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[精索原发性滑膜肉瘤根治性切除术后7年出现腹腔内复发伴出血及局部播散:1例报告]

[Intra-abdominal recurrence with bleeding 7 years after curative resection of primary synovial sarcoma of the spermatic cord with localized dissemination:a case report].

作者信息

Shimojima Yukio, Hirose Yuki, Takano Kabuto, Nomura Tatsuya, Ando Takuya, Kawasaki Takashi, Banba Takeo, Nogami Hitoshi, Takii Yasumasa, Yabusaki Hiroshi, Nakagawa Satoru

机构信息

Department of Digestive Surgery, Niigata Cancer Center Hospital.

Division of Digestive and General Surgery, Niigata University Graduate School of Medical and Dental Sciences.

出版信息

Nihon Shokakibyo Gakkai Zasshi. 2021;118(12):1130-1136. doi: 10.11405/nisshoshi.118.1130.

Abstract

Primary synovial sarcoma of the spermatic cord is quite rare and has not been reported in Japanese literature. We report a case of primary synovial sarcoma of the spermatic cord and localized dissemination of the tumor in a patient who experienced recurrence of intra-abdominal bleeding 7 years after curative resection of the primary lesion. A 70-year-old man was admitted with disturbance on urination and lower abdominal pain. Computed tomography (CT) of the abdomen revealed two lesions:a 10-cm intrapelvic tumor with hemorrhage and a 4-cm tumor adjacent to the bladder. Curative excision of the tumors was performed. Histological examination revealed that the larger lesion was a primary tumor of the spermatic cord with proliferation of spindle cells in cellular fascicles in a monotonous pattern, which was compatible with histologic findings of monophasic fibrous synovial sarcoma. The smaller lesion was a disseminated tumor. The diagnosis of synovial sarcoma was confirmed by the detection of a SS18 (SYT) -SSX1 fusion gene. After discharge, the patient received adjuvant chemotherapy, including ifosfamide and doxorubicin. No recurrence was evident thereafter. Seven years after the operation, the patient experienced sudden abdominal pain and swelling and was transferred to our hospital. CT showed a 17-cm tumor with massive hemorrhage in the omental bursa. Through catheterization of the superior mesenteric artery, bleeding from a branch of the dorsal pancreatic artery was identified. Because of the difficulty of catheterizing the bleeding branch, he underwent emergency resection of the tumor and partial resection of the colon. Histologic examination and genetic testing revealed that the tumor was a recurrence of the synovial sarcoma. After discharge, the patient received treatment with gemcitabine and docetaxel. However, 7 months after the second surgery, intraperitoneal manifestations recurred. The patient died 14 months after the second resection. This case suggests that curative surgical resection of the primary synovial sarcoma of the spermatic cord contributes to prolonged survival. However, because the recurrence rate of synovial sarcoma is high, multidisciplinary treatment, including chemotherapy and radiotherapy, might be necessary.

摘要

原发性精索滑膜肉瘤极为罕见,日本文献中尚无相关报道。我们报告一例原发性精索滑膜肉瘤患者,在原发性病变根治性切除7年后出现腹腔内出血复发,且肿瘤发生局部播散。一名70岁男性因排尿障碍和下腹部疼痛入院。腹部计算机断层扫描(CT)显示有两个病灶:一个位于盆腔内、大小为10厘米且伴有出血的肿瘤,以及一个位于膀胱旁、大小为4厘米的肿瘤。对肿瘤进行了根治性切除。组织学检查显示,较大的病灶是精索原发性肿瘤,梭形细胞在细胞束中呈单调模式增殖,这与单相纤维性滑膜肉瘤的组织学表现相符。较小的病灶是播散性肿瘤。通过检测SS18(SYT)-SSX1融合基因确诊为滑膜肉瘤。出院后,患者接受了包括异环磷酰胺和多柔比星在内的辅助化疗。此后未发现复发迹象。术后7年,患者突然出现腹痛和腹胀,并转入我院。CT显示网膜囊内有一个大小为17厘米且伴有大量出血的肿瘤。通过肠系膜上动脉插管,确定出血来自胰背动脉的一个分支。由于难以对出血分支进行插管,患者接受了肿瘤急诊切除和结肠部分切除术。组织学检查和基因检测显示,该肿瘤为滑膜肉瘤复发。出院后,患者接受了吉西他滨和多西他赛治疗。然而,第二次手术后7个月,腹腔内病变复发。患者在第二次切除术后14个月死亡。该病例表明,精索原发性滑膜肉瘤的根治性手术切除有助于延长生存期。然而,由于滑膜肉瘤的复发率较高,可能需要包括化疗和放疗在内的多学科治疗。

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