Biliary tree gastrinomas in multiple endocrine neoplasia type 1 syndrome

Table 1 Characteristics, surgical treatment and follow-up of multiple endocrine neoplasia type 1 patients with biliary gastrinomas in our series

Pt	Sex/age (yr)	MEN1 mutation	Basal gastrinemia (NV < 108 pg/mL)	Previous surgical treatment	Surgical treatment	Postoperative complications	Latest complications and adverse events	Basal serum gastrin postoperative (pg/mL)	Secretin provocative test (at follow-up)
1	F/46	Missense, Leu413Pro exon 9	213.2	Total PTX	TP and removal of a bile duct gastrinoma		Insulin-dependentdiabetes mellitusCholangitis	39.9	NEG
2	F/55	Frameshift, 843del GA, exon 6	87.1	Left nephrectomy for hydronephrosis, total PTX with autotransplantation	WDP	Pancreatojejunal dehiscence for acute pancreatitis→TP with splenectomy	Abdominal abscess	24.8	NEG
3	F/37	Arg415 exon 9stop	204.3	Less than subtotal PTX	(2010) PPDP(2012) Hepatic resection (V-VIII seg) and enucleation of a pancreatic head nodule	Biliary fistula	Subocclusion	32.4	NEG

PTX: Parathyroidectomy; TP: Total pancreatectomy; WDP: Whipple’s duodenopancreatectomy; PPDP: Pylorus preserving duodenopancreatectomy; MEN1: Multiple endocrine neoplasia type 1.

Table 2 Pathological findings in multiple endocrine neoplasia type 1 patients with biliary gastrinomas

Pt	Gastrinomas (n)	Size of largest duodenal gastrinoma (cm)	Size of largest biliary gastrinoma (cm)	Other pancreatic endocrine neoplasms (n)	Size of largest NF PEN (cm)	Total tumors (n)	Hepatic findings	Gastrinoma metastases
1	2 (D, BD)	1.0	1.2	16 NF	3.4	18	Negative	Negative
2	4 (3D, 1G)	0.2	0.2	32 NF	1.6	32	Negative	Negative
3	3 (2D, 1BD)	0.3	1.5	37 NF	2.0	40	Negative	Positive (N)

D: Duodenal; BD: Biliary duct; G: Gallbladder; N: Lymph node; PEN: Pancreatic endocrine neoplasms; NF: Non-functioning.

Table 3 Clinicopathological characteristics of multiple endocrine neoplasia type 1 patients with extraduodenal-extrapancreatic gastrinomas reported in the literature

Ref.	Sex/age	Site/size (mm)	Gastrin: basal/stimulation	Diagnostic imaging positivity	MEN1 stigmata	Surgical treatment	Metastasis	BPC gastrin
Price et al[14]	F/55	CD (CBD junction)/6 mm	4500/NR	IOUS, discovered at surgery	pHPT, PA, NF-PETs (n°21/1-6 mm)	Distal splenopancreatectomy; RFA hepatic excision BC	Liver, (solitary)	780 (2 yr) (recurrence)
Price et al[14]	F/43	CBD/15 × 12 × 1	580/4300	IOUS, discovered at surgery	pHPT, PA, NF-PET (4), DG (3/1-4 mm); GC, lipoma	PPDP; enucleation; gastric excision	Lymph nodes (CBD HA)	86 (2 yr)
Lee et al[15]	F/39	Liver (II-III seg)/16 × 15 × 0.9	447/NR	CCT, SSRS, IOUS	pHPT, PA, insulinoma	WDP; II-III liver segmentectomy	-	34 (po)

pHPT: Primary hyperparathyroidism; PA: Pituitary adenoma; DG: Duodenal gastrinoma; GC: Gastric carcinoid; WDP: Whipple’s duodenopancreatectomy; PPDP: Pylorus preserving duodenopancreatectomy; D: Duodenal; CBD: Common bile duct; CD: Cystic duct; G: Gallbladder; N: Lymph node; F: Female.