Tle1 expression is not specific for synovial sarcoma. The biphasic type contains epithelial and spindle cell elements, which occur in varying proportions. The differential diagnosis includes malignant peripheral nerve sheath tumors, musclederived sarcomas, and biphasic mesotheliomas. Materials and methods the radiologic and pathologic databases at our institution were searched for cases of synovial sarcoma before beginning our study. Epithelioid cells were present only in biphasic ss. D, histopathology shows a monophasic synovial sarcoma with. In the head and neck region, synovial sarcomas ss are rare tumours.
Definitiondiagnostic criteria ss is a soft tissue sarcoma usually divided into three subtypes. It can also form in soft tissues in the lung or abdomen. Rarer superficial cases present in the skin of hands and feet as smaller tumours, which have a more favourable prognosis histology of synovial sarcoma. The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of tx. Sep 01, 2019 monophasic epithelial synovial sarcoma is extremely rare, and its existence has been debated. Since there are only 8 cases described in the literature, the best therapeutic approach and the prognosis still remain to be clarified. Pdf synovial sarcoma is a unique tumor with substantial promise. Monophasic and biphasic synovial sarcoma tsujimoto. Poorly differentiated pd variant of synovial sarcoma. A subsequent molecular analysis revealed sytssx2 gene fusion, which confirmed the diagnosis of synovial sarcoma. A 46yearold man was admitted to hospital due to progressive dyspnoea and a painless axillary mass that he had had for several months. Tracheal synovial sarcoma ss is even an extremely rare entity.
Printable synovial sarcoma surgical pathology criteria. Department of histopathology, armed forces institute of pathology, rawalpindi, pakistan. Synovial sarcomas sss are soft tissue tumors that occur primarily in adolescents and. Imaging of synovial sarcoma with radiologicpathologic correlation. The reported incidence of synovial sarcomas ranges from 5. Synovial sarcoma orbital and nonorbital location presents a 5year survival rate around 80% but decreases dramatically to 30% if relapse occurs. When the biphasic aspect is totally absent, and the sarcoma is exclusively made up of fibroblastic cells b, or epithelioid cells a, diagnosis is difficult and doubtful monophasic s. Synovial sarcoma is an uncommon soft tissue neoplasm, whose origin is. Clinically, the second pelvic mass was considered recurrent and excised. Author reference patient age sex histopathology molecular studies. The tumors were all subclassified as monopha sic fibrous or biphasic synovial sarcoma, and evaluated for the presence of poorly differentiated areas, tumor necrosis, vascular invasion, type of tumor interface with normal tissues, mitotic activ. Synovial sarcomas are malignant mesenchymal tumours that commonly arise in the limbs1 and sometimes in other internal organs. We report a case of monophasic epithelial synovial sarcoma, which was initially diagnosed as metastatic adenocarcinoma. Monophasic epithelial synovial sarcoma initially diagnosed as.
Mar 31, 2009 synovial sarcomas are a rare form of soft tissue sarcomas. Consideration is given to the concept of a histologically identifiable monophasic type of synovial sarcoma. Synovial sarcoma is usually an aggressive deep seated mass presenting around large joints 80% in knee and ankle in young adults. One fibrous type, known as a spindle or sarcomatous cell, is relatively small and uniform, and found in sheets. Synovial sarcoma identification of favorable and unfavorable. A diagnosis of synovial sarcoma should be considered particularly if an abdominal spindle cell neoplasm shows a haemangiopericytomatous pattern and diffuse cd 99 and cd 56 immunopositivity. Types of ss include monophasic, biphasic and poorly differentiated type. Gastric ulcer that turned out to be metastasis of a. Usefulness of cytokeratin subsets for distinguishing monophasic. Jan 10, 2020 background the incidence of primary tracheal tumors is very low. It is a sarcoma whose cells mimic the two types of cells a and b of the synovial membrane. Primary synovial sarcoma pss is a mesenchymal tumour seen mainly in adolescents and young adults and usually arises from periarticular tissue.
The typical synovial sarcoma has a biphasic growth pattern and commonly occurs near joints thus it was mistakenly deemed as a tumour of synovial differentiation. Author reference patient age sex histopathology molecular st. Learn more about the tumor, diagnosis, and prognosis from the mypart project. Synovial sarcomas are very rare tumours, and primary location is an exceptional finding. Calcifying ss is another subtype, which has extensive calcification and carries a much better prognosis. Jul 01, 2018 high expression of ezh2 helps to distinguish poorly differentiated synovial sarcoma from monophasic and biphasic subtypes j transl med 2012. Ecadherin in the spindle cells of monophasic fibrous synovial sarcomas. Monophasic epithelial synovial sarcoma initially diagnosed.
Abdominal monophasic synovial sarcoma is a morphological and immunohistochemical mimic of gastrointestinal stromal tumour. They are termed ss because of their histologic resemblance to the synovium, but they rarely involve a. 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. Immunohistochemistry showed bcl2, focal cd99 and high molecular weight cytokeratin positivity, while smooth muscle actin, s100 and cd34 immunostainings were negative. Radiotherapy was not given because of a risk of intestinal wall perforation. Two cell types can be seen microscopically in synovial sarcoma. Case presentation we report a case of tracheal ss, which presented with stridor with a history of. Pathology, helsinki, finland, %outhern swedish tumor registry, lund university. Therefore, these soft tissue fragments were sent for histopathological evaluation. Primary synovial sarcoma of tongue is very uncommon. The histopathology in synovial sarcoma may be biphasic consisting of spindled and epithelioid. A 45yearold woman presented with a tender pelvic mass, whose morphology was consistent with a glandular neoplasm. Histology revealed a spindle cell tumour with mixed cellularity, focal myxoid. Synovial sarcoma may also be called malignant synovioma.
Synovial sarcoma ss is a relatively common sarcoma of soft tissue arising in extremities of young adult, which is often diagnosed by histology and immunohistochemistry ihc. Jan 03, 2019 monomorphic spindle cell sarcoma with or without areas of epithelial differentiation monophasic or biphasic, respectively accounts for. Monophasic epithelial variant of synovial sarcoma extremely rare. We present the case of a 46yearold patient with a late diagnosis of a primary thoracic monophasic synovial sarcoma. Slender spindle cells and plump cells were constant components of all the tumors.
Primary monophasic synovial sarcoma of the pancreas. Synovial sarcoma is a soft tissue tumor occurring in the biphasic or monophasic type and consisting of epithelial andor spindle cell components. Abdominal monophasic synovial sarcoma is a morphological and. 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 histology of the lesion was that of a monophasic synovial sarcoma with a hemangiopericytic vascular pattern. Media in category histopathology of sarcomas the following 7 files are in this category, out of 7 total. Abdominal monophasic synovial sarcoma is a morphological. Synovial sarcoma ss of the spine is a rare malignant softtissue tumor, and there are few reported cases.
Epithelial differentiation in synovial sarcoma correlation. A confident distinction between abdominal synovial sarcoma and gist requires kit pdgfra mutation analyses and specific molecular testing for synovial sarcoma. Histologic section of the monophasic synovial sarcoma. Oral synovial sarcoma oral surgery, oral medicine, oral pathology. At the age of 20 years the patient developed two neoplastic lesions in the surgical scar measuring 10 mm and 45. Diagnosis of tracheal ss can be achieved with chromosomal translocation studies along with immunohistochemistry. Synovial sarcomas of the head and neck american journal of. May 30, 2018 synovial sarcoma is a rare tumour of head and neck. It is accepted that a sarcomatous tumour may be encountered where the spindle cells assume a somewhat epithelioid appearance associated. Original articles molecular diagnosis of synovial sarcoma. Synovial sarcomaidentification of favorable and unfavorable.
Monophasic synovial sarcoma, epithelioid sarcoma and chordoid. Synovial sarcoma can be found in various locations and may cause diagnostic dilemma in. Primary pulmonary sarcomas with features of monophasic synovial sarcoma. A dinocop athological, immunohistochemical, and ultrastructural study of 25 cases. Approximately 6080% of all ss patients are young adults and adolescents, with a higher prevalence in men. Primary monophasic synovial sarcoma of the pancreas request pdf.
Synovial sarcoma international journal of contemporary medical. Histology confirmed residual monophasic synovial sarcoma. Unusual histology in primary pulmonary synovial sarcoma. Diagnostic approach to synovial sarcoma of the head and neck. Synovial sarcoma is a tumor whose orbital location is rare. Synovial sarcoma ss is a rare sarcoma driven by a translocation betweenss18 and ssx 1, 2, or 4. An additional 6 cases, with a fiveyear survival of 40%, were contrib uted by hajdu et al. Rarer superficial cases present in the skin of hands and feet as smaller tumours, which have a more favourable prognosis. We discuss the diagnosis and treatment of this case. One third of patients with synovial sarcoma will be diagnosed under the age of 30. Synovial sarcoma is a malignant soft tissue tumor representing 5. Synovial sarcoma can be found in various locations and may cause diagnostic dilemma in tumors arising in unusual locations. Can occur in or around any tissue in the body multiple morphologies, including monophasic spindle cell, biphasic, poorly differentiated, myxoid.
In this article we present a patient with monophasic synovial sarcoma in which the use of these new. Becose of this reoperation was not possible, curative six cycles of doxorubicine and ifosfamide with granulocyte colony stimulating. R0 resection, partial rib resection and abdominal wall reconstruction were performed. Synovial sarcoma is highly unusual among mesenchymal neoplasms because it has variable degrees of epithelial differentiation. May 07, 2018 primary pulmonary synovial sarcoma is an aggressive tumor sharing common histologic features with soft tissue synovial sarcoma 15. Ss can occur anywhere in the body, including the brain, prostate, and heart.
Synovial sarcoma ss is a rare malignant softtissue tumor accounting for 510% of all soft tissue tumors. View the article pdf and any associated supplements and figures for a period of 48 hours. Mar 05, 2021 synovial sarcoma is a malignant softtissue neoplasm that most commonly affects the extremities near to, but not in continuity with, large joints. After an initial wide local excision, the patient developed a recurrence in the right groin and received chemotherapy. We report a case of synovial sarcoma of the pancreas in a 44yearold male who presented with multiple episodes of retroperitoneal hemorrhage. 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 and biphasic synovial sarcoma tsujimoto 1987. Gastric ulcer that turned out to be metastasis of a synovial. A monophasic synovial sarcoma with prominent spindle cell. Monophasic synovial sarcoma, epithelioid sarcoma and. Mucinous, gland predominant synovial sarcoma of a large peripheral nerve.
Based on morphology, it can be classified as classical biphasic variant, monophasic spindle cell variant, monophasic epithelial variant and poorly differentiated variant. The tumor is mainly composed of spindled component which is characterized by elongated cells with ovoid palestaining nuclei, inconspicuous nucleoli, and scanty cytoplasm hematoxylineosin, original magnification. Synovial sarcomas are very rare tumours, and primary location is an exceptional. Original article primary poorly differentiated monophasic synovial.
Remarkable good therapeutic results were reported by cade 1962 in a presentation of 100 cases of synovial sarcoma at the westminster hospital, london, arising from 1950 to 1960. Department of pathology, the first affiliated hospital of sun yatsen university. Synovial sarcomas are malignant soft tissue tumors that most often arise in the extremities of young adults. Synovial sarcoma is a cancer that can come from different types of soft tissue, such as muscle or ligaments. As it is made up of two cellular types, it is called biphasic. Synovial sarcoma ss is a soft tissue tumor with high malignant potential.
Monophasic synovial sarcoma, epithelioid sarcoma and chordoid sarcoma. Diagnosis of synovial sarcoma of the pleura and differentiation. Synovial sarcoma with only spindle cells or purely glandular component is called monophasic synovial sarcoma. Of the 10 tumors, 9 showed classic histologic features of monophasic synovial sarcoma, including a fascicular proliferation of monomorphic. Histopathology synovial sarcoma has the unusual property among soft tissue tumours of displaying epithelial differentiation. Marginfree resection is the gold standard treatment. Synovial sarcoma ss is a malignant mesenchymal tumor of uncertain origin. Primary synovial sarcoma of the orbit sage journals. Seven cases of synovial sarcoma ss, two with biphasic and five with monophasic histology, were studied immunohistochemically using monoclonal antibodies to intermediate filaments, keratin, and vlmentin. High expression of ezh2 helps to distinguish poorly differentiated synovial sarcoma from monophasic and biphasic subtypes j transl med 2012. 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. It is accepted that a sarcomatous tumour may be encountered where the spindle cells assume a somewhat epithelioid appearance associated with a reticulin patte. It is often found in the arm, leg, or foot, and near joints such as the wrist or ankle.
A diagnosis of synovial sarcoma should be considered particularly if an abdominal spindle cell neoplasm shows a. Monophasic synovial sarcoma presenting as a primary ileal. Epithelial cells formed well demarcated glandular nests with a multi or. C, after gadolinium injection, inhomogeneous enhancement is seen within the tumor arrows. They are termed ss because of their histologic resemblance to the synovium, but they rarely involve a synovial. More than 90% of the ss have characteristic translocation i. The recurrent tumor showed biphasic histology with glandular and spindle sarcomatoid.
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