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Polycystic Disease of the Pancreas: a Case Report and Comprehensive Review of the Literature

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Abstract

Isolated polycystic disease of the pancreas is a rare condition with few reported cases in the literature. It refers to multiple dysontogenetic, or congenital, cysts spanning the pancreas excluding those with inherited autosomal cystic diseases of abdominal organs, cystic fibrosis, and Von Hippen-Lindau disease with or without the involvement of cysts in other organs. These cystic lesions prove difficult to distinguish from those of neoplastic nature, including but not limited to intraductal papillary mucinous neoplasms, mucinous cystic neoplasms, and serous cystic neoplasms, potentially leading to unnecessary treatment. We report an incidental finding of the polycystic pancreas in a 73-year-old male upon CT for diverticulitis without any pancreas-related symptoms. A comprehensive collection of all reported cases of the isolated polycystic pancreas in the literature to date revealed that we report the oldest known patient to be diagnosed, as all other patients are between ages 13–62 with 64% being female. A thorough evaluation of the literature was completed and a diagnostic algorithm to diagnose IPDP was created to assist in the appropriate evaluation of these benign lesions to reduce the morbidity associated with unnecessary surgeries prompted by diagnostic uncertainty and the possibility of pre-malignant lesions.

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Authors and Affiliations

  1. University of South Carolina School of Medicine Greenville, Greenville, SC, USA

    M. Little & S. Desai

  2. Pathology Associates, Greenville, SC, USA

    C. Schammel, D. P. Schammel & C. M. Wolff

  3. Department of Radiology, Prisma Health Upstate, Greenville, SC, USA

    A. M. Devane

  4. Department of Surgery, GI Oncology, Prisma Health Upstate, Greenville, SC, 29605, USA

    Steven D. Trocha

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  1. M. Little
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  2. S. Desai
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  3. C. Schammel
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  4. D. P. Schammel
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  5. C. M. Wolff
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Correspondence to Steven D. Trocha.

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Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Patient approval was obtained by universal consent and this retrospective study was approved by the Prisma Health Institutional Review Board and documented on Health Sciences South Carolina (Pro00103421)

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Little, M., Desai, S., Schammel, C. et al. Polycystic Disease of the Pancreas: a Case Report and Comprehensive Review of the Literature. SN Compr. Clin. Med. 3, 1053–1061 (2021). https://doi.org/10.1007/s42399-021-00791-w

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