Prostatic stromal sarcoma with rhabdoid features

Rhabdoid tumors have been reported in many different anatomic sites as an aggressive tumor and usually present with a rhabdoid tumor component (a composite tumor) rather than a pure rhabdoid tumor. Rhabdoid tumor in the prostate has been described only once in the prostatic region as a possible epithelial origin. Rhabdoid features in prostatic stromal sarcomas (PSSs) have never been described in the literature. Here, we report a case of a PSS with rhabdoid features. A 31-year-old man presented with a 4-month history of voiding difficulty and anal pain. Computed tomography of the abdomen revealed an ovoid mass in the prostate invading rectum and urinary bladder. A needle biopsy was diagnosed as an unclassified spindle cell sarcoma, and 2 cycles of adriamycin-based neoadjuvant chemotherapy were given, followed by radical prostatectomy. The prostatectomy specimen revealed a high-grade sarcoma with fascicles of highly cellular spindle cells and numerous mitoses with hemorrhage and necrosis. In areas, the tumor also contained sheets of loosely cohesive epithelioid cells with rhabdoid tumor component. Both spindle and rhabdoid tumor cells were positive for vimentin, CD34, and progesterone receptor and negative for desmin and cytokeratin immunostainings. The rhabdoid tumor cells retained INI1 expression. The tumor recurred in the bladder, and the patient died of sepsis. To the best of our knowledge, this is the first case of PSS with rhabdoid features. The tumor showed an aggressive clinical behavior with a short-term survival (7 months after diagnosis).     

Endometrial stromal sarcoma of the uterus with rhabdoid features

A case of endometrial stromal sarcoma of the uterus with rhabdoid features, occurring in a 57 year old woman is reported. Electron microscopy and immunohistochemistry revealed that the rhabdoid cells contained intermediate filaments which were positive for vimentin, cytokeratin, alpha-smooth muscle actin, and muscle specific actin, but not for myoglobin and desmin. This indicated that the tumor in this case differed somewhat from the three rhabdoid tumors and an endometrial stromal sarcoma with rhabdoid differentiation previously reported and that, therefore, these tumors were heterogeneous.     

Primary synovial sarcoma of the kidney: a case report with literature review

We describe a case of primary renal synovial sarcoma (SS) in a 48-year-old man. The patient presented with hematuria and was found to have a large tumor in his left kidney on computed tomography scan. Histology revealed a highly cellular spindle cell neoplasm with minimal pleomorphism. The major differential diagnoses included leiomyosarcoma, hemangiopericytoma, and SS. The presence of focal areas with a biphasic pattern, uniformly positive immunostain for bcl-2, focally positive immunostains for epithelial membrane antigen and cytokeratin, and negative immunostains for CD-34, smooth muscle actin and S-100 established the diagnosis. This was subsequently confirmed by molecular testing for t(X;18) translocation. Since the existence of primary SS in the kidney was first suggested in 1999, to the best of our knowledge a total of 19 cases including the present case have been reported to date. Although primary renal SS is rare, these findings indicate that it should be included in the differential diagnosis of spindle cell tumors of the kidney.     

Primary pulmonary sarcoma with morphologic features of biphasic synovial sarcoma: a case report.

We report an unusual primary case of pulmonary sarcoma that developed in the lung of a 36-year-old woman. The tumor had histologic, immunologic and ultrastructural features identical to those of biphasic synovial sarcoma of the soft tissue. It consisted of an intimate admixture of cytokeratin and epithelial membrane antigen(EMA)-positive neoplastic epithelial cells and vimentin-positive fibroblast-like spindle cells with areas of hyalinization. The patient had a lobectomy and showed no evidence of recurrence or tumor at other sites 15 months after surgery. This case is an useful addition to the small number of published reports on pulmonary synovial sarcoma. The distinctive features of this neoplasm allow it to be different from other types of primary and metastatic malignancies in the lung.     

Primary synovial sarcoma of the kidney: corroboration with in situ polymerase chain reaction

Synovial sarcoma is a tumor of the soft tissues with a unique chromosomal translocation t(X;18)(p 11.2;q11.2) that can be detected by polymerase chain reaction in tissue homogenates. The case of a 32-year-old woman with a primary synovial sarcoma of the kidney is described, the diagnosis was corroborated by the recently developed method of in situ polymerase chain reaction (IS-PCR). Synovial sarcoma of the kidney may be confused with other spindle cell tumors, for that reason IS-PCR may be useful to confirm the diagnosis in paraffin-embedded material.     

Primary cardiac synovial sarcoma

Primary cardiac synovial sarcoma is an uncommon malignant neoplasm, with only a handful of cases reported in the English literature to date. Synovial sarcomas have also been described at other unusual sites, such as the heart, pleuropulmonary region, kidney, prostate, liver, mediastinum, retroperitoneum, gastrointestinal tract, and peripheral nerve. For synovial sarcomas that arise at these unusual locations, definitive diagnosis is challenging and requires use of ancillary diagnostic procedures, such as immunohistochemistry, electron microscopy, and molecular genetic techniques, for confirmation of diagnosis. The nonrandom occurrence of t(X;18) has been found consistently in synovial sarcomas. It has also been found as a sole cytogenetic abnormality in some cases, suggesting it as a key molecular event in tumor development. This review highlights salient features of primary cardiac synovial sarcoma and the associated diagnostic challenges.     

原发性肾脏滑膜肉瘤临床病理及分子遗传学分析

目的探讨原发性肾脏滑膜肉瘤的临床病理特征、诊断及鉴别诊断。方法对1例罕见的原发性肾脏滑膜肉瘤进行光镜观察和免疫组化染色,并用RT-PCR方法在石蜡包埋组织中检测SYT-SSX融合mRNA表达,结合文献分析讨论。结果肿瘤由核分裂活跃的单形的梭形细胞组成,呈交织的束状或实性排列,部分区域呈血管外皮瘤样图像,未见上皮分化成分;囊性区囊内衬核分裂不活跃的多边形的鞋钉样上皮。免疫表型瘤细胞vimentin、CD99、bcl-2、S-100均阳性,Ki-67强阳性;CK(AE1/AE3)、EMA、SMA、CD117、CD34、p53、ER、PR均阴性。RT-PCR方法检测到肿瘤组织中有SYT-SSXmRNA表达,对照组SYT-SSX无表达。患者术后5月内死亡。结论原发性肾脏滑膜肉瘤罕见,需要与肾脏其他一些梭形细胞肿瘤相鉴别,分子遗传学检测SYT-SSX融合mRNA有助于其诊断和鉴别诊断。     

Primary synovial sarcoma of the parotid gland

Synovial sarcoma typically develops in the deep soft tissues of the extremities in adolescents and young adults. Uncommonly, these tumors may arise (9%) in the head and neck region (9%), especially at the cervical and parapharyngeal sites. Primary synovial sarcoma of the parotid gland is a rare occurrence that may not uncommonly cause differential diagnostic difficulties. In these cases, an origin from within the gland rather than a secondary involvement by tumor from a surrounding structure must be confirmed. We report a new case of biphasic synovial sarcoma arising in the parotid gland and review the previously documented cases.     

Primary biphasic synovial sarcoma of the orbit.

Synovial sarcoma is one of the most common soft tissue malignancies of adolescents and young adults. Despite its name, it is no longer thought to be histogenetically derived from the synovium. What seems to be the first case of synovial sarcoma to arise in the orbit presented in a 21 year old woman as a slowly enlarging subconjunctival mass. Although this tumour was typically biphasic, the monophasic spindle cell variant arising at this site could easily be confused with less aggressive orbital connective tissue neoplasms.     

Identification of poorly differentiated synovial sarcoma: a comparison of clinicopathological and cytogenetic features with those of typical synovial sarcoma

AIMS: Poorly differentiated areas in synovial sarcomas (SS) are known to be associated with a poorer prognosis. The aim of our study was to describe the morphological spectrum of poorly differentiated synovial sarcomas (PDSS) and refine the criteria for their recognition. METHODS AND RESULTS: The clinicopathological features of 28 PDSS were compared with those of 26 classic SS. Common cell types in PDSS included epithelioid, spindle and Ewing sarcoma-like small round cells. Unusual features included presence of desmoplastic small cell tumour-like areas and extraskeletal myxoid chondrosarcoma-like areas. The presence of necrosis (P = 0.002), a mitotic rate over 10/10 high-power fields (P < 0.001), a haemangiopericytomatous vascular pattern (P < 0.001) and vascular invasion (P = 0.003) were significantly associated with PDSS, while mast cells (P < 0.001), calcification (P < 0.001) and hyaline bands (P < 0.001) were significantly associated with classic SS. Poorly differentiated areas showed increased proliferative activity with Ki67. PDSS showed a tendency to be larger (P = 0.008) and to be located in proximal more than distal sites (P = 0.025). Three entirely poorly differentiated tumours were diagnosed by demonstration of the t(X;18)(p11;q11) translocation. PDSS showed additional cytogenetic abnormalities. CONCLUSIONS: Poorly differentiated synovial sarcomas show a spectrum of histological features, which may simulate other malignant neoplasms. The diagnosis of entirely poorly differentiated synovial sarcomas requires cytogenetic analysis.     

Primary retroperitoneal synovial sarcoma: a case report

A case of a 36-yr-old woman with retroperitoneal synovial sarcoma is described. Her presenting symptom was epigastric pain that radiating to the back. On radiologic study, bulky retropancreatic soft tissue mass was detected which showed cystic and solid components. At operation, complete resection of the tumor was not possible because of the adhesion to the vena cava and the liver. During the follow-up, extensive tumor recurrence and liver metastasis were revealed. Primary retroperitoneal synovial sarcoma is a very rare malignant tumor with high mortality and recurrence rates. Retroperitoneal synovial sarcoma usually appears as a nonspecific soft tissue mass that do not have specific imaging features differentiating it from other mesenchymal tumors. However general radiologic findings and anatomic location of the tumor may help the diagnosis. In addition, synovial sarcoma should be included in the differential diagnosis of retroperitoneal soft tissue mass detected in young adults.