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Primary synovial sarcoma of the parapharyngealspace a clinicopathologic study of five cases


, : Primary synovial sarcoma of the parapharyngealspace a clinicopathologic study of five cases.

We report five cases of primary synovial sarcomas arising in the parapharyngeal space. Thepatients were all men with a median age of 35 years (range 22 to 41 years). The tumors werenon-encapsulated solid masses ranging from 2.0 to 6.6 cm in size. Histologically, three caseswere biphasic subtype, and the other two cases were monophasic subtype.Immunohistochemically, the tumor cells were strongly positive for bcl-2 and CD99, partlypositive for CK and EMA, and negative for CD117, CD34, SMA and desmin in all five cases.S-100 protein was detected in one case. The presence of an SYT-SSX1 and/or SYT-SSX2 genefusion resulting from t(X;18) was demonstrated from paraffin blocks by reverse transcriptasepolymerase chain reaction in five cases. All five patients received tumor radical excision andpostoperative radiotherapy, and two patients with pulmonary metastasis received additionalchemotherapy. Follow-up data revealed that two patients with tumor size <5 cm were alivewithout disease for 54 and 57 months, one patient with tumor size <5 cm was alive withpulmonary metastasis for 78 months, and two patients with tumor size >5 cm died of disease26 and 37 months after the diagnosis, respectively. Primary parapharyngeal synovial sarcomais a rare variant that occurs more frequently in males than females. Accurate diagnosisdepends on morphologic and immunohistochemical examination and proper molecularanalysis. The prognosis is relatively good in those patients whose tumor size is less than5 cm.

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