Mucinous Cystic Neoplasm

 Lined by mucinous columnar epithelium with focal cuboidal, flattened, or papillary areas


image May have gastric or intestinal metaplasia

image Varying degrees of dysplasia may be present

image Densely cellular ovarian-like stroma positive for ER, PR, and inhibin


• MCN with associated invasive carcinoma
image Most arise from preexisting MCN

image Invasion of underlying stroma by malignant glands or single cells




Top Differential Diagnoses




• Cystic variant of biliary intraductal papillary neoplasm

• Solitary bile duct cysts

• Ciliated hepatic foregut cyst

• Endometrial cyst


Diagnostic Checklist




• Multilocular cystic neoplasm lined by mucinous epithelial cells with underlying ovarian-type stroma

image
Schematic Representation
Graphic shows a lobulated, complex cystic mass with a vascularized wall, areas of solid growth, and well-defined septa typical of mucinous cystic neoplasm (MCN) of the liver.



TERMINOLOGY


Abbreviations




• Mucinous cystic neoplasm (MCN)


Definitions




• Cystic biliary neoplasm arising within liver or in extrahepatic biliary tree, including gallbladder

• Formerly known as biliary cystadenoma and cystadenocarcinoma


ETIOLOGY/PATHOGENESIS


Unknown




• May originate from müllerian remnants misplaced during embryogenesis

• Proliferation of endodermally derived epithelium and primitive mesenchyme stimulated by female sex hormone


CLINICAL ISSUES


Epidemiology




• Incidence
image Rare; < 5% of cystic lesions of liver

• Age
image Average: 40-50 years

• Sex
image Almost exclusively occurs in women


Presentation




• Pain, mass, and occasionally jaundice
image Some patients are asymptomatic


Laboratory Tests




• CA19-9 and CEA in cyst fluid helps differentiate between simple cyst and MCN


Treatment




• Surgical approaches
image Complete resection


Prognosis




• Complete surgical resection should be curative
image Incompletely resected tumor may recur or undergo malignant transformation


IMAGING


Ultrasonographic Findings




• Large, well-defined, multiloculated, anechoic mass with highly echogenic septations

• Mural or septal calcifications or fluid levels


CT Findings




• Nonenhanced CT
image Large, well-defined, homogeneous, hypodense heterogeneous mass (cystic and hemorrhagic areas)

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Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Mucinous Cystic Neoplasm

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