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               Gangliocytic Paraganglioma 1

                               

 
Laparoscopic resection of duodenal gangliocytic paraganglioma. A case report.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 intra-operative 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.

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Gangliocytic paraganglioma: case report and review of the literature.J Gastrointest Surg. 2007 Oct;11(10):1351-4. Epub 2007 Jul 25.

Gangliocytic paraganglioma is a rare tumor, which occurs nearly exclusively in the second portion of the duodenum. Generally, this tumor has a benign clinical course, although rarely, it may recur or metastasize to regional lymph nodes. Only one case with distant metastasis has been reported. We present a case of duodenal gangliocytic paraganglioma treated first by local resection followed by pylorus-preserving pancreaticoduodenectomy. Examination of the first specimen revealed focal nuclear pleomorphism and mitotic activity, in addition to the presence of three characteristic histologic components: epithelioid, ganglion, and spindle cell. In the subsequent pancreaticoduodenectomy specimen, there was no residual tumor identified in the periampullary area, but metastatic gangliocytic paraganglioma was present in two of seven lymph nodes. This case report confirms the malignant potential of this tumor. We review the published literature on gangliocytic paragangliomas pursuing a malignant course. We conclude that surgical therapy of these neoplasms should not be limited to local resection, as disease recurrence, lymph node involvement, and rarely distant metastasis may occur.

Emergency catheter probe endoscopic sonography (CP-EUS): an effective procedure in the assessment of a bleeding gangliocytic paraganglioma.Dig Liver Dis. 2006 Feb;38(2):134-7. Epub 2006 Jan 18.

Catheter probe endoscopic sonography is a relatively rapid and safe procedure, carried out during standard endoscopy, capable of distinguishing solid from cystic lesions and vascular from avascular masses. Herein we discuss the role of catheter probe endoscopic sonography in the emergency assessment of a patient with recent and severe bleeding from an ulcerated polyp, arising from the papilla. During the endoscopy, catheter probe endoscopic sonography showed the solid and submucosal nature of the lesion, suggesting its localised and benign nature and, most importantly, demonstrating the high risk of rebleeding from vascular structures communicating with the ulcer. Because of this finding three metallic endoclips were positioned. The lesion was not removed endoscopically because of its difficult position and the high risk of haemorrhage. The mass, removed through a duodenotomy, was shown to be a gangliocytic paraganglioma, an uncommon tumour, frequently resulting in surgery because of abdominal pain and gastrointestinal bleeding.

 
March 2008 

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