Parini Umberto, Nardi Mario, Loffredo Andrea, Fabozzi Massimiliano, Roveroni Maurizio
Divisione di Chirurgia Generale, Ospedale Regionale della Valle D'Aosta, Aosta.
Chir Ital. 2007 Jul-Aug;59(4):551-8.
Paraganglioma is an exceedingly rare tumour of the duodenum that arises in close proximity to the ampulla of Vater. To date a total of 133 cases of duodenal paraganglioma have been reported in the literature; of these, 27 (20%) were histologically gangliocytic paragangliomas. This neoplasm generally behaves in a benign fashion, although instances of recurrence and/or lymph node metastasis have been described. The treatment consists in endoscopic polypectomy or surgical resection in relation to the histological features and the macroscopic extent of the neoplasm. We present a case of a benign duodenal gangliocytic paraganglioma treated by a laparo-endoscopic approach. We report a case of gangliocytic paraganglioma in a 75-year-old woman admitted to the General Surgery Division of Aosta Regional Hospital (Aosta-Italy), complaining of melaena and anaemia. Upper gastrointestinal endoscopy followed by enteroscopy with a video-capsula, revealed a pedunculated neoplasm in the second portion of the duodenum, with ulceration of the overlying mucosa. Multiple biopsies were performed during the endoscopic examination and showed the cellular pattern of benign paraganglioma. After stabilisation of the patient's clinical status, we performed a resection of the neoplasm via a laparoscopic transduodenal approach and a concomitant intraoperative duodenoscopy. The histological features showed a gangliocytic paraganglioma without a malignant cell pattern. The size of the neoplasm was 4 cm. The resection margins were free of neoplastic infiltration. The postoperative stay was 9 days and there were no intraoperative or postoperative complications. The patient is currently in good health without any tumour recurrence. Transduodenal laparoscopic resection with intraoperative duodenoscopy is a valuable treatment for benign gangliocytic paraganglioma of the duodenum which is unresectable by upper gastrointestinal endoscopy. This approach affords the advantages of the minimally invasive technique and fulfils the surgical tenets of the open transduodenal approach, if en bloc resection of the neoplasm with the adjacent duodenal wall is performed.
副神经节瘤是一种极为罕见的十二指肠肿瘤,起源于靠近十二指肠乳头处。迄今为止,文献中共报道了133例十二指肠副神经节瘤;其中,27例(20%)在组织学上为神经节细胞性副神经节瘤。这种肿瘤通常表现为良性,尽管也有复发和/或淋巴结转移的病例报道。治疗方法包括根据肿瘤的组织学特征和宏观范围进行内镜下息肉切除术或手术切除。我们报告一例采用腹腔镜-内镜联合手术治疗的十二指肠良性神经节细胞性副神经节瘤病例。我们报道一名75岁女性患者,因黑便和贫血入住意大利奥斯塔地区医院普通外科,被诊断为神经节细胞性副神经节瘤。上消化道内镜检查后,使用视频胶囊进行小肠镜检查,发现十二指肠第二部有一个带蒂肿瘤,其上方黏膜有溃疡。在内镜检查期间进行了多次活检,显示为良性副神经节瘤的细胞模式。在患者临床状况稳定后,我们通过腹腔镜经十二指肠入路并在术中同时进行十二指肠镜检查切除了肿瘤。组织学特征显示为神经节细胞性副神经节瘤,无恶性细胞模式。肿瘤大小为4厘米。手术切缘无肿瘤浸润。术后住院9天,无术中或术后并发症。患者目前健康状况良好,无肿瘤复发。对于无法通过上消化道内镜切除的十二指肠良性神经节细胞性副神经节瘤,经十二指肠腹腔镜切除术并在术中进行十二指肠镜检查是一种有价值的治疗方法。如果能将肿瘤与相邻十二指肠壁整块切除,这种方法具有微创技术的优势,并符合开放经十二指肠入路的手术原则。