PMC10237316_01
(A) CT axial view revealed a complex mass with cystic component and irregular solid part at S3 of liver. The mass was found to be invading gastric body directly (arrowhead). In addition, there was marked dilatation of right IHDs. (B) Magnetic resonance cholangiopancreatography (MRCP) demonstrated diffuse dilatation of intrahepatic and extra-hepatic bile ducts, likely caused by mucous accumulation, which is a signature of mucin-producing IPNB. The images clearly showed the biliary-gastric fistulae (indicated by arrowhead and asterisk). Notably, intraluminal filling defects were found in left IHD and CBD, which may represent intraluminal papillary tumors or mucobilia. However, intraoperative choledochoscopy ruled out papillary tumor growth in the CBD.
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Presence of a complex mass at segment 3 with poorly enhancing mural nodules and indistinct margin between liver and gastric body ( Figure 1A ). Magnetic resonance imaging (MRI) showed marked dilatation of left IHDs with intraluminal soft tissue filling defect and fistulous connections to gastric high body. ( Figure 1B ).
Upper GI barium study showed incomplete distention of lesser curvature side of gastric high body with mucosal irregularities, as well as two spiculations (arrowhead), probably related to fistula tracts. No definite extravasation of barium meal was found during the study.
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Upper gastrointestinal series demonstrated irregular mucosa with spiculated foci at lesser curvature side of upper gastric body ( Figure 2 ).
Endoscopy demonstrated two ulcers at lesser curvature side of the anterior wall of gastric high body near cardia. Upon closer evaluation, suspicious fistulous orifices exude mucin content.
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Gastroduodenoscopy revealed ulcers at lesser curvature side of upper gastric body, with exuding whitish mucous ( Figure 3 ).
(A) Surgical specimen: A direct fistulous connection was found between left IHD to stomach. To confirm this, a forceps was inserted from the orifice of stomach (indicated by "G") to left IHD (indicated by "H"). Several intraductal papillary tumors were found from the cut surface of left IHD (arrowhead). (B) Macroscopic findings of the cut surface: the specimen contains left hepatectomy (on the right) and wedge gastrectomy (on the left), with numerous whitish intraductal papillary excrescences and a hepatogastric fistula. Invasive carcinoma with periductal infiltrating growth pattern around the fistula track is noted. (C) Photomicrograph (hematoxylin and eosin stain, 2x objective lens) shows an intraductal polypoid neoplasm with florid proliferation of papillary fronds, which was characterized by fibrovascular cores covered by mixed intestinal and gastric-type epithelia. (D) Photomicrograph (hematoxylin and eosin stain, 4x objective lens) of the fistula (gastric end): Along the fistulous tract are mucin admixed with papillary fronds of tumor cells and neoplastic glands infiltrating in a desmoplastic stroma. Gastric mucosa is present on the right upper corner.
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Papillary tumors were also found on the cut surface of left IHD ( Figure 4A ). Macroscopically, the specimen demonstrated a mucinous tumor in left IHD with fistulous connection to gastric high body ( Figure 4B ). Microscopic histologic exam showed intraductal papillary neoplasm of bile duct ( Figure 4C ) with associated cholangiocarcinoma, as well as direct transmural invasion to gastric wall with fistula formation ( Figure 4D ).