Pancreatic Cystic Neoplasms: Management and Unanswered Questions

doi:10.1053/j.gastro.2013.01.073

Gastroenterology

Volume 144, Issue 6, May 2013, Pages 1303-1315

https://doi.org/10.1053/j.gastro.2013.01.073 Get rights and content

Approximately 10% of persons 70 years old or older are now diagnosed with pancreatic cysts, but it is not clear which ones require additional analysis, interventions, or follow-up. Primary care doctors rely on gastroenterologists for direction because no one wants to miss a diagnosis of pancreatic cancer, but meanwhile there is pressure to limit use of diagnostic tests and limit costs. We review the different cystic neoplasms of the pancreas and diagnostic strategies based on clinical features and imaging data. We discuss surgical and nonsurgical management of the most common cystic neoplasms, based on the recently revised Sendai guidelines. Intraductal papillary mucinous neoplasm (particularly the branch duct variant) is the lesion most frequently identified incidentally. We report what is known about its pathology, its risk of developing into pancreatic ductal adenocarcinoma, the pros and cons of current guidelines for management, and the potential role of endoscopic ultrasound in determining cancer risk. We also review surgical treatment and strategies for surveillance of pancreatic cysts.

Section snippets

Mucinous Cystic Neoplasms

Mucinous cystic neoplasms (MCNs) are relatively uncommon tumors that comprise about 25% of all resected cystic neoplasms of the pancreas, based on data from a large surgical series.³ They occur most frequently in women (>95%), in the distal pancreas (>95%), and, unlike BD-IPMNs, are always a single lesion.4, 5, 6 They are characterized by a dense, ovarian-like stroma that surrounds the tumor, and an inner-epithelial layer with tall, mucin-producing cells. This layer can exhibit various degrees

BD-IPMNs

BD-IPMNs account for most of the increasingly recognized, asymptomatic, incidental pancreatic cysts.² They can occasionally be symptomatic, in that some patients present with pancreatitis. Their imaging features range from an isolated subcentimeter pancreatic cyst to larger multicentimeter solitary collections of pancreatic cysts. With improved imaging and pathology assessments, BD-IPMNs are now also recognized as a diffuse multifocal disease, with 21%–41% of patients having multiple BD-IPMNs

Role for EUS in Diagnosis and Treatment

Before evaluating a CNP by EUS, EUS with FNA, and cyst fluid aspiration, the likely effects on diagnosis, management, and patient outcomes should be determined. This is best done in a multidisciplinary setting after discussion of the patient's presentation and operative risk, as well as expert review of high-quality CT or MRI data.⁶⁷

CT and MRI alone can be used in the diagnosis and management of CNP when certain features are present. For example, a microcystic pancreatic lesion with a central

Surgery

Surgical resection is the treatment of choice for symptomatic patients with cystic neoplasms of the pancreas and for those with incidentally discovered tumors that have a high likelihood of malignancy, such as mucinous cystic neoplasms, CPENs, SPNs, and main and combined duct IPMNs. The type of resection depends on the location of the lesion. In a recent series composed of 851 resected cystic tumors, 44% required distal pancreatectomy, 43% required pancreatoduodenectomy (Whipple procedure), and

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Cited by (168)

Surveillance of Pancreatic Cystic Neoplasms
2023, Gastrointestinal Endoscopy Clinics of North America
Gα13 loss in Kras/Tp53 mouse model of pancreatic tumorigenesis promotes tumors susceptible to rapamycin
2022, Cell Reports
Gα13 transduces signals from G-protein-coupled receptors. While Gα13 functions as a tumor suppressor in lymphomas, it is not known whether Gα13 is pro-tumorigenic or tumor suppressive in genetically engineered mouse (GEM) models of epithelial cancers. Here, we show that loss of Gα13 in the Kras/Tp53 (KPC) GEM model promotes well-differentiated tumors and reduces survival. Mechanistically, tumors developing in KPC mice with Gα13 loss exhibit increased E-cadherin expression and mTOR signaling. Importantly, human pancreatic ductal adenocarcinoma (PDAC) tumors with low Gα13 expression also exhibit increased E-cadherin expression and mTOR signaling. Treatment with the mTOR inhibitor rapamycin decreases the growth of syngeneic KPC tumors with Gα13 loss by promoting cell death. This work establishes a tumor-suppressive role of Gα13 in pancreatic tumorigenesis in the KPC GEM model and suggests targeting mTOR in human PDAC tumors with Gα13 loss.
Association between pancreatic intraductal papillary mucinous neoplasms and extrapancreatic malignancies: A systematic review with meta-analysis
2022, European Journal of Surgical Oncology
It is unclear whether patients with intraductal papillary mucinous neoplasia harbor a higher risk of developing extrapancreatic malignancies.
We performed a pooled estimate of the incidence of extrapancreatic malignancies in patients with intraductal papillary mucinous neoplasia, with a particular focus on the comparison to the general population.
Computerized bibliographic search of main databases was performed through February 2021. The primary endpoint was the pooled incidence of extrapancreatic malignancies in patients with intraductal papillary mucinous neoplasms. Additional outcome was the comparison between intraductal papillary mucinous neoplasia patients and the general population, expressed in terms of standardized incidence ratio along with 95% confidence intervals.
Eighteen studies with 8709 patients were included. The pooled rate of metachronous extrapancreatic malignancies was 10 (6–13)/1000 persons-year. No difference was observed according to intraductal papillary mucinous neoplasia histology and sex, whereas a significantly superior incidence of extrapancreatic malignancies was observed in patients with main-duct (36.7%, 25.4%–48%) as compared to branch-duct intraductal papillary mucinous neoplasia (26.2%, 17.6%–34.8%; p = 0.03). Pooled standardized incidence ratio comparing expected rates in the general population was 1.01 (0.79–1.29); no difference was observed concerning rates of metachronous gastric cancer (standardized incidence ratio 1.60, 0.72–3.54) and colorectal cancer (1.29, 0.92–1.18), whereas biliary cancer was observed more frequently in intraductal papillary mucinous neoplasia patients (2.29, 1.07–4.93).
Patients with intraductal papillary mucinous neoplasia harbor an overall rate of extrapancreatic malignancies as high as 27.3%. The rate of metachronous extrapancreatic malignancies is not superior to the general population.
Diagnostic yield of EUS-guided through-the-needle biopsy in pancreatic cysts: a meta-analysis
2020, Gastrointestinal Endoscopy
There is currently limited evidence supporting the use of EUS-guided through-the-needle biopsy for sampling pancreatic cystic lesions. The aim of this meta-analysis was to provide a pooled estimate of the diagnostic performance of through-the-needle biopsy for sampling of pancreatic cysts.
A bibliographic search on the main databases was performed in September 2019. Pooled effects were calculated using a random-effects model by means of the DerSimonian and Laird test. The primary outcome was sample adequacy. Additional endpoints were diagnostic accuracy, optimal histologic core procurement, mean number of needle passes, pooled specificity, and sensitivity. Adverse event rates were also analyzed.
Eleven studies enrolling 490 patients were included. Eight articles compared through-the-needle biopsy with cytology/cystic fluid analysis. Most patients were female, and the body/tail was the most frequent location of cystic lesions. Sample adequacy with through-the-needle biopsy was 85.3% (78.2%-92.5%), and subanalysis performed according to cyst morphology, size, and location confirmed the result. Through-the-needle biopsy clearly outperformed FNA both in terms of sample adequacy (odds ratio, 4.83; 95% confidence interval, 1.63-14.31; P =.004) and diagnostic accuracy (odds ratio, 3.44; 95% confidence interval, 1.32-8.96; P =.01). The pooled diagnostic accuracy rate, sensitivity, and specificity of through-the-needle biopsy were 78.8%, 82.2%, and 96.8%, respectively. A mean of 3.121 (2.98-3.25) passes through the cyst was needed to obtain adequate histologic samples. The incidence rates of mild bleeding and pancreatitis were 4% and 2%, respectively.
Our meta-analysis speaks in favor of the use of through-the-needle biopsy as a safe and effective tool in EUS-guided tissue acquisition of pancreatic cysts.
Analytical Validation of Loss of Heterozygosity and Mutation Detection in Pancreatic Fine-Needle Aspirates by Capillary Electrophoresis and Sanger Sequencing
2024, Diagnostics
Are the screening guidelines for branch duct intraductal papillary mucinous neoplasms cost-effective in an Australian setting?
2023, ANZ Journal of Surgery

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: Conflicts of interest The authors disclose no conflicts.

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Disease and Therapy of Pancreatic DisorderReviewPancreatic Cystic Neoplasms: Management and Unanswered Questions

Section snippets

Mucinous Cystic Neoplasms

BD-IPMNs

Role for EUS in Diagnosis and Treatment

Surgery

Surgery

J Gastrointest Surg

J Gastrointest Surg

J Gastrointest Surg 2011

Am J Pathol

Surgery

J Am Coll Surg

Surgery

Surgery

Gastroenterology

Gastroenterology

Gastroenterology

Am J Surg

Pancreatology

Clin Gastroenterol Hepatol

Clin Gastroenterol Hepatol

Surgery

Clin Gastroenterol Hepatol

Clin Gastroenterol Hepatol

Pancreatology

Pancreatology

Pancreatology

Clin Gastroenterol Hepatol

Gastroenterology

Am J Surg

Am J Surg

J Am Col Radiol

Pancreatology

HPB (Oxford)

Clin Gastroenterol Hepatol

Gastrointest Endosc

Eur J Radiol

Gastrointest Surg

Eur J Radiol

Gastroenterology

Gastrointest Endosc

Gastrointest Endosc

Gastrointest Endosc

Pancreatology

Gastrointest Endosc

Gastroenterology

Gastrointest Endosc

Gastrointest Endosc

J Am Coll Surg

Disappointing interobserver agreement among radiologists for a classifying diagnosis of pancreatic cysts using magnetic resonance imaging

Pancreas

Prevalence of unsuspected pancreatic cysts on MDCT

Am J Roentgenol

Mucinous cystic neoplasm of the pancreas is not an aggessive entity: lessons from 163 resected patients

Ann Surg

Clinicopathological features and prognosis of mucinous cystic neoplasm with ovarian-type stroma: a multi-institutional study of the Japan Pancreas Society

Pancreas

Disease and Therapy of Pancreatic Disorder
Review
Pancreatic Cystic Neoplasms: Management and Unanswered Questions