Duodenal gangliocytic paraganglioma with lymph node metastasis and an 8-year follow-up

Abstract

Gangliocytic paraganglioma (GP) is a rare tumor, usually located in the second part of the duodenum. On pathological examination, GP is characterized by the association of the histological features of paragangliomas, ganglioneuromas, and carcinoid tumors. Classical clinical presentations are upper gastrointestinal bleeding and abdominal pain. Preoperative diagnosis is difficult because of the submucosal site of the tumor, with usually negative mucosal biopsies. Endoscopic ultrasound helps establish the diagnosis and allows lymph node staging, which will guide the choice of the treatment. If GPs usually follow a benign course, metastatic spread to regional lymph nodes treated by surgical resection alone has been reported. We report a case of GP with lymph node metastases treated by duodenopancreatectomy with long-term disease-free survival, suggesting that surgical resection is a reasonable approach for metastatic GPs.

A case of carotid body tumor concomitant with carcinoid tumor

“…Hence, it has the features of a ganglioneuroma, a paraganglioma, and a carcinoid tumor. Gangliocytic paraganglioma has been known to arise from endodermal neuroectodermal complex [11]. Similarly, paraganglioma and carcinoid tumor seem to coexist by similar mechanism.…”

Totally laparoscopic resection of a rare duodenal tumour

“…The vast majority of GPs is found in the duodenum (90.1%). However, GPs have also been reported at other sites along the gastrointestinal, respiratory and urogenital tracts, as well as in the scalp, vertebral column and mediastinum 4 5 11 13 14. Paragangliomas and phaeochromocytomas as a collective are attributed to hereditary and germline mutations in at least 30% of cases (mainly due to succinate dehydrogenase gene mutations), with 70% occurring sporadically.…”

“…Given their tendency to arise in the duodenum, GPs most commonly present as gastrointestinal bleeding, abdominal pain and anaemia. Biliary obstruction is considered extremely rare (4.6% of all reported GPs) 4 11. GPs are usually benign and non-functional.…”

“…Lymph node metastasis is uncommon and distant metastases to the liver, lung and bones have been rarely reported. Recent evidence suggests that metastasis is more likely in young patients and in patients with tumours exceeding the submucosal layer 4 11 13…”

Endoscopic Resection as a Possible Radical Treatment for Duodenal Gangliocytic Paraganglioma: A Report of Four Cases

“…2 Previous GP treatment algorithms based on tumor size and lymph node status have proposed that endoscopic removal could be considered if the tumor is small (＜2 cm) with no evidence of peritumoral lymph nodes on CT scan. 4 In terms of tumor size, we suggest that tumors larger than 2 cm can be treated with endoscopic resection. Previous reports have confirmed that endoscopic resection for GP larger than 2 cm can be performed without complication, 8,9 with the largest reported tumor measuring 4 cm in size.…”