A 76-year-old Caucasian man presented to our institution with
progressive fatigue and weight loss. He had undergone
esophagogastroduodenoscopy 1 year previously, which suggested a large duodenal
ulcer [1]
[2].
Laboratory data showed abnormal liver function tests consistent with
obstructive jaundice. Magnetic resonance cholangiopancreatography (MRCP) showed
dilatation of the main pancreatic duct (MPD) and biliary tree, and a
multilobular cystic lesion adhering to the duodenal wall ([Fig. 1]).
Fig. 1 Multilobular cystic
lesion (arrows) adhering to the duodenal wall, seen at magnetic resonance
imaging (MRI): a the cystic lesion (red arrows);
b the cystic lesion (red arrows) and common biliary duct
dilatation (purple arrow).
Esophagogastroduodenoscopy revealed a large crater (3 cm
wide) in the posterior wall of the duodenal bulb, giving a massive mucinous
discharge ([Fig. 2], [Video 1]).
Fig. 2 Endoscopic view of a
large crater full of mucus on the posterior wall of the duodenal bulb.
Qualität:
Video
1 Endoscopic view of a large
crater on the posterior wall of the duodenal bulb producing a massive mucinous
discharge.
Biopsies were taken from the edges of the lesion, and histological
findings showed tubulovillous adenoma with high-grade dysplasia ([Fig. 3]).
Fig. 3 Histological findings
revealing glandular adenoma with high-grade dysplasia (black arrow) and
mucinous cells with minor atypia (red arrow).
Subsequent endoscopic ultrasound (EUS) showed a marked diffuse
dilatation of the MPD, which contained echogenic material compatible with
mucus, and intraductal papillary vegetations. At the level of the isthmus there
was a wide communication between the MPD and a large solid and cystic lesion
(8 × 6 cm) adhering to the duodenal wall ([Fig. 4]).
Fig. 4 Endoscopic ultrasound
(EUS) showing the main pancreatic duct (red arrow) interruption that
communicates with a massive solid and cystic lesion (purple arrows)
(8 × 6 cm) adhering to the duodenal wall.
A disruption of the parietal layers of the bulb was also
demonstrated, consistent with a fistula between the lesion and the bulb
(corresponding to the endoscopic finding) ([Fig. 5], [Video 2]).
Fig. 5 Endoscopic ultrasound
(EUS) showing a fistula (arrow) between the cystic cavity and duodenum.
Qualität:
Video
2 Endoscopic ultrasound (EUS)
showing the main pancreatic duct interruption that communicates with a massive
solid and cystic lesion adhering to the duodenal wall, and fistula between the
cystic cavity and duodenum.
These findings were suggestive of a main-duct malignant intraductal
papillary mucinous neoplasm (IPMN) with mixed solid and cystic degeneration
(typical of the disease), fistulizing into the duodenum. Because of several
co-morbidities, the patient was not a candidate for surgery and was referred
for palliative care.
Fewer than 100 cases have been reported of IPMN forming fistulas in
surrounding organs [1]. The proposed pathogenesis
comprises mechanical compression from the cystic mass and/or direct neoplastic
infiltration. The majority of these cases have been documented with computed
tomography or MRCP, while EUS has rarely been described [2]. In our case, EUS was a useful adjunct in the diagnostic
work-up allowing direct visualization of the fistula tract and clarifying the
nature of the pancreatic disease.
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