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Malignant Transformation of Ectopic Pancreas

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Abstract

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.

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Acknowledgments

Dr. Irina M. Cazacu is supported by 2017 International Travel Grant offered by the American College of Gastroenterology.

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Informed consent was not applicable to this article type. This is a literature review of all of the cases of malignant ectopic pancreas that have already been published.

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Cazacu, I.M., Luzuriaga Chavez, A.A., Nogueras Gonzalez, G.M. et al. Malignant Transformation of Ectopic Pancreas. Dig Dis Sci 64, 655–668 (2019). https://doi.org/10.1007/s10620-018-5366-z

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