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成人巨大无症状腹膜后神经节瘤致主要腹腔脏器和血管移位

Large Asymptomatic Retroperitoneal Ganglioneuroma Displacing Major Abdominal Organs and Vessels in an Adult.

机构信息

Department of Surgery, King Khalid University Hospital, King Saud University, Riyadh, Saudi Arabia.

College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.

出版信息

Am J Case Rep. 2021 Jun 22;22:e931725. doi: 10.12659/AJCR.931725.

Abstract

BACKGROUND Ganglioneuromas (GNs) are benign neuroblastic tumors. These extra-cranial solid tumors are common in childhood but unusual in adults. Patients with GNs typically do not have any symptoms and the tumors usually are incidental findings. However, if a GN is large enough to compress adjacent organs, complications can occur. Furthermore, even in patients who have incomplete resection of a GN, long-term survival rates are high. After a GN is seen on imaging, the diagnosis usually is made with a biopsy and treatment is with surgery alone. CASE REPORT A 29-year-old woman was referred to General Surgery from the Gynecology Clinic for an incidental finding of an abdominal mass on routine ultrasound for secondary infertility and admitted for an investigation. Abdominal and pelvic computed tomography (CT) and magnetic resonance imaging showed a retroperitoneal mass that measured 6.318×22 cm arising from the paravertebral region with intraspinal extension. The mass was displacing the patient's thoracic aorta, abdominal inferior vena cava, and ureters. A CT-guided biopsy revealed a GN. Debulking surgery was performed and a small amount of residual tumor was left in the paravertebral nerve roots. The patient recovered well with no complications. The diagnosis of GN was confirmed with pathology, which was reviewed by the Tumor Board; the Board agreed that only follow-up in the Surgery Clinic was needed. During the patient's last visit, 10 months after surgery, a follow-up CT scan showed that the residual tumor was stable. CONCLUSIONS GNs are benign abdominal and retroperitoneal tumors that are typically asymptomatic and detected incidentally. Surgical resection is the treatment of choice and even when it is incomplete, the prognosis for patients is excellent.

摘要

背景

神经节瘤(GNs)是良性神经母细胞瘤。这些颅外实体瘤在儿童中较为常见,但在成人中较为罕见。GN 患者通常没有任何症状,且肿瘤通常为偶然发现。然而,如果 GN 足够大以至于压迫邻近器官,则可能发生并发症。此外,即使 GN 患者未能完全切除肿瘤,其长期生存率仍很高。在影像学检查发现 GN 后,通常通过活检来做出诊断,且仅采用手术治疗。

病例报告

一名 29 岁女性因继发性不孕在常规超声检查时偶然发现腹部肿块,由妇科转至普外科就诊并入院检查。腹部和盆腔计算机断层扫描(CT)和磁共振成像(MRI)显示,源自椎旁区域的腹膜后肿块,大小为 6.318×22cm,向椎管内延伸。该肿块推移患者的胸主动脉、腹部下腔静脉和输尿管。CT 引导下的活检显示为神经节瘤。进行了减瘤手术,在椎旁神经根中留下少量残余肿瘤。患者术后恢复良好,无并发症。肿瘤委员会对病理结果进行了审查,证实了 GN 的诊断;委员会一致认为仅需要在普外科进行随访。在患者术后 10 个月的最后一次就诊时,进行了 CT 随访扫描,显示残余肿瘤稳定。

结论

GN 是良性的腹部和腹膜后肿瘤,通常无症状且为偶然发现。手术切除是首选治疗方法,即使不完全切除,患者的预后也非常好。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f6a5/8235676/985073dd70f9/amjcaserep-22-e931725-g001.jpg

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