluated. Results revealed that Pgp and GST-pi were expressed in 29.7% and 40.5% of the cases, respectively. In 48.6% of the tumors there was expression of a least one of the drug resistance markers. The markers were coexpressed in 25.0% of the tumors. The prevalence of Pgp expression was lower, but not significantly, in stage I-II (17.6%) than in stage III (40.0%) tumors, and also in cases without clinical progression (16.7%), than in cases with (36.0%). No such differences were observed for GST-pi expression. Pgp and GST-pi expressions were significantly associated with biphasic SS and were particularly noticeable in solid/glandular areas of biphasic SS. The expression of the drug resistance markers was not significantly associated with gender, age, and histo-blood group of the patients, dimension, location, and proliferative activity of the tumors; it was also not significantly related to relapse-free interval and survival of the patients. The expression of Pgp and GST-pi was not significantly associated either to response to chemotherapy or influenced by chemotherapy. We conclude that Pgp and GST-pi expressions are not good predictors response to of the chemotherapy in patients with localized SS. Other drug resistance mechanisms may be active in SS.

Biphasic Synovial Sarcomas Arising in the Pleural Cavity

Five cases of primary synovial sarcoma of the pleura are presented with a discussion of differentiation from other biphasic malignant neoplasms, most notably malignant mesothelioma. The cases have clinical, histologic, and immunohistochemical features consistent with synovial sarcoma of soft tissue. The average age at initial presentation of the reported patients was 25 years with an approximate range of 9 to 50 years. A large pleural-based intrathoracic mass was identified in each case. Histologic analysis showed a biphasic (mixed) pattern composed of epithelial and spindle cells. The epithelial cells showed cleft-like to tubulopapillary growth with focal intraluminal periodic acid Schiff's (PAS)-positive, diastase-resistant secretions identified in four of the five cases. The spindle cell component was composed predominantly of densely packed, elongated, fusiform cells. Immunohistochemical staining showed positivity with antibodies against cytokeratin, BER.EP4, epithelial membrane antigen, and vimentin in all cases. The patients seem to have an aggressive course, with four deaths reported within 3 years from initial surgery. These cases represent the first reported cases of primary synovial sarcoma of the pleura and lend further credence to the theory that synovial sarcomas are derived from immature mesenchymal elements, not from synovium.

Primary synovial sarcoma of the middle ear

Synovial sarcoma is a soft tissue malignancy which most commonly affects the lower limbs of young adults and rarely occurs in the head and neck region. The term synovial sarcoma may be a misnomer as most of these tumours occur in tissues not known to contain synovial tissue. There has only been one previously reported case affecting the middle ear, which was metastatic, and we report the first case of primary synovial sarcoma of the middle ear.

Synovial sarcoma in the neck of an eleven month old girl

Synovial sarcomas are rare primary tumors of the head and neck in any age group. We present a case of primary synovial sarcoma arising in the neck of an 11-month-old girl.

Synovial sarcomas of the neck.

SYNOVIAL sarcomas comprise approxi mately 8.5% of all malignant tumors of the somatic soft tissues and according to Pack and Ariel are the commonest sarcomas of the hands and feet.1 A primary synovial sarcoma of an area besides the extremity is rare, but such lesions have been reported in the abdominal and chest walls, lower back, and neck. In a recent review of tumors of soft tissues, Martin et al record no synoviomas in the cervical region from a total of 412 malignant soft tissue neoplasms.2In 1961, Harrison and his associates, after a review of the literature, accepted only five cases of primary synovial sarcoma of the neck.3Because of its rarity and histological peculiarities, the neoplasm may be a perplexing one for both the surgeon and the pathologist. In the present report, the authors record three additional cases, including one from their own experience. Report