Multifocal/diffuse pancreatic serous cystic neoplasms: Systematic review with a new case

Pancreatology. 2020 Jul;20(5):902-909. doi: 10.1016/j.pan.2020.04.015. Epub 2020 May 5.


Background/objectives: Pancreatic cystic neoplasms (PCNs) are common, among which 13%-23% are serous cystic neoplasms (SCNs). However, diffuse and multifocal variants of SCNs are extremely rare. The differential diagnosis of SCNs from other PCNs is important as the former entities are benign and do not become invasive.

Objective: This study analyzes the clinical characteristics of multifocal/diffuse SCN through a systematic review of the literature and a case report.

Methods: A comprehensive literature search was executed in the Ovid MEDLINE, Embase, and Google Scholar databases. The search strategy was designed to capture the concept of multifocal/diffuse SCN cases with sufficient clinical information for detailed analysis. Using the final included articles, we analyzed tumor characteristics, diagnostic modalities used, initial management and indications, and patient outcomes.

Results: A review of 262 articles yielded 19 publications with 22 cases that had detailed clinical information. We presented an additional case from our institution database. The systematic review of 23 cases revealed that the diffuse variant is more common than the multifocal variant (15 vs 8 cases, respectively). Patients were managed with surgical intervention, conservative treatment, or conservative treatment followed by surgical intervention. Indications for surgery following conservative management mainly included new onset or worsening of symptoms. Only one case reported significant tumor growth after attempting an observational approach. No articles reported recurrence of SCN after pancreatectomy, and no articles reported mortality related to multifocal/diffuse SCNs.

Conclusion: Despite their expansive-growing and space-occupying characteristics, multifocal/diffuse SCNs should be treated similarly to their more common unifocal counterpart.

Keywords: Diffuse; Microcystic adenoma; Multifocal; Pancreas; Serous cystadenoma; Serous cystic neoplasm.

Publication types

  • Meta-Analysis
  • Systematic Review

MeSH terms

  • Adenoma / epidemiology*
  • Adenoma / pathology
  • Adenoma / therapy
  • Cystadenocarcinoma, Serous / epidemiology*
  • Cystadenocarcinoma, Serous / pathology
  • Cystadenocarcinoma, Serous / therapy
  • Cystadenoma, Serous / epidemiology*
  • Cystadenoma, Serous / pathology
  • Cystadenoma, Serous / therapy
  • Humans
  • Pancreatic Neoplasms / epidemiology*
  • Pancreatic Neoplasms / pathology
  • Pancreatic Neoplasms / therapy