Malignant Transformation of Ectopic Pancreas

• Published in: Digestive diseases and sciences Vol. 64; no. 3; pp. 655 - 668
• Main Authors: Cazacu, Irina M., Luzuriaga Chavez, Adriana Alexandra, Nogueras Gonzalez, Graciela M., Saftoiu, Adrian, Bhutani, Manoop S.
• Format: Journal Article
• Language: English
• Published: New York Springer US 01-03-2019; Springer; Springer Nature B.V
• Subjects: Adenocarcinoma - mortality; Adenocarcinoma - pathology; Adenocarcinoma - therapy; Biochemistry; Cell Transformation, Neoplastic - pathology; Choristoma - mortality; Choristoma - pathology; Choristoma - therapy; Gastroenterology; Hepatology; Humans; Intestinal Neoplasms - mortality; Intestinal Neoplasms - pathology; Intestinal Neoplasms - therapy; Medical prognosis; Medicine; Medicine & Public Health; Oncology; Pancreas; Pancreatic cancer; Pancreatic Neoplasms - mortality; Pancreatic Neoplasms - pathology; Pancreatic Neoplasms - therapy; Prognosis; Review; Risk Factors; Stomach Neoplasms - mortality; Stomach Neoplasms - pathology; Stomach Neoplasms - therapy; Transplant Surgery; Tumors; Heterotopic pancreas; Ectopic pancreas; Ectopic pancreatic cancer, cancer, malignant transformation
• ISSN: 0163-2116; 1573-2568
• DOI: 10.1007/s10620-018-5366-z

Malignant transformation of ectopic pancreas tissue is a diagnostic challenge as clinical symptoms and radiographic features of these tumors are non-specific. Given the rarity of these lesions, it is usually neither suspected nor included in the diagnostic workup of different tumors. We conducted a comprehensive literature review regarding malignancy arising from ectopic pancreas for a better understanding of its frequency, clinicopathological features, and prognosis. A literature search was performed in three major databases: PubMed, Cochrane, and Web of Science. Fifty-four well-documented cases of malignant ectopic pancreas were identified in the published literature. Our analysis provided the following observations: (1) there was a slight predominance of males over females; (2) most patients with malignant transformation of ectopic pancreas were middle-aged; (3) most commonly, the tumor was located in the stomach; (4) most tumors were adenocarcinomas; (5) most frequently, the malignancy arose within a type I heterotopia according to Heinrich classification; (6) macroscopically, a subepithelial-like appearance was most frequently observed; and (7) improved prognosis for ectopic pancreatic malignancies in comparison with reported survival data for orthotopic pancreatic cancer. Even if the majority of cases of ectopic pancreas are incidental findings and malignant transformation is a rare event, pancreatic heterotopy should be considered as a source of potentially malignant lesions.