Duodenal gangliocytic paraganglioma with lymph node metastases: a case report and comparative review of 31 cases

Abstract

Gangliocytic paraganglioma (GP) is a rare tumor of uncertain origin most often located in the second portion of the duodenum. It is composed of three cellular components: Epithelioid endocrine cells, spindle-like/sustentacular cells, and ganglion-like cells. While this tumor most often behaves in a benign manner, cases with metastasis are reported. We describe the case of a 62-year-old male with a periampullary GP with metastases to two regional lymph nodes who was successfully treated with pancreaticoduodenectomy. Using PubMed, EMBASE, EBSCOhost MEDLINE and CINAHL, and Google Scholar, we searched the literature for cases of GP with regional lymph node metastasis and evaluated the varying presentations, diagnostic workup, and disease management of identified cases. Thirty-one cases of GP with metastasis were compiled (30 with at least lymph node metastases and one with only distant metastasis to bone), with age at diagnosis ranging from 16 to 74 years. Ratio of males to females was 19:12. The most common presenting symptoms were abdominal pain (55%) and gastrointestinal bleeding or sequelae (42%). Twenty-five patients underwent pancreaticoduodenectomy. Five patients were treated with local resection alone. One patient died secondary to metastatic disease, and one died secondary to perioperative decompensation. The remainder did well, with no evidence of disease at follow-up from the most recent procedure (except two in which residual disease was deliberately left behind). Of the 26 cases with sufficient histological description, 16 described a primary tumor that infiltrated deep to the submucosa, and 3 described lymphovascular invasion. Of the specific immunohistochemistry staining patterns studied, synaptophysin (SYN) stained all epithelioid endocrine cells (18/18). Neuron specific enolase (NSE) and SYN stained most ganglion-like cells (7/8 and 13/18 respectively), and S-100 stained all spindle-like/sustentacular cells (21/21). Our literature review of published cases of GP with lymph node metastasis underscores the excellent prognosis of GP regardless of specific treatment modality. We question the necessity of aggressive surgical intervention in select patients, and argue that local resection of the mass and metastasis may be adequate. We also emphasize the importance of pre-surgical assessment with imaging studies, as well as post-surgical follow-up surveillance for disease recurrence.

Duodenal gangliocytic paraganglioma: A case report

“…In case of positive lymph nodes experts agree on the importance of doing a curative pancreaticoduodenectomy even though this operation carry its own risk of morbidity and mortality. Doing it otherwise will require further studies and long periods of surveillance [12].…”

Management of a rare cause of upper gastrointestinal bleed: the duodenal gangliocytic paraganglioma

“…The incidence of the GP with lymph node or distant organ metastasis is well documented in the literature 3 and with this in consideration, the informed choice of pancreaticoduodenectomy was made by the patient. Multivariate logistic regression by Okubo et al 1 .…”

Prognosis of duodenal gangliocytic paraganglioma with lymph node metastasis: is follow-up >5 years required?

“…According to the recent World Health Organization classification of gastrointestinal (GI) NETs in 2010, there is a debate whether to classify them either as low-grade NETs or as an independent entity depending on their clinical behavior and prognosis [ 3 ]. There were few reported cases of regional lymph node metastasis that could argue DGP is a true neoplasm [ 4 ]. However, majority of tumors had a benign course during the follow-up, raising the question of long-term follow-up.…”