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成人阴囊内巨大胚胎性横纹肌肉瘤:一例报告并文献复习

Giant intrascrotal embryonal rhabdomyosarcoma in an adult: a case report and review of the literature.

作者信息

Gong Wentao, Gao Qingqiang, Xu Zhipeng, Dai Yutian

机构信息

Medical School of Nanjing University, Nanjing, 210093, China.

Department of Andrology, Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School, Nanjing, 210008, China.

出版信息

J Med Case Rep. 2018 May 28;12(1):149. doi: 10.1186/s13256-018-1607-1.

Abstract

BACKGROUND

Intrascrotal embryonal rhabdomyosarcoma in adults is a rare tumor with high aggression and a poor prognosis. We report our patient's case and review the relevant literature to improve the understanding of this rare disease.

CASE PRESENTATION

A 21-year-old Han Chinese man presented to our hospital with a right intrascrotal mass of 1 year's duration. His physical examination revealed an enlarged right scrotum containing a huge tender mass measuring about 10 × 7 cm. Ordinary and contrast-enhanced ultrasonography showed a solid mass in the right scrotum, which was suspected to be a malignant tumor. An abdominopelvic computed tomographic scan revealed metastases in the retroperitoneal lymph nodes. The patient was diagnosed with malignant testicular tumor and underwent a right radical orchiectomy by an inguinal approach. Postoperative pathological examination suggested an intrascrotal embryonal rhabdomyosarcoma.

CONCLUSIONS

Intrascrotal embryonal rhabdomyosarcoma is a rare but highly aggressive tumor. Clinical and imaging manifestations of this tumor are nonspecific, so the definitive diagnosis depends on postoperative pathology and immunohistochemistry. Early suspicion, radical orchiectomy, accurate pathologic diagnosis, and adjuvant chemotherapy and/or radiotherapy are the keys to optimal prognosis.

摘要

背景

成人阴囊内胚胎性横纹肌肉瘤是一种罕见的肿瘤,侵袭性高,预后差。我们报告我们患者的病例并复习相关文献以提高对这种罕见疾病的认识。

病例介绍

一名21岁的汉族男性因右侧阴囊肿物1年就诊于我院。体格检查发现右侧阴囊增大,内有一巨大压痛性肿物,大小约10×7cm。普通超声及增强超声显示右侧阴囊内实性肿物,怀疑为恶性肿瘤。腹盆腔计算机断层扫描显示腹膜后淋巴结转移。患者被诊断为恶性睾丸肿瘤,经腹股沟入路行右侧根治性睾丸切除术。术后病理检查提示阴囊内胚胎性横纹肌肉瘤。

结论

阴囊内胚胎性横纹肌肉瘤是一种罕见但侵袭性很强的肿瘤。该肿瘤的临床和影像学表现无特异性,因此明确诊断依赖于术后病理及免疫组化。早期怀疑、根治性睾丸切除术、准确的病理诊断以及辅助化疗和/或放疗是获得最佳预后的关键。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/17ed/5971421/22bed35cb5a7/13256_2018_1607_Fig1_HTML.jpg

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