Doan Tu Trong, Pham Duong The
Department of Abdominal Surgery 2, Vietnam National Cancer Hospital, Hanoi, Vietnam.
Ann Med Surg (Lond). 2024 Jun 17;86(8):4865-4869. doi: 10.1097/MS9.0000000000002277. eCollection 2024 Aug.
Primary leiomyosarcoma (LMS) of the colon is a rare neoplasm and constitutes less than 0.1% of all colon malignancies. These tumors are more aggressive and have poorer prognoses than other gastrointestinal tumors, including gastrointestinal stromal tumors (GIST) or adenocarcinomas. The authors herein report two cases and review the literature to highlight the epidemiology, diagnosis, treatment and prognosis of this uncommon malignancy.
The authors reported two very rare cases of LMS of left colon, which referred to our institution with symptoms of abdominal pain. After the initial investigations, patients were diagnosed with primary colonic leiomyosarcoma that underwent laparotomy. In both cases pathological examination revealed a spindle cell tumor growing circumferentially and transmurally in the colon. Final immunohistochemistry were positive with SMA, CK and desmin without the expression of GIST markers (CD117, CD34 and DOG1) that confirmed leiomyosarcoma. One patient was diagnosed with diffused peritoneal metastasis at 6 months postoperatively and he died after 2 months of paliative care, another one is still on active surveillance.
LMS of the colon is a really rare entity and is only presented in clinical case reports. LMS has non-specific symptoms and is commonly diagnosed when it reaches a large size. Surgery is a mainstay treatment option. Nowadays, there is no clear evidence for the effectiveness of chemotherapy and radiation therapy.
LMS is a rare neoplasm of colon. For the time being, there is no guidelines for treatment, but surgery still plays a fundamental role.
结肠原发性平滑肌肉瘤（LMS）是一种罕见的肿瘤，占所有结肠恶性肿瘤的比例不到0.1%。与其他胃肠道肿瘤，包括胃肠道间质瘤（GIST）或腺癌相比，这些肿瘤侵袭性更强，预后更差。本文作者报告两例病例并回顾文献，以突出这种罕见恶性肿瘤的流行病学、诊断、治疗及预后情况。
作者报告了两例非常罕见的左半结肠LMS病例，患者因腹痛症状转诊至我院。经过初步检查，患者被诊断为原发性结肠平滑肌肉瘤并接受了剖腹手术。两例病例的病理检查均显示为梭形细胞瘤，在结肠内呈环形生长并穿透肠壁。最终免疫组化结果显示平滑肌肌动蛋白（SMA）、细胞角蛋白（CK）和结蛋白呈阳性，而GIST标志物（CD117、CD34和DOG1）未表达，从而确诊为平滑肌肉瘤。一名患者术后6个月被诊断为弥漫性腹膜转移，在接受2个月的姑息治疗后死亡，另一名患者仍在接受积极监测。
结肠LMS是一种非常罕见的疾病，仅在临床病例报告中出现。LMS症状不具特异性，通常在肿瘤长得很大时才被诊断出来。手术是主要的治疗选择。目前，尚无明确证据表明化疗和放疗的有效性。
LMS是一种罕见的结肠肿瘤。目前尚无治疗指南，但手术仍起着根本性作用。
