Differentiating intraductal papillary mucinous neoplasms from other pancreatic cystic lesions

Table 1 Distinguishing features of pancreatic cystic lesions[1,14-19]

Typical characteristics	IPMN	MCN	SC	PSEUDO	SPN	LEC	cNET	cPDAC
Age Group	Elderly	Middle	Middle-Elderly	Any	Young	Elderly	Middle-Elderly	Elderly
Gender	70% male	95% female	> 50% female	> 50% male	80%-90% female	80% male	50% each	> 50% male
History	Asx; Pain; ± jaundice	Asx; Pain; nausea	Asx; VHL	Pancreatitis	Asx; Pain; nausea	Asx	Asx; Fxnl; MEN	Asx; Pain; ± jaundice
Location in pancreas	Head in 70%; Multi-focal	Body/Tail in 95%	Anywhere	Anywhere	Anywhere	Peripheral	Anywhere	Anywhere
Shape	Ovoid	Spheroid	Ovoid	Spheroid	Ovoid	Ovoid	Spheroid	Variable
Locularity	Any	Uni or Oligo	Oligo or Multi	Uni	Oligo or Multi	Oligo	Uni	Any
Duct Com-munication	Common	No	No	Common	No	No	No	Some
Calcification	No	No	Central sunburst	No	Some	No	Some	No
Cyst fluid appearance	Viscous, clear, muc	Viscous, clear, muc	Thin, clear, nonmuc	Opaque, bloody/necrotic debris	Opaque, bloody/necrotic debris	Nonmuc, crystalline debris	Nonmuc	Thin
High CEA/Mucina	+	+	-	-	-	-	-	±
High Ca 19-9	±	±	-	-	-	-	-	±
High amylase	+	-	-	+	-	-	-	±
Epithelium	Columnar, Papillary	Columnar	Cuboidal	No epithelium	Poorly cohesive cells with nuclear grooves	Squamoid	Uniform	Gland-forming
Stroma	Fibrotic	Ovarian	Fibrotic	Fibrotic	Sometimes hyalinized	Lymphoid	Sometimes hyalinized	Fibrotic

^aMay be positive in cases of luminal contamination of endoscopic needle aspirate. IPMN: Intraductal papillary mucinous neoplasm; MCN: Mucinous cystic neoplasm; SC: Serous cystadenoma; PSEUDO: Pancreatic pseudocyst; SPN: Solid-pseudopapillary neoplasm; LE: Lymphoepithelial cyst; cNET: Cystic neuroendocrine tumor; cPDAC: Pancreatic ductal adenocarcinoma with cystic degeneration; VHL: Von hippel-lindau disease; Muc: Mucinous; Nonmuc: Nonmucinous; Asx: Asymptomatic; Fxnl: Functional. These data are derived generalizations of the literature, with the understanding that there is significant overlap among cyst types and there are inherent sampling errors associated with various tests; diagnostic and treatment decisions should not rely solely on the information presented in this review. An electronic worksheet version of this table is available at http://pathology.jhu.edu/pancreas/professionals/ipmn.php

Table 2 Key questions to aid in making likely diagnoses[19]

	Key question	Likely diagnoses to consider
Demographics and history	Male?	MCN unlikely
	No history of pancreatitis?	PSEUDO unlikely
	Young female?	SPN
	History of MEN?	cNET
	History VHL?	SC
Imaging	Spheroid?	PSEUDO or MCN
	Central sunburst calcification?	SC
	Location in head?	MCN unlikely
Cyst fluid	No CEA/mucin?	IPMN or MCN unlikely
	High CEA, high amylase?	IPMN
	High CEA, low amylase	MCN
	Low CEA, high amylase?	PSUEDO
	High amylase?	IPMN or PSEUDO
Histology	Epithelial lining?	PSEUDO unlikely
	Ovarian stroma?	MCN

IPMN: Intraductal papillary mucinous neoplasm; MCN: Mucinous cystic neoplasm; SC: Serous cystadenoma; PSEUDO: Pancreatic pseudocyst; SPN: Solid-pseudopapillary neoplasm; VHL: Von hippel-lindau disease; MEN: Multiple endocrine neoplasia. These data are derived generalizations of the literature, with the understanding that there is significant overlap among cyst types and there are inherent sampling errors associated with various tests; diagnostic and treatment decisions should not rely solely on the information presented in this review. An electronic worksheet version of this table is available at http://pathology.jhu.edu/pancreas/professionals/ipmn.php