■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不到0.1%。这些肿瘤比其他胃肠道肿瘤更具侵袭性,预后较差,包括胃肠道间质瘤(GIST)或腺癌。作者在此报告了两例病例,并回顾了文献以强调流行病学,诊断,这种罕见恶性肿瘤的治疗和预后。
■作者报告了两例非常罕见的左半结肠LMS病例,提到我们的机构有腹痛的症状。经过初步调查,患者被诊断为原发性结肠平滑肌肉瘤,接受剖腹手术。在两种情况下,病理检查均显示梭形细胞肿瘤在结肠中周向和经壁生长。最终免疫组织化学为SMA阳性,CK和结蛋白无GIST标志物(CD117、CD34和DOG1)的表达,证实平滑肌肉瘤。一名患者在术后6个月被诊断为弥漫性腹膜转移,并在接受治疗2个月后死亡。另一个仍在积极监视。
■结肠的LMS是一种非常罕见的实体,仅在临床病例报告中出现。LMS具有非特异性症状,通常在其达到较大尺寸时被诊断出来。手术是主要的治疗选择。如今,没有明确的证据证明化疗和放疗的有效性.
■LMS是一种罕见的结肠肿瘤。暂时,没有治疗指南,但是手术仍然起着至关重要的作用。
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: LMS is a rare neoplasm of colon. For the time being, there is no guidelines for treatment, but surgery still plays a fundamental role.