Shen Xiang-qian, Shen Hui, Wu Shou-cheng, Lv Ying, Lu Hui, Lin Xiang-jin
Department of Hand Surgery and Microsurgery Center, The First Affiliated Hospital, College of Medicine, ZheJiang University, HangZhou, China.
The Children's Hospital, Zhejiang University School of Medicine, HangZhou, China.
World J Surg Oncol. 2016 Apr 27;14:125. doi: 10.1186/s12957-016-0880-y.
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.
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.
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.
1型神经纤维瘤病患者的巨大神经纤维瘤累及多个区域,通常难以通过手术切除。然而,手术干预是改善生活质量的最有效手段。本文报道的病例独特之处在于,它涉及一个巨大神经纤维瘤,累及患者的腹腔和盆腔、腹膜后间隙及臀部,导致腹部和盆腔器官受压移位。需要进行具有挑战性的手术干预以实现近乎完全切除,从而在保留内脏和生殖肛门功能的同时缓解器官压迫。
报道的病例为一名患者,患有孤立性巨大腹膜后神经纤维瘤,该肿瘤几乎占据了整个腹腔和盆腔,并明显突出至右侧臀区。通过手术分三个部分切除了巨大的哑铃形肿块。术后病理研究确诊为神经纤维瘤。术后三个月的计算机断层扫描图像显示肛门周围区域有残留肿瘤。在八个月的随访中,患者的生活质量有了明显改善。
在巨大神经纤维瘤导致关键器官受压移位的特殊情况下进行手术干预,可减轻肿瘤负担,恢复患者身体和内脏器官的外观及功能,并改善患者的生活质量。