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肛管平滑肌肉瘤:一例病例报告及文献复习

Anal leiomyosarcoma: A case report and review of literature.

作者信息

Haydar Alaa, Saliba Marita, Hijazi Mariam, Alameh Ali, Harake Ali

机构信息

Lebanese University, Beirut, Lebanon.

Faculty of Medicine, University of Balamand, Lebanon.

出版信息

Int J Surg Case Rep. 2023 Sep;110:108744. doi: 10.1016/j.ijscr.2023.108744. Epub 2023 Sep 1.

Abstract

INTRODUCTION

Leiomyosarcoma is a malignant neoplasm that is derived from smooth muscle cells in walls of small blood vessels or branch of the inferior vena cava, the uterus and the gastrointestinal tract. Different treatment options are present for the treatment of LMS. However, due to the rarity of LMS, the optimal treatment option is still to be discussed and determined.

PRESENTATION OF CASE

A 51-year-old male patient, previously healthy, presented for perianal pain. Biopsy of the mass found showed spindle cell tumors with mild atypia, dense cellularity, and pelvic MRI with contrast showed a well-circumscribed mass of the anus, developed between the layers of the external sphincter with possible invasion of the internal sphincter consistent with Leiomyosarcoma Grade I. Wide excision was performed. Close follow-up should be done every 3 to 6 months for the first 2 to 3 years, every 6 to 12 months for the following 3 years, and annually afterwards.

DISCUSSION

The symptoms of LMS include rectal bleeding with rectal and/or abdominal pain, weight loss, constipation, altered bowel motion and protruding mass. Treatment options include wide local excision, abdominoperineal resection, low anterior resection, bloc resection and pelvic exenteration. Patients who underwent wide local excision show a higher local recurrence rate as compared to patients who underwent radical resection. Distant metastasis is higher in patients who underwent radical resection.

CONCLUSION

The treatment options of anal LMS are controversial. At present, very few cases have been reported, thus no universally accepted standard of surgical treatment has been established.

摘要

引言

平滑肌肉瘤是一种恶性肿瘤,起源于小血管壁或下腔静脉分支、子宫及胃肠道的平滑肌细胞。对于平滑肌肉瘤有不同的治疗选择。然而,由于平滑肌肉瘤较为罕见,最佳治疗方案仍有待探讨和确定。

病例介绍

一名51岁男性患者,既往健康,因肛周疼痛就诊。肿块活检发现梭形细胞瘤,具有轻度异型性、细胞密集,盆腔增强MRI显示肛门有一个边界清晰的肿块,位于外括约肌层之间,可能侵犯内括约肌,符合I级平滑肌肉瘤。进行了广泛切除。在最初2至3年应每3至6个月进行密切随访,接下来3年每6至12个月随访一次,之后每年随访一次。

讨论

平滑肌肉瘤的症状包括直肠出血伴直肠和/或腹痛、体重减轻、便秘、排便习惯改变及肿块突出。治疗选择包括广泛局部切除、腹会阴联合切除、低位前切除、整块切除和盆腔脏器清除术。与接受根治性切除的患者相比,接受广泛局部切除的患者局部复发率更高。接受根治性切除的患者远处转移率更高。

结论

肛门平滑肌肉瘤的治疗方案存在争议。目前,报道的病例很少,因此尚未建立普遍接受的手术治疗标准。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/57e8/10509876/dc178c49d6d5/gr1.jpg

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