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成人会阴部胚胎性横纹肌肉瘤：一例报告

Embryonal rhabdomyosarcoma of the perineum in an adult: a case report.

作者信息

Ka Sidy, Gnangnon Freddy, Dieng Mamadou Moustapha, Diouf Doudou, Thiam Jaafar, Gaye Pape Macoumba, Dem Ahmadou

机构信息

Joliot Curie Institute, Dakar, Senegal.

出版信息

J Med Case Rep. 2016 Dec 20;10(1):353. doi: 10.1186/s13256-016-1166-2.

DOI:10.1186/s13256-016-1166-2
PMID:27998313
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5175321/
Abstract

BACKGROUND

We report the case of an adult patient with embryonal rhabdomyosarcoma of the perineum admitted to our practice at Joliot Curie Institute in Dakar. It is a rare tumor at this age and has a bad prognosis at this localization.

CASE PRESENTATION

We describe the case of a 22-year-old African man admitted for a perineal mass that had evolved over 6 months. He complained of tenesmus, obstinate constipation, and dysuria. A clinical examination revealed perineal swelling spread over his anus, scrotum, penis, testicles, and inguinal lymph nodes. A perineal ultrasound and computed tomography scan showed a large mass driving his testicles forward with regional lymph node metastases. An ultrasound-guided biopsy showed embryonal rhabdomyosarcoma on histology and immunohistochemistry, with strong positivity of neural cell adhesion molecule and myogenin while results for cytokeratin AE1/AE3, cluster of differentiation 45, synaptophysin, and chromogranin were negative. Our patient was classified T2N1M1. Outcome was quickly marked by occlusive syndrome and colostomy. Our patient did not opt for chemotherapy and died after 6 months of follow-up.

CONCLUSIONS

The embryonic RMS of the adult is a rare disease. Despite the sensitivity to chemotherapy and surgery. Localization to perineum remains poor prognosis.

摘要

背景

我们报告了一例成年会阴胚胎性横纹肌肉瘤患者，该患者被收治于达喀尔居里研究所。这是一种在这个年龄段罕见的肿瘤，且在此部位预后不良。

病例介绍

我们描述了一名22岁非洲男性患者的病例，该患者因会阴肿物入院，肿物已发展6个月。他主诉里急后重、顽固性便秘和排尿困难。临床检查发现会阴肿胀蔓延至肛门、阴囊、阴茎、睾丸和腹股沟淋巴结。会阴超声和计算机断层扫描显示一个大肿物将睾丸向前推移，并伴有区域淋巴结转移。超声引导下活检在组织学和免疫组织化学检查中显示为胚胎性横纹肌肉瘤，神经细胞黏附分子和肌生成素呈强阳性，而细胞角蛋白AE1/AE3、分化簇45、突触素和嗜铬粒蛋白的结果为阴性。我们的患者被分类为T2N1M1。结局很快以梗阻综合征和结肠造口术为特征。我们的患者未选择化疗，随访6个月后死亡。

结论

成人胚胎性横纹肌肉瘤是一种罕见疾病。尽管对化疗和手术敏感，但位于会阴部位的预后仍然很差。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/73ac/5175321/e88a4d176b64/13256_2016_1166_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/73ac/5175321/9f39b2a07f83/13256_2016_1166_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/73ac/5175321/bef9742b1d0c/13256_2016_1166_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/73ac/5175321/a00660ee436b/13256_2016_1166_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/73ac/5175321/e88a4d176b64/13256_2016_1166_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/73ac/5175321/9f39b2a07f83/13256_2016_1166_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/73ac/5175321/bef9742b1d0c/13256_2016_1166_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/73ac/5175321/a00660ee436b/13256_2016_1166_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/73ac/5175321/e88a4d176b64/13256_2016_1166_Fig4_HTML.jpg

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Treatment and outcome of patients suffering from perineal/perianal rhabdomyosarcoma: results from the CWS trials--retrospective clinical study.会阴/肛周横纹肌肉瘤患者的治疗和转归：CWS 试验的结果——回顾性临床研究。
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Building the bridge between rhabdomyosarcoma in children, adolescents and young adults: the road ahead.
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Pediatric surgical oncology: management of rhabdomyosarcoma.小儿外科肿瘤学：横纹肌肉瘤的管理
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Two consecutive phase II window trials of irinotecan alone or in combination with vincristine for the treatment of metastatic rhabdomyosarcoma: the Children's Oncology Group.儿童肿瘤学组开展的两项关于伊立替康单药或联合长春新碱治疗转移性横纹肌肉瘤的连续II期探索性试验。
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