Introduction

Intraductal Papillary Mucinous Neoplasm (IPMN) is the most frequent type of pancreatic cystic neoplasm found in the general population. Due to the advances in imaging technologies, the rate of detection of pancreatic cystic neoplasms has been increasing in the past years. A large-scale single-center cohort study conducted in Seoul revealed a prevalence of 1.7% in asymptomatic healthy individuals [1]. Polycystic liver disease (PLD) is a rare disorder characterized by the presence of at least 10 parenchymal cysts in the liver. It may be inherited in an autosomal dominant fashion and, depending on the most prominent phenotype, PLD can be an isolated manifestation in autosomal dominant polycystic liver disease (ADPLD) or it can be an extrarenal manifestation in autosomal dominant polycystic kidney disease (ADPKD) [2,3]. In ADPKD, cysts can be detected also in the pancreas, seminal vesicles and arachnoid membrane [4]. In literature there are isolated reports of association between Intraductal Papillary Mucinous Neoplasm and Autosomal Dominant Polycystic Kidney Disease [5,6].

Aims & Methods

A retrospective observational study was conducted to include all PLD patients followed at CEMAD (Centro Malattie Apparato Digerente) Fondazione Policlinico Universitario A. Gemelli to determine the prevalence of IPMNs in PLD patients (isolated or associated to ADPKD) using abdominal resonance imaging (MRIs) and/or abdominal CT scan, in order to evaluate whether IPMN can be a not acknowledged manifestation of PLD.

Results

Abdominal MRIs and/or abdominal CT scans were examined for 92 PLD patients (56 ADPKD and 36 isolated PLD). IPMNs were detected in 8 patients (8.7% of total), 4 with isolated PLD (11.7% of total isolated PLD patients) and 4 with PLD in ADPKD (7% of total ADPKD patients). As for the IPMN site, body-tail localization of the IPMN was the most frequent detected (50% of total IPMNs).

Conclusion

A high prevalence of IPMN in PLD patients was found, which seems to be higher than prevalence found in the general population (8.7% vs 1.7%). Moreover, IPMN appeared to be more frequent in isolated PLD than in PLD associated with ADPKD (11.1% vs 7%). Our results suggest we should consider the possibility of IPMN being another extrahepatic manifestation of PLD. Of course, further studies in a larger population of patients are needed in order to confirm our findings.

References

References:

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4. Harris PC, Torres VE. Polycystic Kidney Disease, Autosomal Dominant. 2002 Jan 10 [updated 2022 Sep 29]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, Gripp KW, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2024.

5. Sato Y, Mukai M, Sasaki M, Kitao A, Yoneda N, Kobayashi D, Imamura Y, Nakanuma Y. Intraductal papillary-mucinous neoplasm of the pancreas associated with polycystic liver and kidney disease. Pathol Int. 2009 Mar;59(3):201-4.
   

6. Naitoh H, Shoji H, Ishikawa I, Watanabe R, Furuta Y, Tomozawa S, Igarashi H, Shinozaki S, Katsura H, Onozato R, Kudoh M. Intraductal papillary mucinous tumor of the pancreas associated with autosomal dominant polycystic kidney disease. J Gastrointest Surg. 2005 Jul-Aug;9(6):843-5.