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

Table 1 Summary of patients with gangliocytic paraganglioma with metastases

Ref.	Year of publication	Age at diagnosis (yr)	Sex	Presenting symptoms	Primary location	Largest diameter, primary (mm)	Site(s) of metastasis	LNs sampled	LNs positive	Therapy	Follow-up (mo)
Büchler et al[7]	1985	50	M	GI bleeding	D2, ampulla	30	Peripancreatic LN	NR	1	Local resection	20, NED
Korbi et al[24]	1987	73	F	GI bleeding, weight loss, cardiac decompensation	D2, ampulla	90	Peripancreatic LN	NR	1	PD	0, died POD 7
Inai et al[4]	1989	17	M	GI bleeding	D2, ampulla	20	Peripancreatic LN	NR	1	Local resection, followed by PD with LND	32, NED
Hashimoto et al[20]	1992	47	M	Asymptomatic, incidental	D2, ampulla	65	Peripancreatic LN	NR	1	PD with LND	14, NED
Dookhan et al[5]	1993	41	M	Abdominal pain, partial duodenal obstruction	D2	25	Mesentery, mesenteric LNs	NR	2-3	Local resection (1981); resection D4, proximal jejunum, mesenteric mass (1992)	131, recurrence and metastasis
Takabayashi et al[33]	1993	63	F	Abdominal pain	D3	32	Regional LN	NR	1	PPPD	24, NED
Tomic et al[22]	1996	74	M	Anemia, steatorrhea, abdominal pain, weight loss	Pancreas, head	40	Peripancreatic LN	NR	1	PD	19, NED
Henry et al[6]	2003	50	M	Jaundice	Pancreas, head	30	Manubrium	NR	0	FNA1, followed by PD, followed by manubrium resection	21, NED
Sundararajan et al[1]	2003	67	F	Asymptomatic, incidental	D2	50	Regional LNs	NR	2	PD with LND	9, NED
Wong et al[23]	2005	49	F	GI bleeding, abdominal pain	D2, periampullary	14	Periduodenal and Peripancreatic LNs	7	6	PPPD with LND, radiotherapy	12, NED
Witkiewicz et al[2]	2007	38	F	Abdominal pain	D2, periampullary	15	Regional LNs	7	2	Local resection, followed by PPPD	NR
Mann et al[8]	2009	17	F	Duodenal obstruction, weight loss, abdominal pain	D2/D3 junction		Regional LNs	11	4	PD	7, NED
Okubo et al[9]	2010	61	M	GI bleeding, abdominal pain	D2, ampulla	30	Regional LNs	NR	1	PPPD with LND	6, NED
Saito et al[10]	2010	28	M	GI bleeding	D2, ampulla	17	Regional LNs	N/A	2	Local resection, followed by PD	N/A
Uchida et al[11]	2010	67	F	Anemia	D2	N/A	LN	N/A	N/A	PD	N/A
Ogata et al[27]	2011	16	M	GI bleeding	D2, ampulla	35	Peripancreatic LNs	NR	4	PPPD with LND	36, NED
Barret et al[12]	2012	51	F	GI bleeding	D2, ampulla	25	Peripancreatic LNs	NR	2	FNA, followed by PD	96, NED
Rowsell et al[13]	2011	52	F	Asymptomatic, incidental	D2, periampullary	10	Regional LNs, liver nodule	23	2	PD, post-op octreotide injections	27, No change in residual liver metastases
Dustin et al[14]	2011	56	F	Abdominal pain, weight loss	D2, periampullary	18	Retroperitoneal LN, later resection Peripancreatic LNs	10	3	Local resection of retroperitoneal mass, followed by duodenal mass FNA, followed by PPPD with LND and cholecystectomy	NR
Fiscaletti et al[25]	2011	61	M	Abdominal pain, weight loss	D2, minor papilla (discovered incidentally)	15	Peripancreatic LN	7	1	FNA2, followed by PPPD	12, NED
Amin et al[15]	2013	57	M	Abdominal pain, vomiting	D2, ampulla	30	Portal hepatic LNs, Liver	NR	NR	Resection of duodenal mass, retropancreatic mass, part of hepatic lesion, enlarged portal lymph nodes	8, Residual liver lesion slowly enlarging
Choi et al[21] (poster)	2014	41	M	GI bleeding	D2	25	Periduodenal LN	11	1	PD	NR
Li et al[16]	2014	47	M	Abdominal pain	D2, periampullary	30	Regional LNs, pelvic cavity, liver	16	7	PD, radiotherapy, chemotherapy	13, died secondary to liver and pelvic metastases
Micev et al[30] (poster)	2014	57	M	Abdominal pain, back pain, intermittent jaundice	D2, ampulla	35	Regional LNs	NR	2	NR	NR
Shi et al[17]	2014	47	M	Abdominal pain, weight loss	D2, ampulla	40	Regional LNs	20	8	PD with LND	24, NED
Dowden et al[32]	2015	59	F	Abdominal pain, weight loss	D2, ampulla	28	Regional LNs	22	2	FNA, followed by PPPD	5, NED
Lei et al[19]	2015	45	M	GI bleeding, weight loss, vomiting and diarrhea, abdominal cramps (functional tumor)	D2	15	Periduodenal LN	NR	1	FNA, followed by ampullectomy with periduodenal and retropancreatic LND	3, functional symptoms and CT showing lymphadenopathy, lost to follow-up
Sun et al[26] (poster)	2015	40	F	Abdominal pain	D2, ampulla	20	Peripancreatic LN	NR	13	FNA, followed by PD	12, NED
Wang et al[18]	2015	49	M	Abdominal pain	D2	33	Regional LNs	9	3	PD with LND, chemotherapy	36, NED
Hu et al[31]	2016	65	M	GI bleeding	D2	30	LN	NR	1	Local resection	2, NED
Current case	2016	62	M	Asymptomatic, incidental	D2, periampullary	20	Regional LNs	8	3	FNA, PD	30, NED

¹Suggestive of ductal adenocarcinoma;

²Fibroinflammatory changes consistent with pancreatitis;

³Involved by direct extension and replacement. GI: Gastrointestinal; D2: Duodenum 2^nd portion; D3: Duodenum 3^rd portion; LN: Lymph node; LND: Lymph node dissection; PD: Pancreaticoduodenectomy; PPPD: Pylorus-preserving pancreaticoduodenectomy; NED: No evidence of disease; N/A: Not available; NR: Not reported; LtFU: Lost to follow-up.

Table 2 Pathologic features of gangliocytic paragangliomas found to have metastases

General	Rare tumor of uncertain origin and low malignant potential, composed of epithelioid cells, spindle cells, and ganglion-like cells
Clinical features	Most often 5th-7th decade of life
	Most often abdominal pain or gastrointestinal bleeding
Gross findings	90% occurring in second portion of duodenum
	10-90 cm in greatest dimension (average 30 cm)
Cytologic findings	Typically cellular specimen
	Epithelioid cells predominate
	All three components may be present
Histologic findings	Epithelioid cells, spindle-like/sustentactular cells, and ganglion-like cells
	Submucosal
	Unencapsulated
	Necrosis absent
	No to rare mitoses
	Frequent extension beyond submucosa and/or lymphovascular invasion
	Metastases: 75% of those reported demonstrated all three cellular components; 25% predominantly epithelioid

Table 3 Treatment recommendations for a duodenal gangliocytic paraganglioma

Ampullary/ periampullary mass	EUS with FNA to rule out pancreatic adenocarcinoma, followed by pancreaticoduodenectomy with resection of suspicious lymph nodes
Duodenal mass, away from pancreas	CT to evaluate disease extent +/- FNA with local resection of primary tumor and suspicious lymph nodes if tumor location permits
Complete resection unattainable and/or surgically unfit candidate	Imaging modality + FNA/biopsy to establish diagnosis, octreotide scan, and trial of medical management with somatostatin analogues
	Tumor debulking should be attempted if surgically fit

EUS: Endoscopic ultrasound; CT: Computed tomography; FNA: Fine needle aspiration.