ReviewDiagnosis and Treatment of Cystic Pancreatic Tumors
Section snippets
Epidemiology
Although the exact prevalence of CPTs is unknown, it was previously estimated at 1% of the general population based on previous large-scale observational imaging studies1 and up to 24% based on autopsy studies.2 However, recent magnetic resonance imaging (MRI)3, 4 and computed tomography (CT) studies5 indicate a prevalence ranging between 2.4% and 14%. These studies also suggest that pancreatic cysts occur equally in males and females and that prevalence increases with age. It is unclear if
Pathological Classification and Malignancy Risk
Pathologically, pancreatic cystic lesions can be classified by the presence or absence of epithelium lining the cyst. Pseudocysts, which lack an epithelial lining, are typically associated with acute or chronic pancreatitis.6 Neoplastic CPTs which constitute 15% of all pancreas cysts are lined by epithelium which may harbor a potential risk of progression to malignancy.6, 7 These CPTs include serous cystadenomas (SCA), intraductal papillary mucinous neoplasms (IPMN), mucinous cystic neoplasms
Clinical Presentation
The clinical presentation of CPTs is highly variable and often depends on the size, location, and pathology of the underlying lesion (Table 1). SCAs are most commonly seen in females during the seventh decade of life. They are typically asymptomatic and may be found incidentally on imaging studies performed for other reasons. Rarely, initial presentation occurs with compression of adjacent structures such as the gastrointestinal tract. Although most reports indicate that the majority arise in
Radiology
With the thin slices and high contrast resolution of current CT and MRI scanners, CPTs are incidentally discovered in an increasing population of patients. A study using 16-slice CT found that 3% of outpatients had an unsuspected pancreatic cystic lesion.5 While imaging tests are good for cyst detection and follow-up, they are less accurate for characterization. Nevertheless, several classic CPT imaging patterns may increase the likelihood of providing a correct diagnosis. For example, a
Cytology
Because of the limitations of imaging alone, the additional utility of FNA for cytology and fluid analysis of CPTs has been evaluated. The specificity of EUS-FNA cytology for the diagnosis of CPTs exceeds 90% in most published studies;75, 76, 77 however sensitivity is generally less than 50%.75, 76, 77, 78 Possible reasons for this wide variation in the reported sensitivity of EUS-FNA cytology for the diagnosis of CPTs may include the variable use of on-site cytology interpretation and
Genetic Markers
Recent advances in the diagnosis of CPTs include identification of specific genetic changes associated with various tumors and premalignant potential. The increasing knowledge about common genetic alterations leading to pancreatic adenocarcinoma, like p53 and K-ras mutations, increased interest in the evaluation of similar changes in CPTs. In malignant transformation of CPTs,88 K-ras mutations appear to occur early in the malignant transformation process.89 In IPMN, this is reported to be a
Management of CPTs
Management of CPTs continues to evolve as knowledge of their natural history increases. Nevertheless, significant management variability exists among practitioners. Practically, the decision to follow rather than recommend surgery for a CPT is a clinical judgement and is based on consideration of the patient's age, comorbidities, life expectancy, and estimation of the cancer risk in the lesion. The following section of this review will discuss available management options for these patients. A
Conclusions
CPTs are increasingly recognized by clinicians in symptomatic and asymptomatic populations. A multidisciplinary approach involving gastroenterologists, radiologists, and surgeons optimizes management of these patients. Use of cross-sectional imaging studies and EUS-FNA when available provides cyst fluid for cytology and tumor markers that may help to further characterize mucinous and malignant cysts. While surgical resection is recommended for symptomatic and high risk mucinous CPTs, periodic
References (138)
- et al.
High prevalence of pancreatic cysts detected by screening magnetic resonance imaging examinations
Clin Gastroenterol Hepatol
(2010) - et al.
Endoscopic ultrasound characteristics of mucinous cystic neoplasms of the pancreas
Am J Gastroenterol
(2000) - et al.
EUS in the evaluation of pancreatic cystic lesions
Gastrointest Endosc
(2003) - et al.
Pancreatic mucinous cystic neoplasm defined by ovarian stroma: demographics, clinical features, and prevalence of cancer
Clin Gastroenterol Hepatol
(2004) - et al.
Surgical management of intraductal papillary mucinous tumor of the pancreas
Surgery
(2002) - et al.
International consensus guidelines for management of intraductal papillary mucinous neoplasms and mucinous cystic neoplasms of the pancreas
Pancreatology
(2006) - et al.
Solid pseudopapillary tumors of the pancreasClinical features, surgical outcomes, and long-term survival in 45 consecutive patients from a single center
J Gastrointest Surg
(2011) - et al.
Pancreatic cystic neuroendocrine tumors: preoperative diagnosis with endoscopic ultrasound and fine-needle immunocytology
J Gastrointest Surg
(2008) - et al.
EUS-guided FNA of pancreatic metastases: a multicenter experience
Gastrointest Endosc
(2005) - et al.
Lymphoepithelial cysts of the pancreas: case report and review of the literature
J Gastrointest Surg
(2004)
EUS-guided FNA of a peripancreatic lymphocele
Gastrointest Endosc
Cystic neoplasms of the pancreas: benign to malignant epithelial neoplasms
Surg Clin North Am
Incidental pancreatic cysts: do we really know what we are watching?
Pancreatology
Diagnostic evaluation of cystic pancreatic lesions
HPB (Oxford)
Benefits of routine use of coronal and sagittal reformations in multi-slice CT examination of the abdomen and pelvis
Clin Radiol
CT vs MRCP: optimal classification of IPMN type and extent
J Gastrointest Surg
Risk of malignancy in resected cystic tumors of the pancreas < or =3 cm in size: is it safe to observe asymptomatic patients?A multi-institutional report
J Gastrointest Surg
Intraductal papillary mucinous neoplasms of the pancreas: correlation of helical CT features with pathologic findings
Eur J Radiol
Diagnosis and patient management of intraductal papillary-mucinous tumor of the pancreas by using peroral pancreatoscopy and intraductal ultrasonography
Gastroenterology
Solitary cystic tumor of the pancreas: EUS-pathologic correlation
Gastrointest Endosc
Can EUS alone differentiate between malignant and benign cystic lesions of the pancreas?
Am J Gastroenterol
Interobserver agreement among endosonographers for the diagnosis of neoplastic versus non-neoplastic pancreatic cystic lesions
Gastrointest Endosc
Performance of endosonography-guided fine needle aspiration and biopsy in the diagnosis of pancreatic cystic lesions
Am J Gastroenterol
Diagnosis of pancreatic cystic neoplasms: a report of the cooperative pancreatic cyst study
Gastroenterology
Detection and tumor staging of malignancy in cystic, intraductal, and solid tumors of the pancreas by EUS
Gastrointest Endosc
Utility of EUS in the evaluation of cystic pancreatic lesions
Gastrointest Endosc
Cyst fluid analysis in the differential diagnosis of pancreatic cystic lesions: a pooled analysis
Gastrointest Endosc
Intraductal papillary mucinous tumors of the pancreas: the preoperative value of cytologic and histopathologic diagnosis
Gastrointest Endosc
Safety and efficacy of cytology brushings versus standard FNA in evaluating cystic lesions of the pancreas: a pilot study
Gastrointest Endosc
Preliminary experience with a new cytology brush in EUS-guided FNA
Gastrointest Endosc
Preoperative cyst fluid analysis is useful for the differential diagnosis of cystic lesions of the pancreas
Gastroenterology
Molecular analysis to detect pancreatic ductal adenocarcinoma in high-risk groups
Gastroenterology
Does “clonal progression” relate to the development of intraductal papillary mucinous tumors of the pancreas?
J Gastrointest Surg
Molecular characterization of pancreatic serous microcystic adenomas: evidence for a tumor suppressor gene on chromosome 10q
Am J Pathol
Comparison of epigenetic and genetic alterations in mucinous cystic neoplasm and serous microcystic adenoma of pancreas
Mod Pathol
Pancreatic cyst fluid DNA analysis in evaluating pancreatic cysts: a report of the PANDA study
Gastrointest Endosc
Comparison of carcinoembryonic antigen and molecular analysis in pancreatic cyst fluid
Gastrointest Endosc
Cystic pancreatic neoplasms: observe or operate
Ann Surg
Analysis of small cystic lesions of the pancreas
Int J Pancreatol
Prevalence of incidental pancreatic cysts in the adult population on MR imaging
Am J Gastroenterol
Prevalence of unsuspected pancreatic cysts on MDCT
AJR Am J Roentgenol
Cystic tumors mistaken for pancreatic pseudocysts
Ann Surg
Cystic neoplasms of the pancreas
N Engl J Med
WHO-classification 2000: exocrine pancreatic tumors
Verh Dtsch Ges Pathol
Cystic tumors of the pancreasNew clinical, radiologic, and pathologic observations in 67 patients
Ann Surg
Mucinous cystic neoplasm of the pancreas is not an aggressive entity: lessons from 163 resected patients
Ann Surg
Intraductal papillary mucinous neoplasms of the pancreas: an analysis of clinicopathologic features and outcome
Ann Surg
Pathologically and biologically distinct types of epithelium in intraductal papillary mucinous neoplasms: delineation of an “intestinal” pathway of carcinogenesis in the pancreas
Am J Surg Pathol
Prognostic relevance of morphological types of intraductal papillary mucinous neoplasms of the pancreas
Gut
An illustrated consensus on the classification of pancreatic intraepithelial neoplasia and intraductal papillary mucinous neoplasms
Am J Surg Pathol
Cited by (60)
Biomarker Risk Score Algorithm and Preoperative Stratification of Patients with Pancreatic Cystic Lesions
2021, Journal of the American College of SurgeonsCytoreductive surgery and hyperthermic intraperitoneal chemotherapy in a patient with peritoneal carcinomatosis from a pancreatic cystadenocarcinoma: A case report
2019, International Journal of Surgery Case ReportsCitation Excerpt :Fig. 4 is an overview of the timeline of events that occurred. The prevalence of pancreatic cystic tumors in the general population ranges from 2.4 to 14%; of these, mucinous cystadenocarcinomas (MCAC) of the pancreas make up approximately 15% of all pancreatic cystic neoplastic lesions, making them relatively rare tumors [9,10]. Traditionally, pancreatic cancer with peritoneal metastases has been regarded as a terminal illness and is typically managed palliatively [11].
Incidental Intraductal Papillary Mucinous Neoplasm, Cystic or Premalignant Lesions of the Pancreas: The Case for Aggressive Management
2018, Surgical Clinics of North AmericaImaging in Gastroenterology
2018, Imaging in GastroenterologyEndoscopic Ultrasound Imaging for Diagnosing and Treating Pancreatic Cysts
2017, Gastrointestinal Endoscopy Clinics of North AmericaCitation Excerpt :MD-IPMN and mixed-type IPMN are slightly more prevalent in men,8,9 with a peak age of incidence in the 6th to 7th decades (Table 1).9,10 The majority of patients are asymptomatic and most BD-IPMNs are diagnosed incidentally on imaging studies.9,11 However, IPMNs can present with symptoms such as abdominal pain, jaundice, weight loss, diabetes, steatorrhea, and pancreatitis.11,12
Radiological description of cystic pancreatic tumors
2016, Radiologia
Conflicts of interest The authors disclose no conflicts.