title: Ampullary region carcinomas: definition and site specific classification with delineation of four clinicopathologically and prognostically distinct subsets in an analysis of 249 cases.
abstract: Ampullary (AMP) carcinomas comprise a heterogeneous group of cancers lacking adequate subcategorization. In the present study, 249 strictly defined primary AMP carcinomas (ACs) identified in 1469 malignant pancreatoduodenectomy specimens were analyzed for defining features. Gross and microscopic findings were used to determine tumor epicenter and extent of preinvasive component. ACs were classified into 4 distinct subtypes based on location: (1) Intra-AMP (25%): Invasive carcinomas arising in intra-ampullary papillary-tubular neoplasms with zero to minimal, duodenal surface involvement (<25% of the tumor). These tumors were more commonly found in men, they had a relatively large overall size (mean, 2.9 cm) but had smaller invasive component (mean, 1.5 cm), and were predominantly of a lower TNM stage (85%, T1/2; and 72% N0). They carried the best prognosis among the 4 groups (3-y survival, 73%). (2) AMP-ductal (15%): These were tumors forming constrictive, sclerotic, plaque-like thickening of the walls of the common bile duct and/or pancreatic duct resulting in mucosa-covered, button-like elevations of the papilla into the duodenal lumen. There was no significant exophytic (preinvasive) growth. These were the smallest tumors (mean overall size, 1.9 cm; mean invasion size 1.7 cm), but carried the worst prognosis (3-y survival, 41%), presumably due to the pancreatobiliary histology/origin (in 86%); however, even this group had significantly better prognosis when compared with 113 ordinary pancreatic ductal adenocarcinomas (3 y, 11%; P<0.0001). (3) Peri-AMP-duodenal (5%): Massive exophytic, ulcero-fungating tumors growing into the duodenal lumen and eccentrically encasing the ampullary orifice with only minimal intra-ampullary luminal involvement. These were mostly of intestinal phenotype (75%) and some had mucinous features. Although these tumors were the largest (mean overall size 4.7 cm; and mean invasion size 3.4 cm), and had the highest incidence of lymph node metastasis (50%), they carried an intermediate prognosis (3-y survival, 69%) to that seen among a group of 55 nonampullary duodenal carcinoma controls. (4) AC-not otherwise specified ("papilla of Vater"; 55%): Ulcero-nodular tumors located at the papilla of Vater, which do not show the specific characteristics identified among the other 3 subtypes. In conclusion, ACs comprise 4 clinicopathologic subtypes that are prognostically distinct.
authors.affiliation: Department of Pathology, Emory University School of Medicine, Atlanta, GA 30322, USA. volkan.adsay@emory.edu
authors.forename: Volkan
authors.initials: V
authors.lastname: Adsay
authors.forename: Nobuyuki
authors.initials: N
authors.lastname: Ohike
authors.forename: Takuma
authors.initials: T
authors.lastname: Tajiri
authors.forename: Grace E
authors.initials: GE
authors.lastname: Kim
authors.forename: Alyssa
authors.initials: A
authors.lastname: Krasinskas
authors.forename: Serdar
authors.initials: S
authors.lastname: Balci
authors.forename: Pelin
authors.initials: P
authors.lastname: Bagci
authors.forename: Olca
authors.initials: O
authors.lastname: Basturk
authors.forename: Sudeshna
authors.initials: S
authors.lastname: Bandyopadhyay
authors.forename: Kee-Taek
authors.initials: KT
authors.lastname: Jang
authors.forename: David A
authors.initials: DA
authors.lastname: Kooby
authors.forename: Shishir K
authors.initials: SK
authors.lastname: Maithel
authors.forename: Juan
authors.initials: J
authors.lastname: Sarmiento
authors.forename: Charles A
authors.initials: CA
authors.lastname: Staley
authors.forename: Raul S
authors.initials: RS
authors.lastname: Gonzalez
authors.forename: So Yeon
authors.initials: SY
authors.lastname: Kong
authors.forename: Michael
authors.initials: M
authors.lastname: Goodman
mesh_terms: D000230:Adenocarcinoma / Q000145:classification* / Q000401:mortality; D000368:Aged; D014670:Ampulla of Vater / Q000473:pathology*; D003138:Common Bile Duct Neoplasms / Q000145:classification* / Q000401:mortality; D004386:Duodenum / Q000473:pathology; D005260:Female; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D010190:Pancreatic Neoplasms / Q000145:classification*; D016577:Pancreaticoduodenectomy; D012737:Sex Factors; D015996:Survival Rate; D014481:United States / Q000453:epidemiology