Surgically treated solitary giant gluteal and retroperitoneal neurofibroma: a case report

被引:7
|
作者
Shen, Xiang-qian [1 ,2 ]
Shen, Hui [1 ,2 ]
Wu, Shou-cheng [1 ,2 ]
Lv, Ying [3 ]
Lu, Hui [1 ,2 ]
Lin, Xiang-jin [4 ]
机构
[1] Zhejiang Univ, Coll Med, Affiliated Hosp 1, Dept Hand Surg, Hangzhou 310003, Zhejiang, Peoples R China
[2] Zhejiang Univ, Coll Med, Affiliated Hosp 1, Microsurg Ctr, Hangzhou 310003, Zhejiang, Peoples R China
[3] Zhejiang Univ, Sch Med, Childrens Hosp, Hangzhou 310003, Zhejiang, Peoples R China
[4] Zhejiang Univ, Coll Med, Affiliated Hosp 1, Dept Orthopaed, 79 Qingchun Rd, Hangzhou 310003, Zhejiang, Peoples R China
来源
基金
中国国家自然科学基金;
关键词
Neurofibromatosis type 1; Giant neurofibroma; Surgical treatment; Gluteal; Retroperitoneal; MANAGEMENT;
D O I
10.1186/s12957-016-0880-y
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
Background: Giant neurofibromas in patients with neurofibromatosis type 1 involve multiple regions and are often difficult to surgically extirpate. However, surgical intervention is the most effective means for improving quality of life. The case reported herein is unique in that it involves a giant neurofibroma, involving the patient's peritoneal and pelvic cavities, retroperitoneal space, and buttock, which was causing compressive displacement of abdominal and pelvic organs. A challenging surgical intervention was required to accomplish near-total resection to relieve organ compression while preserving visceral and genitoanal function. Case presentation: The case reported is of a patient presenting with a solitary giant retroperitoneal neurofibroma that threatened to obliterate both peritoneal and pelvic cavities and protruded conspicuously into the right gluteal region. The enormous dumbbell-shaped mass was surgically removed in three parts. Postoperative pathology studies confirmed a diagnosis of neurofibroma. Follow-up computed tomography images taken three months postoperatively revealed residual tumor in the perianal region. The patient's quality of life had measurably improved on follow-up at eight months. Conclusions: Surgical intervention in such extraordinary circumstances of a giant neurofibroma causing compressive displacement of critical organs reduces tumor burden, restores appearance and function of patient's body and internal organs, and improves the patient's quality of life.
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页数:4
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