Review
Diagnosis and Treatment of Cystic Pancreatic Tumors

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Cystic pancreatic tumors (CPTs) have more frequently been identified in the last decade because of increased use of cross-sectional abdominal imaging. Although serous CPTs follow an indolent course and do not necessarily require surgical resection or long-term follow-up, mucinous CPTs (mucinous cystic neoplasms and intraductal papillary mucinous neoplasms) have a greater risk for malignancy. Although most CPTs are initially detected with imaging modalities such as computed tomography or magnetic resonance imaging, these tests alone rarely permit an accurate clinical diagnosis. Endoscopic ultrasound and endoscopic ultrasound-guided, fine-needle aspiration allow real-time examination and biopsy analysis of CPTs, which increases diagnostic accuracy because cytopathology features and tumor markers in cyst fluid can be analyzed. Management of patients with mucinous CPTs by surgery or imaging surveillance is controversial, partially because of limited information about disease progression and the complexities of surgical resection. We review approaches to diagnosis and management of common CPTs.

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

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