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[肛管平滑肌肉瘤]

[Leiomyosarcoma of the anal canal].

作者信息

Consentino B, Arnaud A, Sarles J C

机构信息

Service de Chirurgie Digestive, CHU Nord, Marseille.

出版信息

J Chir (Paris). 1988 Apr;125(4):245-8.

PMID:3392131
Abstract

Leiomyosarcoma originating in the anal canal internal sphincter is an extremely rare malignant tumor of often late diagnosis, since usually asymptomatic, and with malignancy criteria that are sometimes difficult to define. These tumors affect men and women with equal frequency, usually in the 6 th decade. Two cases are reported in patients aged 67 and 65 respectively in whom the tumor was revealed by a painful perianal mass shown on rectoscopy to be due to a submucous tumor projecting into anal canal, Prognosis and treatment of these tumors is discussed, diagnosis being confirmed only after excision biopsy. Tumoral extension was both local and regional by continuity but blood dissemination had occurred with metastases more frequently in liver (1 case) but also in lungs. Glandular extension was exceptional. Limited local exeresis of small tumors appears justified if sufficiently wide, since malignancy of leiomyosarcoma appears to remain circumscribed over long periods. However, this limited procedure runs the risk of local recurrence with the need for abdominoperineal amputation (the case in the 2 patients reported), although long-term results appear to be similar whether excision is or is not extensive, the abdominoperineal amputation failing to avoid the unfavorable course. Complementary radiotherapy or chemotherapy is ineffective, and prognosis is related more to the degree of tumoral differentiation than to the operative treatment itself.

摘要

起源于肛管内括约肌的平滑肌肉瘤是一种极为罕见的恶性肿瘤,通常因无症状而诊断较晚,且其恶性标准有时难以界定。这些肿瘤在男性和女性中的发病率相同,发病年龄通常在60多岁。本文报告了两例患者,年龄分别为67岁和65岁,均因直肠镜检查发现肛周疼痛性肿块而确诊,肿块为突入肛管的黏膜下肿瘤。本文讨论了这些肿瘤的预后和治疗方法,只有在切除活检后才能确诊。肿瘤通过连续性在局部和区域扩散,但已发生血行播散,转移更常见于肝脏(1例),也见于肺部。腺性扩散罕见。对于小肿瘤,如果切除范围足够宽,进行有限的局部切除似乎是合理的,因为平滑肌肉瘤的恶性程度似乎在很长一段时间内都局限在一定范围内。然而,这种有限的手术有局部复发的风险,可能需要进行腹会阴联合切除术(本文报告的2例患者均如此),尽管无论切除范围是否广泛,长期结果似乎相似,腹会阴联合切除术并不能避免不良病程。辅助放疗或化疗无效,预后更多地与肿瘤分化程度有关,而非手术治疗本身。

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