Monophasic synovial sarcoma presenting as a primary ileal. Synovial sarcoma is an uncommon soft tissue neoplasm, whose origin is. The pathological variants of synovial sarcoma include biphasic, monophasic epithelial, monophasic fibrous and poorly differentiated types. Synovial sarcoma ss is a mesenchymal spindle cell tumour which displays variable epithelial differentiation, including glandular formation and has a specific chromosomal translocation tx.
Synovial sarcoma explained by a soft tissue pathologist. Management of monophasic synovial sarcoma of the small. They are termed ss because of their histologic resemblance to the synovium, but they rarely involve a. Monophasic pattern of synovial sarcoma is characterized by monomorphic population of spindle shaped cells arranged in fascicles. Department of pathology, the first affiliated hospital of sun yatsen.
Consideration is given to the concept of a histologically identifiable monophasic type of synovial sarcoma. Synovial sarcoma is a rare entity representing 5% to 10% of all soft tissue sarcomas, typically occurring around the joints, mainly the knee 2, 3. Synovial sarcoma is one of the most common soft tissue malignancies of adolescents and young adults. Monophasic synovial sarcoma as a cause of obstructive. The spindle cells were immunoreactive for oscar, ema, bcl2, vimentin, cd99, and pgp 9. E cadherin in the spindle cells of monophasic fibrous synovial sarcomas.
Webpathology is a free educational resource with 10304 high quality pathology images of benign and malignant neoplasms and related entities. All neoplastic cells were positive for vimentin, and approximately 5% of them. Mucinous, gland predominant synovial sarcoma of a large peripheral nerve. Abdominal monophasic synovial sarcoma is a morphological. A histopathological examination showed a monophasic synovial sarcoma. Primary pulmonary sarcoma with morphologic features of. Despite its name, ss does not appear to be of synovial origin, but rather from.
The differential diagnosis includes malignant peripheral nerve sheath tumors, musclederived sarcomas, and biphasic mesotheliomas. Synovial sarcoma, a rare tumor in the head and neck, has been historically diagnosed by its characteristic biphasic histologic pattern. It can occur at any age, but it is more common among teenagers and young adults. Primary renal sarcoma with morphologic and immunohistochemical aspects compatible with synovial sarcoma. A clinicopathological, immunohistochemical, and ultrastructural study of 25 cases handan zeren, md, cesar a. This video discusses the pathologic features of monophasic and biphasic synovial sarcoma including immunostains, molecular testing, the major differential diagnoses, and potential diagnostic pitfalls. It affects soft tissues, which connect, support, and surround bones and organs in your body.
Ultrastructural evidence for a common histogenesis, despite light microscopic diversity histopathology, 6 1982, pp. The tumor was composed of closely packed spindle cells with scant pale cytoplasm forming variably arranged fascicles fig. Monophasic synovial sarcoma represents 50%60% the most common subtype of all lesions, and in this subtype the. Synovial sarcoma, monophasic fibrous and poorly differentiated. Monophasic synovial sarcoma as a cause of obstructive ileus. Primary monophasic synovial sarcoma of the colon j r parfitt department of pathology, london health sciences centre and university of western ontario, london, department of pathology, sudbury regional hospital, northern ontario school of medicine, sudbury.
A synovial sarcoma ss is an aggressive soft tissue tumor, which mainly occurs in the paraarticular region of extremities with a predilection of lower limb. Synovial cell sarcoma scs is reported to be the most common joint tumor of dogs. A clinicopathological, immunohistochemical, and ultrastructural study of 25 cases handan zeren, cesar a. Synovial sarcoma genetic and rare diseases information. Sep 10, 2015 synovial sarcoma is a rare and aggressive soft tissue sarcoma. In the early stages of the condition, it may cause no noticeable signs or symptoms. Synovial sarcoma seems to have a slight preference for males, with 12 male patients for every 10. Primary pulmonary sarcomas with features of monophasic. Synovial sarcoma is the fourth most commonly occurring sarcoma, accounting for 810% of all sarcomas. N a c s wong, department of histopathology, bristol royal infirmary, marlborough.
Printable synovial sarcoma surgical pathology criteria. More than 90% of synovial sarcomas, regardless of histological type, bear the x. Synovial sarcoma is a mesenchymal spindle cell tumor which displays variable epithelial. Abdominal monophasic synovial sarcoma is a morphological and immunohistochemical mimic of gastrointestinal stromal tumour. Synovial sarcomas are a rare form of soft tissue sarcomas. We report the case of a 29yearold saudi woman of arabian ethnicity with synovial sarcoma arising primarily from the ileum who presented with abdominal pain, a palpable mass and incomplete intestinal obstruction. It is a relatively rare tumor, occurs mainly in adolescents and young adults, has a slight male predominance, and comprises approximately 10 % of all soft tissue sarcomas. Role of immunophenotyping in the differential diagnosis of synovial sarcoma.
The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of tx. This tumor had histologic, immunophenotypic, and ultrastructural features consistent with monophasic fibrous synovial sarcoma. Department of pathology, childrens hospital and health sciences. Primary monophasic synovial sarcoma of the colon parfitt. Synovial sarcoma is a soft tissue tumor occurring in the biphasic or monophasic type and consisting of epithelial andor spindle cell components.
This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Although this tumor generally affects adults, about 30% of reported cases occur in children and adolescents. Mar 31, 2009 synovial sarcomas are a rare form of soft tissue sarcomas. A confident distinction between abdominal synovial sarcoma and gist requires kit pdgfra mutation analyses and specific molecular testing for synovial sarcoma. Prognosis is better for synovial sarcoma associated with osseous metaplasia or extensive calcification. Monomorphic spindle cell sarcoma with or without areas of epithelial differentiation monophasic or biphasic, respectively accounts for. They are termed ss because of their histologic resemblance to the synovium, but they rarely involve a synovial. Primary poorly differentiated monophasic synovial sarcoma of ileum.
Monophasic synovial sarcoma, epithelioid sarcoma and chordoid sarcoma. Ossifying synovial sarcomas have characteristic spotty radioopacities caused by focal calcifications pathol res pract 2009. A diagnosis of sarcoma was made with synovial cell sarcoma scs and histiocytic sarcoma being the 2 main differentials. Synovial sarcoma is a rare and aggressive soft tissue sarcoma. Histopathology confirmed the diagnosis of sarcoma and provided the same differentials. Synovial sarcoma is usually an aggressive deep seated mass presenting around large joints 80% in knee and ankle in young adults. The majority occur in the deep soft tissue, near large joints of the extremities in young adults. Mar 16, 2020 webpathology is a free educational resource with 10304 high quality pathology images of benign and malignant neoplasms and related entities. The cells contain uniform tapering nuclei and pale cytoplasm. Malignant peripheral nerve sheath tumor fibrosarcoma.
Further histopathologic analysis revealed a monophasic synovial sarcoma see histopathology below. In some cases, the tumor can limit range of motion or cause numbness andor pain if it presses on nearby nerves. Koss, md we present 25 cases of a primary pulmonary sarcoma bearing histological, immunohistochemical, and ultrastructural features. Monophasic synovial sarcoma of the liver amitabh srivastava, mbbs, petur g. Introduction synovial sarcoma ss is a so tissue sarcoma of uncertain histogenesis, chie y occurring in young adults, primarily in the paraarticular region of the extremities. Primary pulmonary sarcomas with features of monophasic synovial sarcoma.
Pdf primary monophasic synovial sarcoma of the kidney. The reported incidence of synovial sarcomas ranges from 5. For synovial sarcoma of the head and neck region, the prognosis is better. Synovial sarcoma is a malignant mesenchymal tumor of uncertain histogenesis.
One fibrous type, known as a spindle or sarcomatous cell, is relatively small and uniform, and found in sheets. Synovial sarcomas present typically as a mass lesion in the extremities. Synovial sarcoma has a fusion gene, sytssx, which is the result of a chromosomal translocation unique for this tumor, tx. An additional 6 cases, with a fiveyear survival of 40%, were contrib uted by hajdu et al. However, as the tumor grows larger, affected people may notice a lump or swelling. Surgery forms the main mode of treatment followed by adjuvant radiotherapy. Rarer superficial cases present in the skin of hands and feet as smaller tumours, which have a more favourable prognosis histology of synovial sarcoma. Immunohistochemistry augments the conventional histopathology and offers a definitive diagnosis. Synovial sarcoma ss is a rare and aggressive soft tissue tumor, which accounts for 78 % of all human malignant sarcomas. Tle1 expression is not specific for synovial sarcoma. It may be biphasic, monophasic, or poorly differentiated. After rhabdomyosarcoma, synovial sarcoma ss is the most common type of soft tissue sarcoma sts in childhood and adolescence, with a predilection for young adults.
Synovial sarcoma ss is a rare malignant neoplasm that arises most commonly in joint capsules and articular tendons, but its relationship to the synovium is not always obvious. Monophasic epithelial variant of synovial sarcoma extremely rare. These may include muscle, fat, blood or lymph vessels. Department of histopathology, bristol royal infirmary, bristol, uk.
Poorly differentiated pd variant of synovial sarcoma. We present a case of a 62 yearold male presenting with a left thyroid lump initially though to be a thyroid adenoma but subsequently diagnosed as a monophasic synovial sarcoma of the pharynx. The authors report an unusual spindle cell sarcoma that arose in the lung of a 12yearold girl. Dec 22, 2006 primary monophasic synovial sarcoma of the colon j r parfitt department of pathology, london health sciences centre and university of western ontario, london, department of pathology, sudbury regional hospital, northern ontario school of medicine, sudbury. The glandular component may predominate and occasionally obscure the spindle cell elements, an appearance suggestive of adenocarcinoma and that has been referred to as the purely glandular type monophasic synovial sarcoma. Primary monophasic synovial sarcoma of the brachial plexus. Synovial sarcoma whether monophasic or biphasic stains positive for vimentin and epithelial makers such as cytokeratin and epithelial membrane antigen ema. A study of monophasic fibrous and poorly differentiated synovial sarcomas, with the diagnosis established by the presence of the translocations tx. Management of monophasic synovial sarcoma of the small intestine. Research open access primary synovial sarcoma ss of the. Most sss reported in the pleural cavity have been biphasic, whereas in the lung most of the tumors have been monophasic 1, 2. Monophasic variants exist which can be difficult to diagnose. Mar, 2012 synovial sarcoma is a rare malignant mesenchymal tumor mainly arising in the periarticular tissue in young adults.
Synovial sarcoma sss consist of biphasic and monophasic types figure 12. I, 151157 monophasic synovial sarcomaa histological entity. Histologically, tumors were monophasic n 16 or biphasic n 3. 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. Pdf poorly differentiated synovial sarcoma is a rare soft tissue tumor.
Synovial sarcoma ss is a malignant neoplasm that arises from the primitive pluripotent mesenchymal cells near to or remote from the articular surfaces. Intrathoracic synovial sarcomas show a high proportion that are poorly differentiated immunohistochemistry. Approximately 800 new cases of synovial sarcoma are diagnosed per year in the united states. Monophasic synovial sarcoma of the liver archives of. Despite its name, it is no longer thought to be histogenetically derived from the synovium. Monophasic synovial sarcoma, epithelioid sarcoma and chordoid. Two cell types can be seen microscopically in synovial sarcoma. Synovial sarcomapowerpoint presentation new authorstream. Synovial sarcoma is a malignant soft tissue tumor representing 5. It usually develops in adolescents and young adults between the age of 15 and 40 years 1 3. Mackenzie westminster hospital medical school, london s wi accepted for publication 5 november 1976 mackenzie 1977 histopathology d. Primary intraabdominal synovial sarcomas are rare soft tissue.
A rare case of monophasic synovial sarcoma of pharynx of a young boy is reported here. Poorly differentiated areas of synovial sarcoma and some monophasic cases may be difficult to impossible to distinguish from tumors such as. The histopathology in synovial sarcoma may be biphasic consisting of spindled and epithelioid cells, monophasic only spindled cells, or undifferentiated difficult to recognise without cytogenetic tests. Synovial sarcoma of the carpal tunnel monophasic fibrous type. We discuss the diagnosis and treatment of this case. Occasionally, it arises in the head and neck 4, 5, lungs 68, heart, retroperitoneum, prostate. Imaging of synovial sarcoma with radiologicpathologic. Synovial sarcoma symptoms, causes, diagnosis, treatment. The extremities are the commonest site of occurrence. Case report monophasic synovial sarcoma of prostatic. The diagnosis and prognosis of synovial tumors in dogs. The histopathology in synovial sarcoma may be biphasic consisting of spindled and epithelioid. Only about 1 to 3 individuals in a million people are diagnosed with this disease each year. In the authors experience, it is a soft tissue sarcoma that presents as a painful mass more commonly than other sarcomas.
An extremely rare case of obstructive ileus due to monophasic synovial sarcoma in a 54. Synovial sarcoma accounts for 510% of soft tissue sarcomas. Synovial sarcomas are rare, unique spindle cell tumors and account for approximately 10% of all soft tissue tumors. Rosenberg, md from the departments of pathology, tuftsnew england medical center dr srivastava, james homer wright laboratories, massachusetts general hospital drs nielsen and rosenberg, and division of. Identification of poorly differentiated synovial sarcoma.