Journal of Pathology and Translational Medicine

Case Reports

Primary Monophasic Synovial Sarcoma Arising in the Mesentery: Case Report of an Extremely Rare Mesenteric Sarcoma Confirmed by Molecular Detection of a SYT-SSX2 Fusion Transcript: Han Suk Ryu, Ilyeong Heo, Jae Soo Koh, Sung-Ho Jin, Hye Jin Kang, Soo Youn Cho; Korean J Pathol. 2012;46(2):187-191. Published online April 25, 2012; DOI: https://doi.org/10.4132/KoreanJPathol.2012.46.2.187

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Synovial sarcoma arises in the para-articular tissues, and it can also occur in various unexpected sites. We report a rare case of primary monophasic synovial sarcoma (MSS) arising in the mesentery. A 59-year-old man presented with a palpable abdominal mass. On microscopic examination, the entire tumor comprised a dense proliferation of the spindle cells without epithelial components. The tumor cells were positive for transducin-like enhancer of split 1, bcl-2, epithelial membrane antigen and CD99 but negative for CD34, CD117, alpha-smooth muscle actin, cytokeratin, and calretinin on immunohistochemistry. The reverse transcriptase-polymerase chain reaction revealed a single 151-bp fragment representing the SYT-SSX2 fusion transcript. Because mesenteric MSS is extremely rare and many cases display histologic findings that overlap with those of more frequently involved tumors such as hemangiopericytoma and gastrointestinal stromal tumor, there is a chance of making an incorrect diagnosis that can result in an inappropriate treatment.

Citations

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A case of primary mesenteric synovial sarcoma: a challenging presentation
Nihed Abdessayed, Malek Barka, Samiha Mabrouk, Zeineb Nfikha, Zeineb Maatoug, Yosra Fejji, Mohamed Salah Jarrar, Sabri Youssef, Moncef Mokni
Surgical Case Reports.2023;[Epub] CrossRef
Giant solitary fibrous tumor of the pelvis: A case report and review of literature
Gerardo Palmieri, Carmine Grassi, Luigi Conti, Filippo Banchini, Maria Diletta Daccò, Gaetano M. Cattaneo, Patrizio Capelli
International Journal of Surgery Case Reports.2020; 77: S52. CrossRef
Tumeur neuroectodermique gastro-intestinale (GNET) : à propos d’un cas de tumeur du grêle avec métastases hépatiques
Thibault Kervarrec, Claire Lecointre, Rémy Kerdraon, Guido Bens, Arnaud Piquard, Patrick Michenet
Annales de Pathologie.2015; 35(6): 506. CrossRef

Primary Synovial Sarcoma of the Kidney: A Case Report and Literature Review.: Mee Ja Park, Tae Hwa Baek, Joo Heon Kim, Dong Wook Kang, Hye Kyung Lee, Hyun Jin Son; Korean J Pathol. 2009;43(3):274-278.; DOI: https://doi.org/10.4132/KoreanJPathol.2009.43.3.274

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Abstract PDF

Synovial sarcoma is a rare renal neoplasm that is not easy to diagnose unless SYT-SSX fusion transcripts are identified. We report here on a case of primary renal synovial sarcoma in a 35-year-old woman. A mass was discovered by accident in the lower part of the right kidney when ultrasonography was performed, and it was removed via radical nephrectomy. Grossly, the tumor was a homogeneously tan-brown soft mass that measured 4.5x3.2x3.0 cm, and it was encircled by a well-defined cystic space. The lesion exhibited hypercellularity of the oval or short spindle cells that were arranged in various solid sheets or intersecting fascicles. Immunohistochemically, the tumor showed diffuse positivity for vimentin, bcl-2 and CD99, and it showed focal positivity for epithelial membrane antigen. The SYT-SSX fusion transcripts were detected by reverse transcription-polymerase chain reaction (RT-PCR). Synovial sarcoma should be considered in the differential diagnosis when a spindle cell neoplasm is encountered in the kidney.

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Primary Renal Synovial Sarcoma and Clinical and Pathological Findings: a Systematic Review
Leandro Blas, Javier Roberti
Current Urology Reports.2021;[Epub] CrossRef
Primary Renal Synovial Sarcoma - A rare histology
Premkumar Krishnappa, Mohan keshavamurthy, Shakir Tabrez, Sreeharsha Harinatha, Mohan Balaiah Aswathaiya
Urology Case Reports.2020; 33: 101402. CrossRef

Retroperitoneal Synovial Sarcoma: A case report.: Seoung Wan Chae, Jung Weon Shim, Hye Kyung Ahn, Min Chul Lee, Young Euy Park; Korean J Pathol. 1995;29(4):540-542.

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Abstract PDF: Synovial sarcoma most commonly affects the extremities, especially the lower thigh and knee region. However, a smaller number develops in a central or axial distribution, an area which encompasses the trunk, orofacial, cervical and parapharyngeal regions. The retroperitoneum is an extremely unusual site and has never been recorded in the literature as primary a site for synovial sarcoma. We investigated a case of retroperitoneal synovial sarcoma in a 40-year-old woman. The specimen consisted of fragmented large bulky multinodular masses separated by slit-like spaces. The tumor was 130 gm in weight. Microscopically, the tumor was composed of nests of plump ovoid to polygonal shaped cells and bundles of spindle shaped cells, which had vesicular nuclei and a small amount of cytoplasm. In some areas, there were cleft like spaces and pseudoglandular structures lined by flat or cuboidal cells. Myxoid change, collagen deposition, foci of calcification and osseous metaplasia were also present. Immunohistochemically, the polygonal cells and some of the spindle cells reacted positively for keratin. The spindle cells, especially in the perivascular area were positive for vimentin. S-100 protein and GFAP were negative in both type of cells.

Original Article

Calcifying Synovial Sarcoma.: Nam Bok Cho, Tae Jin Lee, Mi Kyung Kim, Yong Wook Park, Kye Yong Song; Korean J Pathol. 1995;29(4):536-539.

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Abstract PDF: A case of calcifying synovial sarcoma, occuring in the deep muscle fascia of the left thigh is reported. The presence of extensive calcification in synovial sarcoma is a favorable sign for prognosis. The patient was a 31-year-old female. The tumor mass had been present for 3 years, accompanying local tenderness. X-ray revealed a soft tissue tumor with central calcification, which was located between the adductor magnus and brevis of the left thigh. The tumor size was 7 x 5.5 cm. There was no connection with the knee joint or the femur. Grossly, the tumor was a relatively well circumscribed hard tumor with massive calcification. Microscopically, the tumor was composed of predominantly spindle cells with accompanying hyalinization, numerous spherical concretions and ossification. The epithelial component was not clearly noted. Mitotic figures were rarely noted in the densely cellular area. Immunohistochemical staining for EMA, S-100, vimentin, and carcinoembryonic antigen was negative while vimentin and cytokeratin were weakly and focally positive. EM study revealed multiple desmosome-like calcification intercellular junctions with a slit-like lumen and an incomplete basal lamina, which suggest that tumor show these cells were undergoing epithelia] differentiation. Above evidence suggest that this tumor is a synovial sarcoma associated with a large area of calcification, a so called calcifying synovial sarcoma. No recurrence was noted in one and half years of follow up.

Case Reports

Clinical Pathology, Catholic University Medical CollegeSynovial Sarcoma Manifested as a Subcutaneous Nodule of Lip: A case report.: Jae Hwa Lee, Young Ok Kim, Bang Hur, Man Ha Hur; Korean J Pathol. 1996;30(9):868-871.

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Abstract PDF: Synovial sarcoma is one of the most common malignant soft tissue tumors in paraarticular regions. However, it is very rare to occur without any apparent relationship to synovial or articular structures. We report a case of synovial sarcoma manifested as a subcutaneous nodule in the upper lip. A 12-year-old boy complained of a palpable tender mass in the upper lip for several months. Under the impression of a benign soft tissue neoplasm, surgical excision was done. The mass, 1.5 cm in maximum diameter, was easily separated from surrounding tissues. Microscopically, this tumor showed a characteristic biphasic pattern of synovial sarcoma. On immunohistochemical and ultrastructural studies, the tumor cells showed evidence of epithelial differentiation. There was no evidence of joint-related, primary lesions or metastatic foci. This report describes a case of synovial sarcoma arising in very unusual location and size for histologically typical synovial sarcoma.

Imprint Cytologic Features of Poorly Differentiated Synovial Sarcoma: A Case Report.: Mi Jin Gu, Joon Hyuk Choi, Young Kyung Bae; Korean J Cytopathol. 2003;14(1):27-31.

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Abstract PDF: Poorly differentiated synovial sarcoma is a variant of synovial sarcoma. We report a case of poorly differentiated synovial sarcoma imprinted after resection. The patient was a 47-year-old woman with a right shoulder pain for 6 months. The cytologic features showed malignant round to oval, monotonous tumor cells with high nuclear to cytoplasmic ratio. Some tumor cells showed perivascular distribution and nuclear molding. Vague rosette-like structures were seen. On immunohistchemical stains, tumor cells were diffusely positive for CD99 and focally positive for epithelial membrane antigen. Ultrastructural examination showed desmosomes and microvilli.

Synovial Sarcoma with Massive Myxoid Feature: A Case Report.: Joon Hyuk Choi, Young Ran Shim, Young Kyung Bae, Mi Jin Kim, Duk Seop Shin, Kil Ho Cho; Korean J Pathol. 2005;39(4):273-277.

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Abstract PDF: Focal myxoid change in synovial sarcoma is not uncommon, although the presence of predominantly myxoid stroma is very rare. Recognition of synovial sarcomas with massive myxoid feature is important because these can easily be mistaken for other myxoid soft tissue neoplasms. We report a case of a synovial sarcoma with massive myxoid feature in the left thigh of a 54-year-old woman. Wide excision of an 8.5*7.0*5.0 cm, well-circumscribed and lobulated tumor was performed. The cut surface was gray, soft, and myxoid. Histological examination showed proliferation of spindle cells in the predominantly myxoid stroma. There were small areas with features more typical of synovial sarcoma, including uniform, spindled cells with fascicular growth patterns, collagenous stroma, mast cell infiltration, and hemangiopericytoma-like vascular patterns. Immunohistochemical examination showed focal positivity of the tumor cells for epithelial membrane antigen (EMA). Tumor cells were all negative for cytokeratin (AE1/AE3), cytokeratin 7, S-100 protein, smooth muscle actin, and desmin. Ultrastructurally, tumor cells showed desmosomes and microvilli. Our case underscores that, in order to make a correct diagnosis, immunohistochemical and ultrastructural examination is essential.

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