Otabe Masaya, Abe Tomoyuki, Sumi Yusuke, Yonehara Shuji, Noriyuki Toshio, Nakahara Masahiro
Departments of Surgery, Onomichi General Hospital, 1-10-23, Hirahara, Onomichi, Hiroshima, 722-8508, Japan.
Departments of Pathology, Onomichi General Hospital, Onomichi, Hiroshima, Japan.
Surg Case Rep. 2022 Aug 1;8(1):147. doi: 10.1186/s40792-022-01497-4.
A leiomyosarcoma (LMS) is a rare tumor that mainly originates from the urinary tract and digestive system; however, non-visceral organ-derived patterns are rare. Herein, we report that a vessel-derived pleomorphic LMS (PLMS) originating from the mesenteric vein has a poor prognosis even after curative-intent surgery.
The patient was a 41-year-old woman with no relevant medical history. The patient presented with abdominal pain and an abnormal bulge on the left lower abdomen. Magnetic resonance imaging revealed a large tumor occupying the left abdomen. Enhanced computed tomography revealed a bulky tumor with a maximum size of 13 × 13 cm with impending rupture, and a small amount of ascites was detected around the tumor. As the tumor directly invaded the small intestine and descending colon, left hemicolectomy and partial resection of the small intestine were performed. The patient was discharged on postoperative day 10, without any complications. On histopathological analysis, the tumor was diagnosed as a vessel-derived LMS with a pleomorphic pattern. The patient died due to disseminated intravascular coagulation because generalized peritonitis was induced by the super-early recurrence of the tumor 2 months after the surgery.
Regardless of curative-intent surgery for a vessel-derived PLMS, super-early local recurrence and distant metastasis were observed. A vessel-derived PLMS requires further investigation to determine its characteristics and therapeutic strategies to improve long-term prognosis.
平滑肌肉瘤（LMS）是一种罕见肿瘤，主要起源于泌尿道和消化系统；然而，非内脏器官来源的类型较为少见。在此，我们报告一例起源于肠系膜静脉的血管源性多形性LMS（PLMS），即使进行了根治性手术，其预后仍较差。
患者为一名41岁女性，无相关病史。患者因腹痛及左下腹异常隆起就诊。磁共振成像显示左腹部有一个巨大肿瘤。增强计算机断层扫描显示一个巨大肿瘤，最大尺寸为13×13 cm，有破裂风险，肿瘤周围检测到少量腹水。由于肿瘤直接侵犯小肠和降结肠，遂行左半结肠切除术及小肠部分切除术。患者术后第10天出院，无任何并发症。组织病理学分析显示，肿瘤被诊断为血管源性多形性LMS。患者术后2个月因肿瘤超早期复发诱发弥漫性腹膜炎，最终死于弥散性血管内凝血。
对于血管源性PLMS，无论是否进行根治性手术，均观察到超早期局部复发和远处转移。血管源性PLMS需要进一步研究以确定其特征和治疗策略，从而改善长期预后。
