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Neoplastic pancreatic cysts

Background

Overview

Definition
Neoplastic pancreatic cysts are abnormal, fluid-filled sacs in the pancreas that have the potential to develop into tumors.
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Pathophysiology
Neoplastic pancreatic cysts are usually caused by genetic mutations and abnormal cell growth. Mutations in genes such as KRAS and GNAS have been detected in pancreatic fluid samples.
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Epidemiology
The incidence of neoplastic pancreatic cysts in the US is estimated at 31.3 per 100,000 person-years.
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Risk factors
Risk factors for pancreatic cysts include advancing age, male sex, tobacco use, familial history of pancreatic cancer, and specific genetic syndromes.
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Disease course
Clinical manifestations include symptoms like abdominal pain, jaundice, unexplained weight loss, and new-onset diabetes. However, many of these cysts are asymptomatic and are incidentally discovered on routine cross-sectional imaging.
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Prognosis and risk of recurrence
The prognosis varies depending on their biological behavior and potential for progression to malignancy. Routine surveillance of pancreatic cysts without high-grade dysplasia or malignancy at surgical resection is not recommended, as these cysts have a lower risk of progression.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of neoplastic pancreatic cysts are prepared by our editorial team based on guidelines from the American College of Radiology (ACR 2020), the American College of Gastroenterology (ACG 2018), the American Society for Gastrointestinal Endoscopy (ASGE 2016), and the American Gastroenterological Association (AGA 2015).
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Screening and diagnosis

Clinical manifestations: as per ACG 2018 guidelines, be cautious when attributing symptoms to pancreatic cyst, recognizing that the majority of pancreatic cysts are asymptomatic and the non-specific nature of symptoms requires clinical discernment.
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Diagnostic investigations

Diagnostic imaging: as per ACR 2020 guidelines, obtain abdominal MRI without and with IV contrast with MRCP for initial evaluation of patients with an incidentally detected pancreatic cyst 2.5 cm in size or > 2.5 cm in size but with no high-risk stigmata or worrisome features.
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  • Further evaluation

Diagnostic procedures

FNA: as per ACG 2018 guidelines, perform EUS with or without FNA and/or refer to a multidisciplinary pancreatic group for further evaluation in patients with intraductal papillary mucinous neoplasms or mucinous cystic neoplasms with any of the following features:
Situation
Guidance
Symptoms or signs
Jaundice secondary to the cyst
Acute pancreatitis secondary to the cyst
Significantly elevated serum CA 19-9
Imaging findings
The presence of a mural nodule or solid component either within the cyst or in the pancreatic parenchyma
Dilation of the main pancreatic duct of > 5 mm
A focal dilation of the pancreatic duct concerning for main duct intraductal papillary mucinous neoplasms or an obstructing lesion
Intraductal papillary mucinous neoplasms and mucinous cystic neoplasms measuring ≥ 3 cm in diameter
Cytologic findings
High-grade dysplasia
Pancreatic cancer
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  • Cyst fluid analysis

Surgical interventions

Surgical resection: as per ACG 2018 guidelines, refer patients with a solid-pseudopapillary neoplasm to a multidisciplinary group for consideration of surgical resection.
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Patient education

General counseling: as per AGA 2015 guidelines, ensure that patients have a clear understanding of programmatic risks and benefits before starting any pancreatic cyst surveillance program.

Follow-up and surveillance

Surveillance imaging: as per ACR 2020 guidelines, obtain the following investigations for the follow-up imaging in patients with pancreatic cyst:
abdominal CT with IV contrast multiphase
abdominal MRI without and with IV contrast with MRCP
abdominal MRI without IV contrast with MRCP.
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  • Postoperative surveillance