Suppr超能文献

一例罕见的肛管直肠恶性黑色素瘤(ARMM):临床表现与手术结果

An uncommon case of anorectal malignant melanoma (ARMM): Clinical presentation and surgical outcome.

作者信息

Wong Jing Yuan, Tiang Ko-Ping, Aziz Nora Binti Abdul

机构信息

Department of Surgery, University of Malaya Medical Centre, Kuala Lumpur, Malaysia.

Department of Surgery, University of Malaya Medical Centre, Kuala Lumpur, Malaysia.

出版信息

Int J Surg Case Rep. 2024 Nov;124:110394. doi: 10.1016/j.ijscr.2024.110394. Epub 2024 Oct 1.

Abstract

INTRODUCTION AND IMPORTANCE

Anorectal mucosal melanoma (ARMM) is a rare disease with a poor prognosis. However, surgery is often difficult, due to the lentiginous growth pattern of such melanoma.

CASE PRESENTATION

A 61 years old lady presented with anal pain for 1 year, associated with painless fresh per rectal bleeding post defecation and altered bowel habit. Physical examination showed hyperpigmentation at the anal verge, extending to the dentate line. CT, MRI and PET imaging showed localised disease. She underwent pelvic exanteration and radical lymph node dissection with gracilis flap coverage. Post operatively, she recovers well, and was discharged well on day 8. HPE came back as malignant melanoma, with 1 out 12 lymph nodes involved. She was subsequently referred to oncology, started on pembrolizumab immunotherapy.

CLINICAL DISCUSSION

Anorectal melanoma is an aggressive disease, often present with delayed diagnosis. Multiple imaging has been proposed, however none is standardized to diagnose ARMM. Immunohistochemical stains such as S-100 protein, MelanA and tyrosinase and with HMB-45 help in diagnosis and are sensitive for melanocytic differentiation. Surgery excision remains the most common and superior initial treatment for ARMM. One retrospective study done to compare different treatment modalities has shown that patients with surgical excision and radiation therapy had the highest median survival at 32.3 months but surgical excision remains the single best modality for ARMM.

CONCLUSION

Suspicious hyperpigmentation at the anal region should raise clinical awareness. Surgical excision with optimal margin is indicated to achieve favourable symptom control, reduce local recurrence and improve survival rate.

摘要

引言与重要性

肛管黏膜黑色素瘤(ARMM)是一种预后较差的罕见疾病。然而,由于此类黑色素瘤呈雀斑样生长模式,手术往往具有难度。

病例介绍

一位61岁女性因肛门疼痛1年就诊,伴有排便后无痛性新鲜直肠出血及排便习惯改变。体格检查显示肛门边缘色素沉着,延伸至齿状线。CT、MRI和PET成像显示为局限性疾病。她接受了盆腔脏器清除术及根治性淋巴结清扫术,并采用股薄肌皮瓣覆盖。术后,她恢复良好,于第8天顺利出院。组织病理学检查结果为恶性黑色素瘤,1枚(共12枚)淋巴结受累。随后她被转诊至肿瘤科,开始接受帕博利珠单抗免疫治疗。

临床讨论

肛管黑色素瘤是一种侵袭性疾病,常出现诊断延迟。已有人提出多种影像学检查方法,但均未标准化用于诊断ARMM。免疫组织化学染色如S-100蛋白、MelanA、酪氨酸酶以及HMB-45有助于诊断,且对黑素细胞分化敏感。手术切除仍然是ARMM最常见且最佳的初始治疗方法。一项比较不同治疗方式的回顾性研究表明,接受手术切除和放射治疗的患者中位生存期最长,为32.3个月,但手术切除仍然是ARMM的单一最佳治疗方式。

结论

肛门区域可疑的色素沉着应提高临床警惕。应进行具有最佳切缘的手术切除,以实现良好的症状控制,减少局部复发并提高生存率。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6df4/11490695/f35208fb2177/gr1.jpg

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验