Biphasic synovial sarcoma of oral cavity

Synovial sarcoma is a mesenchymal spindle cell tumour, which is unrelated to synovium and shows variable epithelial differentiation. Typically, synovial sarcoma arises in the soft tissues of the extremities but cases in the head and neck region are less common and oral cavity involvement is extremely rare. A 17-year-old girl presented with a gradually increasing swelling on the right cheek for 2 years, which on biopsy, revealed a biphasic tumour comprising fascicles of spindle shaped cells with gland formation by epithelial cells and scattered masts cells. Histological diagnosis of biphasic synovial sarcoma was confirmed on immunohistochemistry by strong positivity for EMA, S-100 and CD-99 in both epithelial as well as spindle cell areas.     

Primary synovial sarcoma of the kidney

Primary renal synovial sarcomas (SS) are rare tumors of the kidney. Faria first described primary renal synovial sarcoma in 1999. Twenty-one cases of primary renal synovial sarcoma have been reported to date. Primary renal synovial sarcomas can exist in either a monophasic or a biphasic pattern. The monophasic variant of primary renal synovial sarcoma is more common and tends to have a better prognosis than the biphasic variant. We present the case of a 61 year-old woman with a monophasic variant of primary renal synovial sarcoma.     

Synovial sarcoma with squamous differentiation of its mesenchymal glandular elements. A case report with light-microscopic, ultramicroscopic, and immunologic correlation

A 25-year-old male patient with a biphasic synovial sarcoma of his right flank is reported. The tumor was not only richly glandular but also showed a rare finding in association with a sarcoma, namely, squamous differentiation. Light microscopy and electron microscopy demonstrated the classical features of a biphasic synovial sarcoma except for foci of squamous change. Antibody stain directed against keratin demonstrated this substance in both the glandular and squamous cells but not in the stromal elements. Therefore, the finding of squamous differentiation in association with a malignant, deep soft-tissue tumor must include in the differential diagnosis not only carcinomas but rare sarcomas as well, including the biphasic synovial sarcoma and the glandulosquamous variant of malignant schwanomma.     

Synovial sarcoma of the head and neck.

Primary synovial sarcoma is an unusual tumor of the head and neck. Fewer than 75 cases have been reported in the literature. We have treated 7 additional cases; 3 in the hypopharynx, 2 in the parapharyngeal space, 1 in the oral pharynx and 1 in the posterior triangle of the neck. An enlarging cervical mass, voice change, and dysphagia were among the presenting complaints. CT revealed solitary nonhomogenous tumors from 3 to 7 cm in diameter. Microscopically, all cases showed a biphasic cellular pattern verified by immunohistochemical staining. Multimodality treatment consisted of surgery and postoperative radiation therapy with 3 patients receiving chemotherapy. Although the original pathology report was incorrect in 3 cases, clinical suspicion for synovial sarcoma ensured proper diagnosis.     

Primary renal synovial sarcoma in a 13-year-old boy

Primary renal synovial sarcoma is a rare entity with fewer than 40 cases reported in the literature. Its clinical presentation and radiographic features, namely, its often complex cystic appearance, make it difficult to differentiate from other benign or malignant renal lesions. Although there are certain consistent morphological and immunohistochemical features, diagnosis ultimately depends on molecular studies. Prognosis is poor, and there currently exists no defined treatment protocol. Herein, we describe the youngest reported case of primary renal synovial sarcoma in the literature.     

Synovial sarcoma: a clinical, pathological, and ultrastructural study of 26 cases supporting the recognition of a monophasic variant

Twenty-six cases of synovial sarcoma (14 biphasic, 12 monophasic) were subjected to a clinicopathological study that included electron-microscopic examination of six tumors. Monophasic tumors were composed predominantly of uniform, densely packed, small spindle cells with scant cytoplasm identical to those of the "stromal" elements of typical biphasic tumors. The arrangement of these cells into narrow interlacing fascicles, forming tight whorls and showing little collagenization, was distinctive for this tumor. Major clinical differences between the two types of synovial sarcoma were the tendency for monophasic tumors to arise in distal extremity locations (seven of 10), and the poorer prognosis of monophasic tumors, 30% surviving 5 years compared to 58% for biphasic tumors. At the ultrastructural level, monophasic tumors and the spindle-cell components of biphasic tumors were identical. Both were composed of spindle or polygonal cells attached by numerous desmosomes. Prominent Golgi, abundant RER, perinuclear microfilaments, and glycogen aggregates were characteristic. Intercellular spaces containing elongated cytoplasmic filopodia were observed consistently, as were fragments of basement membrane-like material. The EM findings concur with those described previously in normal and pathologic synovium, and support a synovioblastic origin for the monophasic variant of synovial sarcoma.     

Primary mediastinal synovial sarcoma: A rare case report

IntroductionSynovial sarcomas commonly occur in the extremities of young adults. A primary occurrence in the mediastinum is very rare with only a few reported cases in the world literature.We report a case of mediastinal synovial sarcoma. This paper is about a 47-year-old male who presented with retrosternal chest pain and shortness of breath on exertion. Imaging showed an anterior mediastinal mass. Pathological examination of the resected mass showed a biphasic neoplasm with a spindle cell component admixed with gland-like elements. The tumor showed positive staining with cytokeratin, epithelial membrane antigen and vimentin confirming the diagnosis of a biphasic synovial sarcoma.DiscussionA wide range of neoplasms, both primary and metastatic, occur in the mediastinum, which pose considerable diagnostic difficulties. A synovial sarcoma should always be considered in the differential diagnosis, and immunohistochemistry is an important adjuvant tool in this situation.ConclusionThis paper highlights the importance of recognizing an unusual presentation of this aggressive neoplasm to aid appropriate clinical management.     

A biphasic tumor of the mediastinum with features of synovial sarcoma. A report of four cases

Four cases of a biphasic mediastinal tumor histologically identical to synovial sarcoma of soft tissue were observed. The tumors presented as solitary mediastinal masses. Although the tumors were frequently adherent to adjacent pleura or pericardium, none appeared to be arising from a mesothelial surface. All cases were composed of an intimate admixture of keratin-positive epithelial cells and vimentin-positive spindle cells with areas of transition, hyalinization, and calcification. Follow-up was available on three patients, who died of their disease 10 months, 14 months, and 4 years after diagnosis, respectively. Although synovial sarcoma usually occurs in deep soft tissues near joints in the extremities, it has been reported in locations removed from synovial, tendon sheath, and bursal structures. This report adds a previously unrecognized location for synovial sarcoma, the mediastinum. Occurrence in this location further supports an origin from pluripotential mesenchyme as opposed to synovium. The differential diagnosis includes mesothelioma, thymoma, germ cell tumors, malignant peripheral nerve sheath tumor with glandular differentiation, and metastatic carcinoma.     

Histochemical characterization of mucosubstances in synovial sarcoma

Six cases of synovial sarcoma were examined histochemically in order to clarify the components of mucosubstances in the tumor tissues. The tumors were classified into 1) monophasic type, 2) predominantly monophasic type with focal biphasic differentiation, and 3) biphasic type. The former two groups and sarcomatous areas in the biphasic tumors contained various amounts of hyaluronic acid, chondroitin sulfate, and, in some cases, heparitin sulfate. By contrast, the epithelioid regions in the biphasic-type tumors had periodic acid-Schiff-positive glycoproteins which contained various amounts of sialic acid, in addition to hyaluronic acid and chondroitin sulfate. The significance of the presence of glycoproteins in the mesenchymal tumors is emphasized. It seems likely that the synovial sarcomas contain various kinds of mucosubstances and that sensitivity to hyaluronidase treatment is not necessarily the diagnostic criterion of synovial sarcoma.     

Synovial sarcoma. A clinicopathological study of 31 cases

Summary Thirty-one surgically treated cases of synovial sarcoma were reviewed. The mean age of the patients at primary surgery was 37 years (range 10–78 years). Twenty-nine of the tumors were of the biphasic type and two were monophasic. Currently a monophasic synovial sarcoma can be considered as a specific entity. In contrast to other soft-tissue sarcomas, synovial sarcomas present the characteristics of a carcinosarcoma. The 5-year survival rate in this study was 55% and the 6-year survival rate 50%; after 6 years there were no recurrences. The primary treatment should follow the same guidelines that are currently given for other soft-tissue sarcomas: wide and radical excisional margins should be aimed at. Excisional treatment even of repeated pulmonary recurrences may be rewarding.I am grateful to Dr. Markku Miettinen for reviewing the histological material. I also thank Professor Erkki Saxén for making his histologic material available. Financial support was received from the Juselius Foundation and the Paulo Foundation     

Abklärung einer progredienten Okklusionsstörung

A 61-year-old male patient presented with a progressive malocclusion and neuropathic pain mimicking trigeminal neuralgia which had been unsuccessfully treated with carbamazepin over a long period. Magnetic resonance imaging revealed a tumor mass in the lower compartment of the left temporomandibular joint. Total excision of the disk was carried out and histological examination revealed a synovial sarcoma. This case shows the urgent need for using imaging techniques of every non-traumatic progressive malocclusion.     

Synovial sarcoma of the pleura: a clinical and pathologic study of three cases

Synovial sarcomas are rare soft tissue malignancies that most commonly affect the extremities in the vicinity of large joints. These malignancies typically occur in adolescents and young adults between the ages of 15 and 40 years.(1,2) Historically they are believed to originate from primitive pluripotent mesenchyme capable of synovial differentiation. This belief is consistent with the malignancy's origin from sites devoid of normal synovium, such as the pleural cavity. A variety of pleural cavity sarcomas have been described, including liposarcoma,(3) chondrosarcoma,(4) osteosarcoma,(5) and malignant schwannoma.(6) Pleural synovial sarcoma, however, is a much rarer entity. In fact, pleural synovial sarcoma was first described only 6 years ago(7) and has not yet been reported in the surgical literature. Because of its rarity, pleural synovial sarcoma is often mistaken for the histologically similar malignant mesothelioma, the most common of the pleural neoplasms. This is a critical distinction, because synovial sarcoma may be extremely aggressive. Studies in the last 10 years have shown it to be extremely sensitive to ifosfamide-based chemotherapy, and survival of patients with synovial sarcoma has recently increased with chemotherapy, with 5-year survivals now as high as 57%.(8-10) In this report, we describe 3 cases of synovial sarcoma of the pleura. Clinical findings are correlated with pathologic features, including immunohistochemical stains and fluorescence in situ hybridization (FISH) for the identification of the diagnostic chromosomal translocation, t(X;18)(p11.2;q11.2). This delineation of the clinical and pathologic aspects of this rare, newly recognized tumor should increase awareness among the surgical community.     

Synovial sarcoma--identification of favorable and unfavorable histologic types: a Scandinavian sarcoma group study of 104 cases

Synovial sarcoma has traditionally been regarded as a high-grade sarcoma and treated as such. Recently, specific types of poorly differentiated synovial sarcoma have been defined and shown to affect prognosis adversely. We studied 104 primary synovial sarcomas of the extremities and trunk wall without metastasis at diagnosis that were retrieved from the Scandinavian Sarcoma Group Registry (SSG) and the Swedish Cancer Registry from 1986 to 1994. Follow-up was available in all patients, median 6 (3-11) years for the survivors. There were local recurrences in 15% of patients and metastases in 33%. Histologically, the tumors were divided into favorable and unfavorable types. The favorable type had no significant cytologic atypia, and in most instances, no necrosis and a mitotic count of < 10/10 hpf. The unfavorable type included so-called poorly differentiated synovial sarcomas as well as recognizable biphasic and monophasic synovial sarcomas with prominent nuclear atypia, extreme cellularity and nuclear crowding. Designation of a tumor as having favorable vs. unfavorable histology conveyed more prognostic information than any single histologic factor. Kaplan-Meier estimates of metastasis-free survival at 5 years were 83% for patients with histologically favorable tumors and 31% for patients with histologically unfavorable tumors (95% confidence intervals 72-92% and 13-51%, respectively). These findings may influence future treatment protocols for synovial sarcoma.     

原发性肾脏滑膜肉瘤2例报告并文献复习

目的探讨原发性肾脏滑膜肉瘤的诊断和治疗。方法对2例患原发性肾脏滑膜肉瘤的患者的临床资料进行总结,检索Pubmed及中国生物医学数据库。肾脏原发性滑膜肉瘤的相关文献。结果患者1术后病理报告：肾脏滑膜肉瘤,侵及肾皮质、髓质及肾周围脂肪组织。免疫组化结果：Vim、CK7、广谱CK、低分子CK、EMA、CD99、Bcb2均阳性,Des阴性。患者2术后病理报告：肾脏滑膜肉瘤,血管内见有瘤栓。免疫组化结果：Vim阳性、CK部分细胞、Des部分细胞、CD99部分细胞阳性,Myodl、CD34、Melanoma、CD68阴性。患者1术后7个月,患者2术后10个月均死于癌转移。检索相关文献显示有随访的33例中死亡11例,转移或复发14例。结论原发性肾脏滑膜肉瘤是一种高度恶性的罕见肿瘤,预后不良,确诊依赖病理检查、免疫组织化学。     

Intraarticular synovial sarcoma.

A 43-year-old man presented with decreased range of motion in his left knee and a painful medial joint mass that was grossly visible. Arthroscopy demonstrated a mobile, flat mass 3 cm in diameter in the knee joint that seemed to be loosely tethered to the synovium. The mass was excised, and light microscopic examination demonstrated a biphasic synovial sarcoma. There was no transition with the attached normal synovium. Immunohistochemically, the epithelial component was intensely positive for epithelial membrane antigen and cytokeratins (CAM 5.2 and AE 1/AE 3), and the spindle cell component was focally positive for these markers. The patient has no evidence of disease 9 years after only local excision. Although the term synovial sarcoma suggests a relationship to normal synovium, only rarely has truly intraarticular disease been reported.     

Biphasisches primäres Synovialsarkom der Niere

Primary synovial sarcoma of the kidney is a rare neoplasm, which was first described by Argani et al. in 2000. We report a case of a 47-year-old man presenting with a renal mass of 11.7x9.3x8.1 cm size and a caval thrombus, which was classified as a biphasic differentiated spindle cell tumor. Fluorescence in situ hybridization (FISH) revealed a translocation t(X;18), specific for synovial sarcoma. In younger patients presenting with spindle cell-like renal tumors, a primary synovial sarcoma should be considered as a differential diagnosis.     

特制人工髋关节假体在髋部骨肿瘤的应用分析

目的探讨特制人工假体在髋部肿瘤保肢术中的应用价值。方法回顾性分析我院自1999年3月至2005年5月髋部原发肿瘤患者行特制人工假体置换术后疗效、并发症及处理资料。本组肿瘤病例中,髋臼11例,股骨近端15例;肿瘤类型:软骨肉瘤3例,骨肉瘤6例,滑膜肉瘤1例,骨巨细胞瘤12例,良性纤维组织瘤2例,动脉瘤样骨囊肿2例。假体类型:人工全髋关节假体17例,人工双极股骨头7例,马鞍式关节假体2例。结果随访时间为18个月～6年,平均4年3个月;10例恶性肿瘤局部复发率40%,保肢率60%,16例中间性及良性肿瘤复发率12.5%,保肢率87.5%,参照Enneking(MSTS)评定标准平均得分19分,优良率76.9%。结论特制人工髋关节假体具有良好的术后肢体功能,是髋部骨肿瘤较为满意的保肢治疗方法之一。     

Synovialosarcoma of the pharynx: A case report and literatture review

IntroductionSynovial sarcoma is a rare tumor to be encountered in the head and neck region and is always a challenge in terms of diagnosis, treatment, as our case.Presentation of caseWe present a 23-year old female patient with synovial sarcoma of posterolateral pharyngeal wall. The radiological and clinicopathological features along with various diagnostic tests and treatment options are discussed.DiscussionThe objective of this study is to describe - from a clinical case reported from our institution, and from literature review- the clinical, radiological and histological features of pharyngeal synovial sarcoma and to discuss its therapeutic management.ConclusionSynovial sarcoma of pharynx is extremely a rare tumor in current practice.