Primary pancreatic synovial sarcoma: Report of a rare case and review of the literature.

Rebaz M Ali, Karzan Seerwan, Shano M Ali, Ari M Abdullah, Omar Hawrami, Dlsoz M Hussein, Bruj Jamil Mohammed, Muhammad Karim, Fakher Abdullah, Berun A Abdalla, Fahmi H Kakamad
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Abstract

Synovial sarcoma is a mesenchymal spindle cell tumor. Primary pancreatic sarcomas are extremely rare. The present study describes a rare case of synovial sarcoma in the head of the pancreas. A 35-year-old male presented with left upper quadrant abdominal pain. An endoscopic ultrasound examination revealed a complex solid-cystic lesion in the pancreatic head. He had undergone pancreaticoduodenectomy (Whipple procedure). A histological examination yielded negative results for AE1/AE3, CD10, S100, CD34, desmin, smooth muscle actin, β-catenin, CD117, HMB45, chromogranin and synaptophysin. However, the results were positive for TLEI and vimentin, which is consistent with synovial sarcoma. Synovial sarcoma is a soft tissue malignant tumor. Primary pancreatic sarcomas frequently present as large, high-grade tumors in the pancreatic head. Histologically, there are several types of synovial sarcoma, such as monophasic, biphasic and poorly differentiated. A histological examination is necessary for the diagnosis as the imaging findings are not specifically suggestive of synovial sarcoma. The preferred course of treatment is complete resection with wide margins, followed by adjuvant chemotherapy and/or radiotherapy. Primary mesenchymal tumors of the pancreas are extremely uncommon. As a result, a diagnosis requires careful evaluation. Surgical resection is the main modality of treatment.

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原发性胰腺滑膜肉瘤1例报告及文献复习。
滑膜肉瘤是一种间充质梭形细胞肿瘤。原发性胰腺肉瘤极为罕见。本研究报告一例罕见的胰脏头部滑膜肉瘤。35岁男性,左上腹腹痛。超声内镜检查显示胰腺头部有复杂的实性囊性病变。患者行胰十二指肠切除术(Whipple手术)。组织学检查AE1/AE3、CD10、S100、CD34、desmin、平滑肌肌动蛋白、β-连环蛋白、CD117、HMB45、嗜铬粒蛋白和突触素均为阴性。然而,结果显示TLEI和vimentin阳性,这与滑膜肉瘤一致。滑膜肉瘤是一种软组织恶性肿瘤。原发性胰腺肉瘤通常表现为胰腺头部的大而高级别肿瘤。组织学上,滑膜肉瘤有几种类型,如单相、双相和低分化。组织学检查对于诊断是必要的,因为影像学表现不能明确提示滑膜肉瘤。首选的治疗方案是大切缘完全切除,然后辅助化疗和/或放疗。原发性胰腺间充质瘤极为罕见。因此,诊断需要仔细的评估。手术切除是治疗的主要方式。
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