Premalignant and Malignant Lesions of the Heterotopic Pancreas in the Esophagus: a Case Report and Review of the Literature

Abstract

Heterotopic pancreas is a congenital pathology of the gastrointestinal tract, particularly rare in the esophagus. Both symptomatology and findings during preoperative examinations are non-specific and therefore do not often lead to an accurate diagnosis, which is usually revealed only by histopathological assessment of a resected specimen. We report an unusual case of a patient suffering from severe dysphagia caused by heterotopic pancreas in the distal esophagus with chronic inflammation and foci of premalignant changes. This article also reviews 14 adult cases of heterotopic pancreas in the esophagus previously reported in the literature, with the aim of determining the clinical features of this disease and possible complications including rare premalignant lesions and malignant transformation. Especially with regard to those complications, we suggest that both symptomatic and incidentally found asymptomatic lesions should be resected.

Intestinal Heterotopic Pancreas Involved by Simultaneous PanIN-2 Lesion and Endocrine Microadenoma: a Unique Case

“…26 No 2: 199-202 by Zhang et al [5]. The present case was not associated with chronic pancreatitis, and morphologically the pancreatic ectopic tissue displayed papillary proliferation in a dilated duct, with moderate atypia, compatible with PanIN-2 diagnosis, which is the most frequent type encountered [3,13,15]. For the differential diagnosis of the PanIN lesions, IPMN should be considered.…”

“…Generally, it is supposed that all the pathologic conditions that affect the normal pancreas may be seen in HP, such as acute/chronic pancreatitis, pseudocystic changes, intraductal papillary mucinous neoplasm (IPMN), intraepithelial lesions or pancreatic neuroendocrine tumors being described [10], but malignant transformation is exceedingly rare [2 ,9, 12]. In the rare cases of PanIN lesions reported in the HP [3,5,[13][14][15], the premalignant changes seemed to occur with approximately the same incidence in HP as in the orthotopic pancreas [13]. The progression model from low-grade intraepithelial lesions (PanIN-1) to ductal adenocarcinoma in HP was demonstrated At the level of a dilated duct (3.6 mm), with predominantly flat ductal-type epithelium, a focal intraductal papillary proliferation with a pseudostratified epithelium was seen, composed of columnar cells, with variable mucin production, round to oval nuclei, predominantly basally located, having some atypia (focal crowding, loss of polarity, enlarged and hyperchromic, some nucleolated) but without atypical mitotic figures (Fig.…”

Interventional endoscopic ultrasound for a symptomatic pseudocyst secondary to gastric heterotopic pancreas

“…HP is most frequently located in the stomach (25%-38%), followed by the duodenum (17%-36%) and the jejunum (15%-21.7%); it is rarely found in the esophagus, common bile duct, spleen, mesentery, mediastinum or fallopian tube[ 4 ]. In the gastrointestinal tract, HP is most frequently located only in the submucosa (54%) and is less frequently found in both the submucosa and muscularis propria (23%), only the muscularis propria (8%), the subserosa (11%), and the whole wall (4%)[ 1 ].…”

“…Heterotopic pancreas (HP) is defined as the presence of pancreatic tissue in an abnormal location without anatomical or vascular connection to the orthotopic pancreas. HP was first described by Jean-Schultz in 1729 and was confirmed histologically by Klobb[ 1 ]. Other terms for HP, such as ectopic pancreas, aberrant pancreas, and accessory pancreas, have been used in the literature[ 2 ].…”

“…Other terms for HP, such as ectopic pancreas, aberrant pancreas, and accessory pancreas, have been used in the literature[ 2 ]. In most cases, HP is asymptomatic and found incidentally[ 1 ], but it can occasionally produce non-specific symptoms that depend on its location, size, and types of complications. Here, we describe a case involving a 40-year-old man with gastric outlet obstruction secondary to heterotopic pancreatitis with pseudocyst formation.…”