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本文引用的文献

1
Primary embryonal rhabdomyosarcoma of the liver in a young male.一名年轻男性的肝脏原发性胚胎性横纹肌肉瘤
J Coll Physicians Surg Pak. 2013 Oct;23(10):750-1. doi: 10.2013/JCPSP.750751.
2
Hepatic rhabdomyosarcoma in an adult: a rare primary malignant liver tumor. Case report and literature review.成人肝横纹肌肉瘤:一种罕见的原发性肝脏恶性肿瘤。病例报告及文献综述。
Acta Gastroenterol Belg. 2011 Dec;74(4):576-81.
3
Pediatric sarcomas occurring in adults.发生于成人的小儿肉瘤。
J Surg Oncol. 2008 Mar 15;97(4):360-8. doi: 10.1002/jso.20969.
4
Age is an independent prognostic factor in rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group.年龄是横纹肌肉瘤的独立预后因素:来自儿童肿瘤学组软组织肉瘤委员会的报告。
Pediatr Blood Cancer. 2004 Jan;42(1):64-73. doi: 10.1002/pbc.10441.
5
Clinicopathologic analysis of patients with adult rhabdomyosarcoma.成人横纹肌肉瘤患者的临床病理分析
Cancer. 2001 Feb 15;91(4):794-803.
6
Hepatic undifferentiated (embryonal) sarcoma and rhabdomyosarcoma in children. Results of therapy.儿童肝未分化(胚胎性)肉瘤和横纹肌肉瘤。治疗结果。
Cancer. 1987 Feb 1;59(3):396-402. doi: 10.1002/1097-0142(19870201)59:3<396::aid-cncr2820590307>3.0.co;2-w.

肝脏原发性胚胎性横纹肌肉瘤

Primary embryonal rhabdomyosarcoma of the liver.

作者信息

Arora Aditi, Jaiswal Ritika, Anand Nidhi, Husain Nuzhat

机构信息

Department of Pathology, Dr Ram Manohar Lohia Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

出版信息

BMJ Case Rep. 2016 Dec 30;2016:bcr2016218292. doi: 10.1136/bcr-2016-218292.

DOI:10.1136/bcr-2016-218292
PMID:28039349
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5237799/
Abstract

Rhabdomyosarcomas are malignant neoplasms with striated muscle differentiation. This is the most common type of soft-tissue sarcoma in children, but occurs rarely in adults. Its occurrence in liver is infrequent. We report a case of primary hepatic embryonal rhabdomyosarcoma in a 67-year-old man. The tumour was occupying the left lobe of the liver with large component of lesion seen bulging in left subhepatic space indenting over the stomach, compressing the pancreas and gall bladder. A percutaneous biopsy was performed which revealed embryonal rhabdomyosarcoma. He underwent a successful left lobectomy with complete resection of the tumour, followed by adjuvant chemotherapy. The patient is free of disease at 24 months of follow-up. Hence, an early diagnosis, prompt surgical resection with negative resection margin along with adjuvant chemotherapy can provide complete remission. This case is extremely rare both due to the age of the patient at presentation and location of the tumour.

摘要

横纹肌肉瘤是具有横纹肌分化的恶性肿瘤。这是儿童中最常见的软组织肉瘤类型,但在成人中很少见。其在肝脏中的发生并不常见。我们报告一例67岁男性原发性肝胚胎性横纹肌肉瘤病例。肿瘤占据肝脏左叶,病变大部分可见于肝下左间隙膨出,压迫胃,压迫胰腺和胆囊。进行了经皮活检,结果显示为胚胎性横纹肌肉瘤。他接受了成功的左叶切除术,肿瘤完全切除,随后进行辅助化疗。随访24个月时患者无疾病。因此,早期诊断、手术切除切缘阴性并及时进行辅助化疗可实现完全缓解。由于患者就诊时的年龄和肿瘤的位置,该病例极为罕见。