Know the Facts

FAQ's

What are pancreatic cysts?

Pancreatic cysts are fluid-filled neoplasms (i.e., abnormal growths) in the pancreas. “Pancreatic cysts” is somewhat of a misnomer. So called “pancreatic cysts” may be true cysts, false cysts or enlargements of the pancreatic ductal (i.e., pipe) system mimicking a cyst. True cysts are fluid-filled structures in the pancreas lined with cells (e.g., serous cystic neoplasms, mucinous cystic neoplasms). False cysts (i.e., pseudocysts) are fluid-filled structures in the pancreas without a cell lining. Sometimes the pancreatic ductal system can enlarge and appear cystic (e.g., intraductal papillary mucinous neoplasms).

What types of pancreatic cysts are there?

Common pancreatic cysts include pseudocysts, serous cystic neoplasms, and mucinous cysts. The most common are mucinous cysts of which there are 2 types; namely, intraductal papillary mucinous neoplasms (IPMN) and mucinous cystic neoplasms (MCN).

Why are pancreatic cysts important?

Some pancreatic cysts may progress to pancreatic cancer. Mucinous pancreatic cysts are the most common pancreatic cysts and have significant potential to progress to pancreatic cancer. Since patients with mucinous cysts are at increased risk of pancreatic cancer, active screening, risk stratification and pancreatic cyst removal in select patients at highest risk promotes pancreatic cancer early detection and treatment.

How are pancreatic cysts detected?
Pancreatic cysts may be detected incidentally, i.e., when a patient undergoes testing for another indication. Many pancreatic cysts are being diagnosed in this way due to an increase in the use of high resolution cross-sectional imaging (i.e., CT and MRI scans) and recognition of their significance. Pancreatic cysts may also cause symptoms and signs and be discovered in this manner.
What signs and symptoms may develop in a patient with a pancreatic cyst?
  1. Pain
    • abdomen (up under ribs)
    • back (just below the shoulder blades)
  2. pancreatitis (i.e.,inflammation of pancreas which is commonly associated with abdominal/back pain, loss of appetite, nausea and vomiting)
  3. pancreatic digestive enzyme deficiency (exocrine insufficiency) manifested by:
    • bloating/indigestion
    • flatulence (foul smelling)
    • steatorrhea (i.e., frequent bowel motions which appear oily, greasy and often float on the top of toilet bowel)
    • weight loss (unintentional)
    • malnutrition, muscle wasting
  4. diabetes (endocrine insufficiency) especially in patients who are not obese and do not have a family history of diabetes:
    – this could be diabetes of new onset manifested by:
    • increased thirst
    • increased urination
    • increased appetite
    • – this could also be worsening of pre-existing diabetes manifested by:
    • increase in the strength or number of oral hypoglycemics (i.e., diabetes pills) needed to control diabetes
    • increase in required insulin needed to control diabetes
    • transition from oral hypoglycemics (i.e., diabetes pills) to insulin
  1. jaundice manifested by:
    • yellow eyes
    • clay-colored stools
    • tea colored urine
    • pruritis (itchy skin)
Do all pancreatic cysts need to be surgically removed?
No. Pancreatic cysts have variable malignant potential. Mucinous pancreatic cysts are the most common pancreatic cysts and have significant potential to progress to pancreatic cancer. Despite this, most mucinous pancreatic cysts without worrisome features may be safely but carefully watched.

Early Detection & Screening

Early Detection

Pancreatic cancer may occur without warning signs and symptoms. This underscores the importance of early detection. Although no screening test is applicable for the population in general, screening is indeed effective in high risk groups.

High risk groups include:

  • Patients with pancreatic cysts
  • Patients with hereditary pancreatic cancer

By accurately identifying patients at increased risk, screening and proper risk stratification may occur to promote early detection and prevention of pancreatic cancer.

In addition, following such patients and understanding their natural history, will promote identification of more robust early detection biomarkers for use in the population in general.

Screening

Screening is conducted in patients at increased risk for pancreatic cancer. It also allows the physician to stratify a patient’s risk of pancreatic cancer development and counsel the patient accordingly.

Stratification of risk depends upon 9 parameters: age, symptoms/signs/conditions, x-rays, biopsies, biochemical and molecular profile (CEA, DNA mutations), blood tests, family history of pancreatic cysts/cancer, tobacco history, and lifestyle history (obesity/fatty diet/sedentary). Patients without worrisome parameters are considered lower risk. In these patients, screening examinations are recommended at periodic intervals to re-stratify patient’s risk. Patients with worrisome parameters are considered higher risk. In these patients, shorter interval screening examinations are recommended to re-stratify risk.

When the risk of pancreatic cancer development outweighs the risk associated with pancreatic surgery, the patient is offered surgical remedy. A more detailed screening protocol (below) is provided for patients and physicians as a general guide.

PCC-EDC Surveillance protocol for patients “at risk”(lower risk) for pancreatic cancer

Lower risk patients (tend to have the following features):

Age (Pancreatic cancer incidence increases with age. At age 70, risk of pancreatic cancer is 1%, based on achieving age 70 alone. In patients with index lesions, age is also an indirect reflection of the duration in years of anticipated surveillance, (i.e., the younger age = greater total number of years of anticipated surveillance and correspondingly greater cumulative risk of malignancy).

No symptoms attributable to pancreas

No worrisome radiographic features

Absence of high grade atypia on biopsy

Molecular pancreatic fluid profile <1 mutations and no increased DNA quantity/quality

No abnormalities of serum Amylase, Lipase, Alkaline Phosphatase, CA 19-9, CEA, HGB A1C or AST (or clear alternative explanation of demonstrated abnormality)

Absence of tobacco use

Lifestyle characterized by absence of obesity/fatty diet/sedentary activity level

Family history of pancreatic cancer without an index lesion (e.g., IPMN) in the person being screened

  • <= 1 first degree relative (FDR) with pancreatic cancer

Family history of IPMN

  • <= 1 FDR with IPMN

Screening Recommendations:

Baseline Testing: History/physical, MRI-MRCP, EUS-FNA of “cyst” of MPC for cytopathologic, biochemical and molecular analyses (cysts >10 mm or MPD>4mm), serum Amylase, Lipase, Alkaline Phosphatase, CA 19-9, CEA, HGB A1C, AST and C-Peptide.

Annual Surveillance Testing

History/physical

*MRI-MRCP (or CT if MRI-MRCP contraindicated)

Serum Amylase, Lipase, Alkaline Phosphatase, CA 19-9, CEA, HGB A1C, AST and C-Peptide (Consider labs 2-4 times per year ​especially if history of concerning trends or abnormalities)

Every 3-5 years Surveillance Testing

Upper EUS (endoscopic ultrasound)**

Upper EUS-FNA of “cyst” or MPD for cytopathologic, biochemical and molecular analyses (cysts >10mm or MPD>4m)

Consider EGD (endoscopic retrograde examination) with secretin and collection of duodenal aspirate for cytopathologic, biochemical and molecular analyses (cysts <10mm or MPD <4mm)

PCC-EDC Surveillance protocol for patients “at risk” (higher) for pancreatic cancer

Higher risk patients: (if fit, many of these will be offered surgery; if unfit or surgery averse, they will be offered close interval surveillance and/or endoscopic cyst ablation)

High Risk:

Age >=70, or anticipated prolonged (i.e., multiple decades) of surveillance (Pancreatic cancer incidence increases with age. At age 70, risk of pancreatic cancer is 1%, based on achieving age 70 alone. In patients with index lesions, age is also an indirect reflection of number of years of anticipated surveillance (i.e., the younger age = greater number of years of anticipated surveillance and the greater risk of malignancy over time).
Symptoms attributable to pancreas (sp. steatorrhea, weight loss, diabetes, jaundice, pancreatitis; but less so abdominal/back pain)

Worrisome radiographic features

  • Main duct dilation >6mm
  • ***Main duct diameter increasea during surveillance 6 month visit (e.g., progression from 5mm to 7mm)
  • Mural nodule(s) or thick (>2mm) septae
  • Associated mass
  • Pancreatic duct stricture or cutoff with upstream main pancreatic duct dilation
  • Quantity of cysts so numerous that surveillance is unreliable
  • Size >3cm (unproven; insufficient as single risk factor; but still recommend high risk protocol for surveillance)
  • Greatest diameter increase by >5mm for lesions >1cm since last surveillance 6 month visit (unproven; but still recommend high risk protocol for surveillance)
  • Doubling of size for lesions <1cm since last surveillance visit (unproven; but still recommend high risk protocol for surveillance)
  • New IPMN in previously negative pancreatic remnant (prior resection)

Worrisome pathology, pancreatic fluid or serum markers

  • ***Cytopathology: Moderate or High grade atypia
  • Surgical pathology (prior resection): High grade dysplasia

Worrisome pancreatic fluid

  • DNA/cyst fluid molecular analysis (>1 of the following: increased DNA quantity/quality, KRas mutation, LOH mutation)

Worrisome serum markers

  • ***Rising/abnormal serum CA19-9 and/or CEA
  • Rising/abnormal serum Alkaline Phosphatase, Bilirubin or AST
  • Rising/abnormal serum HGB A1C (+/-) especially with lowering of C-Peptide and loss of weight
  • Abnormal Amylase, Lipase (elevated or suppressed above/below patient’s established baseline)

Family history of pancreatic cancer (or other relevant hereditary cancer syndrome) without an index pancreas lesion (e.g., IPMN) in the person being screened

  • 2 FDR with pancreatic cancer (20 fold)
  • 2 FDR with IPMN
  • 1 FDR with pancreatic cancer and one with IPMN
  • ***>2 FDR with pancreatic cancer (17-38.5% lifetime risk “LTR” by age 70)
  • ***FAMM (CDKN2A mutation: 17% LTR by age 70)
  • ***HP (PRSS1 mutation: 30-55% LTR by age 70))
  • ***PJ (STK11 mutation: 11-60% LTR by age 70)
  • ***HBOS (BRCA2: 2% LTR by age 70) Most common inherited mutation

Family history of pancreatic cancer with an index pancreas lesion (e.g., IPMN) in person being screened

  • >=1 first degree relative with pancreatic cancer
  • >=1 first degree relative with IPMN
  • >=1 first degree relative with pancreatic cancer and one with IPMN

Tobacco use
Lifestyle characterized by obesity/fatty diet/sedentary activity level

Baseline Testing: History/physical, MRI-MRCP, EUS-FNA, cytopathologic, biochemical and molecular analyses, serum Amylase, Lipase, Alkaline Phosphatase, CA 19-9, CEA, HGB A1C and AST.

Annual Surveillance Testing

EUS

EUS-FNA, cytopathologic, biochemical and molecular analysis semi-annually or annually (BD >=10mm or MPD>=4mm)

EGD/ER (endoscopic retrograde examination) with secretin and collection of duodenal aspirate for cytopathologic, biochemical and molecular analyses (cysts <=10mm or MPD <=4mm)

Semi-Annual Surveillance Testing

History/physical

MRI-MRCP (or CT if MRI-MRCP contraindicated)

Serum Amylase, Lipase, Alkaline Phosphatase, CA 19-9, CEA, HGB A1C and AST (Consider labs 3-4 times a year especially if history of concerning trends or abnormalities)

Every 1-3 years Surveillance Testing

Upper EUS

Upper EUS-FNA, cytopathologic, biochemical, and molecular analysis semi-annually or annually (BD >=10mm or MPD>=4mm)

Consider EGD with secretin and collection of duodenal aspirate for cytopathologic, biochemical, and molecular analyses (cysts <=10mm or MPD <=4mm)

* Patients with current (within last 3 months) benign molecular pancreatic fluid profile (no mutations and no increased DNA quantity/quality) who are negative for any of the other 8 risk criteria (age, symptoms/signs, x-rays, biopsies, blood tests, family history of pancreatic cysts/cancer, tobacco, and obesity/fatty diet) could forego annual cross sectional imaging for a 2 year interval. We strongly recommend the patients still return annually to PCC-EDC, however, for education, H&P and serum tumor marker labs. There is a high rate of attrition of patients who are not followed at least annually.

** Patients in the “lower risk” group undergoing Upper EUS surveillance need not undergo cross-sectional imaging (MRI-MRCP or CT) in the same visit unless this is desired as a roadmap b the EUS physician or there are anticipated “blind spots” that need cross sectional imaging review.

*** These patients in the “higher risk” group may require even higher intensity of surveillance, i.e., H&P, serum tumor markers, cross sectional imaging 3-4 times per year and EUS-FNA, cytopathologic, biochemical and molecular analysis semi-annually.

We recommend the applicability of such a protocol in any particular case be discussed with one’s physician. We have also included a reference to the International Consensus Guidelines for the treatment of patients with pancreatic cysts which were published in 2006 and then updated in 2012. Dr. Schmidt was a co-author of the 2012 International Consensus Guidelines. The International Consensus Guidelines remain a leading guide toward management with a data driven algorithm. Nevertheless, the guidelines are somewhat outdated in comparison with the PCC-EDC protocol. The 2015 AGA Institute guidelines for management of asymptomatic neoplastic pancreatic cysts are by the author’s own admission “weak recommendations” based on “very low quality evidence” and run counter to the International Consensus Guidelines so are not cited here as a guide at this time.

  • Masao Tanaka, MD, PhD, FACS
    Professor & Chairman
    Department of Surgery and Oncology
    Graduate School of Medical Sciences, Kyushu University
    Fukuoka 812-8582, Japan
  • Volkan Adsay, MD
    Professor, Director
    Department of Anatomic Pathology
    Emory University Hospital
    Atlanta, GA
  • Carlos Fernandez-del Castillo, MD, FACS
    Director
    Pancreas and Biliary Surgery Program
    Massachusetts General Hospital
    Boston, MA
  • Suresh Chari, MD
    Professor of Medicine, Head
    Pancreas Interest Group, Division of Gastroenterology and Hepatology
    Mayo Clinic
    Rochester, MN
  • Massimo Falconi, MD
    U.O. Chirurgia B
    Dipartimento di Scienze chirurgiche e Gastroenterologiche
    Policlinico “G.B. Rossi”
    Verona, Italy
  • Michio Shimizu, MD, PhD
    Director
    Department of Pathology
    Saitama Medical University, International Medical Center
    1397-1 Yamane, Hidaka city, Saitama 350-1298, Japan
  • Koji Yamaguchi, MD, PhD
    Department of Surgery I
    University of Occupational and Environmental Health
    Orio, Fukuoka, Japan
  • Kenji Yamao, MD, PhD
    Aichi Cancer Center Hospital
    Aichi, Japan
  • Wataru Kimura, MD, PhD
    First Department of Surgery
    2-2-2 Iida-nishi, Yamagata 990-9585, Japan
  • Jin-Young Jang, MD, PhD
    Associate Professor, Division of Hepatobiliary-Pancreatic Surgery
    Department of Surgery
    Seoul National University College of Medicine
    28 Yongon-dong, Chongno-gu, Seoul 110-744, KOREA
  • Philippe Levy, MD
    Pôle des Maladies de l’Appareil Digestif
    Service de Gastroentérologie-Pancréatologie
    Hopital Beaujon
    Clichy-Cedex, France
  • Martha Bishop Pitman, MD
    Associate Professor, Assistant Director of Cytology and Director, FNA Service
    Department of Pathology
    Massachusetts General Hospital, Harvard Medical School
    Boston, MA
  • C. Max Schmidt, MD, PhD, MBA, FACS
    Associate Professor of Surgery, Biochemistry and Molecular Biology
    Department of Surgery
    Indiana University School of Medicine
    Indianapolis, IN
  • Christopher L. Wolfgang, MD, PhD, FACS
    Assistant Professor of Surgery and Oncology, Cameron Division of Surgical Oncology and The Sol Goldman Pancreatic Cancer Research Center
    Department of Surgery
    Johns Hopkins University
    Baltimore, MD
Screening Locations

Pancreatic Cyst and Cancer Early Detection Clinic (PCC-EDC)
IU Health University Hospital
550 University Blvd; 1st Floor (SOPA)
Indianapolis, IN 46202
317-948-8358
Information: C. Max Schmidt, MD maxschmi@iupui.edu

 


 

Johns Hopkins Pancreas Cyst Clinic
601 N. Caroline St
Baltimore, MD 21287
410-933-PANC
Information: Christopher Wolfgang, MD cwolfga2@jhmi.edu
http://pathology.jhu.edu/pancreas/cyst/index.php

 


 

Massachusetts General Hospital
15 Parkman St, ACC/460
Boston, MA 02114
617-726-5644
Information: Carlos Fernández-del Castillo, MD cfernandez@partners.org

 


 

Memorial Sloan Kettering
1275 York Avenue
New York, NY 10065
Office: 212-639-2016 / Appt: 646-497-9070
Information: Peter Allen, MD
allenp@mskcc.org
http://www.mskcc.org/cancer-care/adult/pancreatic

 


 

Kyushu University
Kyushu University Hospital
Fukuoka 812-8582, Japan
+81.92.642.5453
Information: Masao Tanaka, MD
masaotan@surg1.med.kyushu-u.ac.jp

 


 

Elkins Pancreas Center
Baylor Clinic
6620 Main, Suite 1475
Houston, Texas 77030
Toll-free phone number (answered 24/7):
877-PANC-CTR / 877-726-2287
Information: William E. Fisher, MD, FACS
wfisher@bcm.edu / c: 713-297-1335
http://www.bcm.edu/pancreascenter

Diagnosis & Treatment

Diagnosis & Treatment

Diagnosis of pancreatic cysts is complicated. Importantly, the type of pancreatic cyst needs to be determined, and if a pre-cancerous (i.e., mucinous) type, the risk (low or high) of pancreatic cancer needs to be determined. Treatment of low risk pancreatic cysts may entail observation with periodic interval history, physical exam, lab tests, MRI or CT scans, endoscopic ultrasound, biopsy and molecular analyses. The treatment of high risk pancreatic cysts is most often surgical removal. New treatments are being investigated involving injection of pancreatic cysts with alchohol and chemotherapeutic agents (e.g., paclitaxel) in select patients.

Have you been diagnosed with a pancreatic cyst?

If so, you will need to be seen by a physician who specializes in pancreatic disease. Contact your primary care physician for a referral to a pancreatic disease specialist. Most commonly this is a gastroenterologist or a general surgeon who has received specialized training in the management of pancreatic diseases. This individual may provide screening themselves or may be able to direct you to a qualified screening clinic in your area. Such screening clinics, if available, will typically be at major medical centers housed within a university (academic) setting. You may also contact us (317-948-8358 or maxschmi@iupui.edu), and we may facilitate a referral.

Have you been diagnosed with pancreatic cancer?

If so, you will need to be seen by a physician who specializes in pancreatic disease. Contact your primary care physician for a referral to a pancreatic disease specialist. Most commonly this is a gastroenterologist, oncologist or a general surgeon who has received specialized training in the management of pancreatic diseases. This individual may provide treatment themselves or may be able to direct you to a qualified treatment clinic in your area. Most major medical centers housed within a university (academic) setting will have such resources. You may also contact us (317-948-8358 or maxschmi@iupui.edu), and we may facilitate a referral. Several excellent web-based resources for patients with pancreatic cancer are already established (see links below).

Several excellent web-based resources for patients with pancreatic cancer are already established.

The Lustgarten Foundation is the largest private funder of pancreatic cancer research. (PRNewsfoto/The Lustgarten Foundation)

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