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巨大腹部与会阴侵袭性血管黏液瘤:1例误诊病例报告及文献复习

Huge abdominal and perineal aggressive angiomyxoma: A misdiagnosed case report and literature review.

作者信息

Ma Jianhua, Wan Jianghou, Jiang Bin, Wang Hua, Zhang Wei, Zhang Xiangbo

机构信息

Department of Urology, The First Hospital of Lanzhou University, Lanzhou 730000, China.

出版信息

Cell Mol Biol (Noisy-le-grand). 2018 May 15;64(6):110-113.

Abstract

Aggressive angiomyxoma (AA) is a distinctive soft tissue tumor with a high risk of local recurrence. Clinicians must be aware of this rare tumor pre-operatively. Excision is the preferred method of AA treatment. The case report presents a case of a 36-year-old woman who was difficulty in walking due to a non-painful tumor in the abdomen and perineum. She was misdiagnosed as abdomen neurofibroma for more than 10 years, and an operation was performed in 1997. However, the tumor was incompletely resected because its huge volume accompanies with extensive infiltration and bleeding. The tumors in her abdomen and perineum were growing gradually, and the latter became a large lump which impeded her daily life. In 2008, the perineal tumor was incompletely resected, which weighed 10725 g. The severe hemorrhage had been ceased by Gonadotropin-Releasing Hormone treatment. She is alive till now. Details of the history and operative procedures are presented. An AA diagnosis was made by microscopy immunohistochemically. Long-time misdiagnosis and improper treatment are the important reasons for making it impossible to be radically resected. Pathological and immunohistochemical examination are important for avoiding misdiagnosis. For this case, there is a remaining tumor in her abdomen. A special project including further follow-up and treatment will be taken out.

摘要

侵袭性血管黏液瘤(AA)是一种具有局部复发高风险的独特软组织肿瘤。临床医生术前必须了解这种罕见肿瘤。手术切除是AA治疗的首选方法。该病例报告介绍了一名36岁女性患者,因腹部和会阴无痛性肿瘤而行走困难。她被误诊为腹部神经纤维瘤超过10年,并于1997年接受了手术。然而,由于肿瘤体积巨大且伴有广泛浸润和出血,手术未能完全切除肿瘤。她腹部和会阴的肿瘤逐渐生长,后者形成一个大肿块,影响了她的日常生活。2008年,会阴肿瘤再次不完全切除,重达10725克。通过促性腺激素释放激素治疗止住了严重出血。她至今仍存活。文中介绍了病史和手术过程的详细情况。通过显微镜免疫组织化学检查做出了AA诊断。长期误诊和不当治疗是无法根治性切除的重要原因。病理和免疫组织化学检查对于避免误诊很重要。对于该病例,她腹部仍有残留肿瘤。将开展包括进一步随访和治疗的专项计划。

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