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Key demographic and clinical features of patients with pancreatic cystic neoplasms[1-4]

Key demographic and clinical features of patients with pancreatic cystic neoplasms[1-4]
  Serous cystic tumor Mucinous neoplasm Main-duct intraductal papillary mucinous neoplasm Branch-duct intraductal papillary mucinous neoplasm Solid pseudopapillary neoplasm
Age of presentation Variable, usually 5th to 7th decade Variable, usually 5th to 7th decade Variable, usually 5th to 7th decade Variable, usually 5th to 7th decade Usually 2nd to 3rd decade
Gender distribution Females >males Almost exclusively females Females = males Females = males Females >males
Typical clinical presentation Incidental or abdominal pain or mass effect Incidental or abdominal pain or malignancy related Incidental or pancreatitis or pancreatic insufficiency or malignancy related Incidental or pancreatitis or malignancy related Incidental or abdominal pain or mass effect
Typical imaging characteristics

Microcystic/honeycomb appearance

Oligocystic appearance less common

Unilocular or septated cyst ± wall calcifications

Solid component, if present, may suggest malignancy

Dilated main pancreatic duct ± parenchymal atrophy

Solid component, if present, may suggest malignancy

Dilated pancreatic duct branch or branches

Solid component, if present, may suggest malignancy
Solid and cystic mass ± calcifications
Typical aspirate characteristic Thin, often bloody Viscous Viscous Viscous or thin Bloody
Typical cytology findings Cuboidal cells that stain positive for glycogen; yield <50%

Columnar cells with variable atypia

Stains positive for mucin; yield <50%

High yield from solid component for malignancy

Columnar cells with variable atypia

Stains positive for mucin; yield <50%

High yield from solid component for malignancy

Columnar cells with variable atypia

Stains positive for mucin; yield <50%

High yield from solid component for malignancy

Characteristic branching papillae with myxoid stroma

High yield from solid component
Typical carcinoembryonic antigen (CEA) level <5 to 20 ng/mL in majority of lesions >200 ng/mL in approximately 75% of lesions >200 ng/mL in approximately 75% of lesions >200 ng/mL in approximately 75% of lesions Insufficient data
Typical glucose level >50 mg/dL in majority <50 mg/dL in majority <50 mg/dL (limited data) <50 mg/dL in majority Insufficient data
Typical DNA analysis Allelic loss affecting chromosome 3p and VHL mutation specific

K-ras mutation specific (>90%), not sensitive (<50%)

TP53, PTEN, PIK3CA, high DNA amount or high-amplitude allelic loss seen in malignancy

K-ras and GNAS mutation specific (>90%), not sensitive (<50%)

TP53, PTEN, PIK3CA, high DNA amount or high-amplitude allelic loss seen in malignancy

K-ras and GNAS mutation specific (>90%), not sensitive (<50%)

TP53, PTEN, PIK3CA, high DNA amount or high-amplitude allelic loss seen in malignancy
CTNNB1 mutation specific
Relative malignant potential Negligible Moderate High Low to moderate Moderate to high
Treatment Resect if symptomatic Resection Resection and post-resection surveillance

Closely monitor or resect

Post-resection surveillance required
Resection
References:
  1. Khalid A, Brugge WR. ACG practice guidelines for the diagnosis and management of neoplastic pancreatic cysts. Am J Gastroenterol 2007; 102:2339.
  2. Wu J, Jiao Y, Dal Molin M, et al. Whole-exome sequencing of neoplastic cysts of the pancreas reveals recurrent mutations in components of ubiquitin-dependent pathways. Proc Natl Acad Sci U S A 2011; 108:21188.
  3. Singhi AD, McGrath K, Brand RE, et al. Preoperative next-generation sequencing of pancreatic cyst fluid is highly accurate in cyst classification and detection of advanced neoplasia. Gut 2018; 67:2131.
  4. McCarty TR, Garg R, Rustagi T. Pancreatic cyst fluid glucose in differentiating mucinous from nonmucinous pancreatic cysts: a systematic review and meta-analysis. Gastrointest Endosc 2021; 94:698.
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