Biphasic synovial sarcoma of the abdominal wall

Synovial sarcoma arising in the abdominal wall is a rare tumor. We report a case of a 38-year-old man who complained of abdominal pain. Physical examination revealed a firm mobile mass, 25 cm in diameter, in the left lower abdominal wall. The tumor was first thought to be a sarcoma arising from the omentum or mesentery. During surgery, a large tumor was found attached to the inner surface of the abdominal wall and compressing the gastrointestinal tract. On microscopic examination the tumor corresponded to a biphasic synovial sarcoma immunoreactive for cytokeratins (AE1/AE3, 7 and 19), epithelial membrane antigen and carcinoembryonic antigen in the epithelial tumor cells, for E-cadherin especially in their glandular structure, vimentin, CD99, and CD56 in the spindle cell component and for bcl-2 protein. The tumor recurred at the same site, and clinical course progressed to death 3 months after the initial diagnosis.     

Synovial sarcoma enzyme histochemistry of a typical case

Summary A typical case of biphasic synovial sarcoma was studied using enzyme histochemistry. A marked difference between the staining characteristics of the spindle cells and the epithelial-like cells was demonstrated by reactions for various hydrolytic enzymes. The epithelial-like cells exhibited a strong reactivity for alkaline phosphatase, acid phosphatase, adenosine triphosphatase and nonspecific esterase, whereas spindle-cells were completely unreactive when tested for these enzymes.This is, to our knowledge, the first report demonstrating differences in the enzymatic pattern of the two cell populations which compose synovial sarcoma.     

Synovial sarcoma, a primary liver tumor--a case report

An 18-year-old female presented with a mass involving the liver which was resected. Microscopically, it was diagnosed as monophasic synovial sarcoma. Cytogenetic study (FISH) revealed translocation t(X; 18), confirming the diagnosis.     

Synovial sarcoma: an immunohistochemical study

The immunohistochemical staining pattern of 18 cases of synovial sarcoma with two epithelial-specific monoclonal antibodies is described. This is compared with normal synovium, cases of giant cell tumour of tendon sheath (benign synovioma) and a variety of spindle celled sarcomas. Sixteen cases of synovial sarcoma showed staining of the epithelial component with at least one antibody. No staining was seen in normal synovium or in giant cell tumours of tendon sheath. A small number of malignant schwannomas contained groups of cells which stained positively whilst other spindle cell sarcomas either did not stain or showed 'cross-reaction' type staining only. These results add weight to the proposition that synovial sarcomas do not arise from normal synovium, despite their morphological similarities, but from mesenchymal connective tissue. It is also shown that immunohistochemical staining with anti-epithelial antibodies will emphasize the biphasic pattern of synovial sarcomas allowing their distinction from other sarcomas.     

Synovial sarcoma of the abdominal wall

Summary A synovial sarcoma of the abdominal wall in a 56-year old woman showed the typical features of this tumor type. Histologically a characteristic biphasic cellular pattern with epithelium-like cell complexes and sarcomatous spindle cell areas was found. The histochemical examination revealed that tumor cells synthesize glycoproteins and weakly acid glycosaminoglycans (mainly hyaluronic acid). Electron microscopically the tumor cells in epithelium-like cell islets were sometimes arranged in gland-like formations with microvilli at the luminal side, specialized intercellular junctions and a peripheral basement membrane-like condensation of the ground substance. There was no fundamental cytological difference between cells of epithelium-like and spindle cell areas. Generally the tumor cells imitated cells of the synovial membrane and we found no evidence for origin from cells of the nerve sheath. Because of the submicroscopic relationship and histochemical similarities of synovial sarcomas and mesotheliomas we suggest that they should be united in a group of sarcomas with possible biphasic cellular pattern, while preserving their clinicopathologic definition.     

Epithelial markers in synovial sarcoma

Summary Immunohistochemical studies on synovial sarcomas have proved the potentiality of these neoplasm for epithelial and mesenchymal differentiation and antibodies detecting epithelial cells have been found to be helpful in determining the histological types. In this study different epithelial markers directed against various cytokeratins, HMFG-2 and EMA were investigated on paraffin embedded tissues of 13 cases of synovial sarcomas, with regard to their reliability in unmasking the epithelial components demonstrable in this type of neoplasm. The results lead to three conclusions firstly, synovial sarcomas possess the capacity for generating different epithelial cell types with uncommon compositions of intermediate filaments as well as of membrane proteins, secondly, these features may be expressed in a heterogenous pattern even within the same tumour and finally, the use of wide range anti-cytokeratin antibodies covering the spectrum of basic as well as acidic type proteins seems to be necessary for the detection of all epithelial components demonstrable in synovial sarcomas.     

Morphological and Cytogenetic Studies of a Human Synovial Sarcoma Xenotransplanted into Nude Mice

Specimens of a synovial sarcoma from the left thigh of a 60 year old woman and cells from the tumor transplanted into nude mice were examined morphologically and immunohistochemically, and the karyotype was analyzed. lmmunohistochemically, cells of both the parent tumor and the transplanted tumor were positive for vimentin and cytokeratin. The multicystic features of the parent tumor were not reproduced in the mice. On ultrastructural examination, the parent tumor cells were found to have dilated channels of endoplasmic reticulum as well as a junctional apparatus, and they formed extracellular spaces. The transplanted tumor cells, in contrast, had a poorly developed endoplasmic reticulum and were devoid of extracellular spaces. Chromosome analysis of the tumor cells revealed a translocation, t (X; 18) (p 11; q11), as reported previously for synovial sarcoma, thus suggesting the diagnostic utility of the chromosome pattern for identification of synovial sarcoma.     

Epithelial Differentiation in Synovial Sarcoma Correlation with Histology and Immunophenotypic Expression

The epithelial properties of the cells in six cases of synovial sarcoma, five biphasic and one monophasic, were examined. Epithelial cells formed well demarcated glandular nests with a multi or single layered lining, poorly defined nestlike clusters, or appeared as scattered single plump cells in a spindle cell stroma. Keratinization was seen in multi-layered epithelia, and was suggested in single layered epithelia from the presence of some enlarged eosinophilic cells. Immunohistochemically, epithelia lining glands showed reactivity for both low and high molecular weight (MW) cytokeratins, and the reactions for both were strongest in keratinized cells. Clustered or single plump cells showed low MW cytokeratin reactivity. All epithelial cells were negative for the largest cytokeratin (no. 1), and occasionally stained positively for vimentin. Spindle-shaped stromal cells usually stained positively for vimentin only. Type IV collagen was distributed linearly around well circumscribed epithelia, but not around poorly defined epithelia or plump cells. Epithelial membrane antigen was distributed linearly along glandular spaces and irregularly or granularly in clustered plump cells. The immunophenotypic epithelialization in synovial sarcoma was restricted, but showed considerable correlation with the histological grade of differentiation. Acta Pathol Jpn 39: 381 387, 1989.     

Synovial sarcoma of the neck masquerading as a malignant second branchial cleft cyst

Synovial sarcoma is an uncommon, aggressive malignant tumor of the soft tissues primarily involving the extremities of young adults. Head and neck synovial sarcoma is rare, and its diagnosis and therapy are still challenging.We report a case of a young patient with synovial sarcoma, clinically masquerading as cystic mass of the neck and malignant second branchial cleft cyst. The pathological diagnosis of the sarcoma was confirmed by a multimodality diagnostic protocol, including histological, immunohistochemical and molecular genetic analysis. The patient underwent complete surgical excision followed by postoperative radiotherapy and recovered well.     

Synovial sarcoma of the heart

Primary synovial sarcomas of the heart are exceptionally rare tumours, only five being previously documented. We report a sixth case and review published data. Ages ranged from 13–53 (mean 40.8) years. There were four men and two women who characteristically presented with syncope or dyspnoea. Most of the tumours arose in the right atrium where they formed pedunculated or polypoid masses. In the previously documented cases, all patients died within 9 months. However in our case, local excision was felt to have been complete and the patient remains disease free at 10 months.     

Synovial metaplasia of the skin

We present two female patients, aged 15 and 45 years, with synovial metaplasia of the skin. Both lesions, localized in the knee and hand respectively, arose after preceding local trauma. Case 1 had intradermal irregular cystic spaces in the adjacent myxoid stroma of which large polygonal eosinophilic cells were found. In contrast, case 2 was characterized by a longitudinal space within the lower dermis and subcutis which was lined by a membrane similar to hyperplastic synovium. The cells of the membrane showed an eosinophilic spindle shaped cytoplasm with processes towards the lumen. In both cases the eosinophilic cells, strongly suggestive of fibroblasts, showed staining for vimentin only, whereas no reactivity could be obtained with antibodies to actin, desmin, S-100 protein, Factor VIII related antigen, cytokeratin, epithelial membrane antigen, carcinoembryonic antigen and CD 68. The absence of CD 68 positivity differs from that seen in normal and hyperplastic synovium. Delayed wound healing around a nidus seems the most likely cause of the change.     

Synovial sarcoma arising in an anatomical bursa

Summary In previous studies, the origin of synovial sarcoma directly from synovium has not been satisfactorily established. This case report describes the light and electron microscopic features of a biphasic synovial sarcoma occurring within the popliteal fossa. At surgery, a cystic mass was identified in relationship to the semitendinosus tendon at the anatomical site of the semitendinosus bursa. The tumour originated from the inner surface of the bursa as multiple papillary projections with no evidence of extension beyond the capsule of the bursa. Portions of the synovial surface were hyperplastic but otherwise normal. The findings indicate that biphasic synovial sarcoma can arise directly from synovium and support the hypothesis of a mesenchymal histogenesis for this tumour.     

Primary sarcoma of the anterior cruciate ligament - a case report

Clear cell sarcoma of tendons and aponeuroses (CCSTA) is a rare, aggressive soft tissue malignancy, which is found in intimate association with tendon, aponeurosis or fascia. It has not previously been reported in association with intraarticular ligaments. We report the first case of an intraarticular CCSTA, in this case of the anterior cruciate ligament and describe the diagnostic and treatment challenges of intraarticular tumours of the knee.     

粒细胞肉瘤38例临床病理分析

目的 探讨粒细胞肉瘤的临床病理特征及鉴别诊断.方法 对38例粒细胞肉瘤患者的病理组织进行HE及免疫组织化学(EnVision法)染色观察,结合临床资料进行分析,并复习相关文献.结果 患者发病年龄2～77岁,平均年龄43.3岁,男23例,女15例.临床主要表现为体表淋巴结肿大、局部软组织肿块及疼痛.其中随访18例,死亡14例,平均生存时间16.9个月.组织学表现为肿瘤细胞弥漫成片,小～中等大小,形态较一致,胞质少而淡染,核呈圆形或不规则形,可见核仁,核分裂象易见.部分肿瘤具有"列兵样"组织结构,肿瘤细胞间可见散在分布的幼稚嗜酸性粒细胞.免疫组织化学染色显示瘤细胞表达抗髓过氧化物酶、CD43,部分表达CD68、溶菌酶、CD99和末端脱氧核苷酸转移酶,而CD3、CD20、CD79a、AE1/AE3和胎盘碱性磷酸酶阴性.结论 粒细胞肉瘤临床少见,形态学上易误诊为非霍奇金淋巴瘤、Ewing肉瘤/PNET、胚胎性横纹肌肉瘤等,免疫组织化学技术对于确诊本病具有重要价值.     

Clinicopathologic study of 24 patients with primary cardiac sarcomas: a 10-year single institution experience

Primary cardiac sarcomas are exceptionally rare. We present a 10-year, single-institution experience with 24 primary adult cardiac sarcomas. These cases were retrieved from the Department of Pathology data file of the Methodist Hospital at Houston, TX. Clinical presentation and pathologic features were analyzed. Histologic classification was followed according to the criteria set by the World Health Organization, and grading according to the system proposed by the Federation Nationale des Centres de Lutte Contrele Cancer. There were 14 men and 10 women (male/female, 1.4:1) with a mean age of 42.2 years (range 20-68 years). The tumors involved the right atrium in 14 cases, left atrium in 6 cases, right ventricle in 2 cases, and left ventricle in 2 cases. The tumor size ranged from 2.0 to 17.0 cm (mean 7.2 cm), and, histologically, there were 10 angiosarcomas, 9 unclassified sarcomas, 3 synovial sarcomas, and 2 leiomyosarcomas. All 10 angiosarcomas originated from the right atrium, whereas 5 of the unclassified sarcomas were from the left atrium. Although cases were limited, no predilection site was found for the other histologic types. All tumors were graded as 2 (5 cases) or 3 (19 cases) in differentiation. The prognosis was poor with a median survival time of 25 months after diagnosis. The grade was not statistically significant on survival (P = .14). In conclusion, angiosarcoma and unclassified sarcomas are the most common sarcomas of the heart accounting for 76%, but rare tumors such as synovial sarcoma and leiomyosarcoma may also occur in this organ. The survival of cardiac sarcomas is poor.     

Recurrence of synovial sarcoma of the abdomen in a 10-year old child

The case of a 10 year old child with a synovial sarcoma of the left iliac fossa, which recurred 12 months after simple large surgical excision, is reported here. No related lesions and, in particular, no lesion of the articular capsule of the left hip could be found, either on X-ray or at the time of the two excisions. Microscopically, the tumor showed, in all the areas examined, the existence of two characteristic morphological aspects, associating epithelial like type cells and elongated fusiform cells. These features correspond exactly to the classic "biphasic" type of synovial sarcoma. This location of a synovial sarcoma is an uncommon one, both in children and in adults.