Mucinous Cystic Pancreatic Tumor

 “Macrocystic” pattern: Few (< 6) macrocystic locules, which are relatively large (> 2 cm)


image Usually of simple fluid attenuation, but may be mildly hyperdense due to hemorrhage or protein

image Frequent peripheral curvilinear calcifications or calcifications in septations (16% of cases)

image Presence of thick wall, mural nodularity, or thick septations suggests invasive malignancy

image No apparent communication with main pancreatic duct


• MR: Usually simple fluid signal (high T2; low T1), but may be slightly less T2 hyperintense due to mucin content
image Internal septations (which are T2 hypointense) easier to perceive on MRI compared to CT

image MRCP: No communication with main pancreatic duct




PATHOLOGY




• Tumor shares clinical and pathologic characteristics of biliary tumors, ovarian tumors, and IPMN

• Presence of ovarian stroma lining cyst is key feature for diagnosis of mucinous cystic neoplasm (MCN)


CLINICAL ISSUES




• Strong preponderance in middle-aged women (99%)

• Considered premalignant or frankly malignant: Rate of malignancy in different series ranges between 10-40%

• Risk factors for invasive malignancy: Older age, lesion size, mural nodularity, thick wall, patient symptoms (pain, pancreatitis), and ↑ CEA and CA 19-9

• MCNs typically undergo complete surgical resection with adjuvant chemotherapy for malignant lesions

• Malignant MCNs receive adjuvant chemotherapy after surgical resection

image
(Left) Graphic of a mucinous cystic tumor shows a multiseptate, mucin-filled, cystic mass in the pancreatic tail that displaces the pancreatic duct.


image
(Right) Axial CECT in a 35-year-old woman demonstrates a large complex cystic mass image arising from the pancreatic tail with multiple internal cystic locules and septations, some of which are thick image. This mass was found to be a mucinous cystic neoplasm (MCN) with invasive adenocarcinoma at surgery.

image
(Left) Axial CECT in a 46-year-old woman demonstrates a large, simple-appearing cyst image arising from the upstream pancreatic body.


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(Right) Axial T2 FS MR in the same patient confirms the lack of complexity within the T2-hyperintense cyst image. MR can sometimes show complexity and suspicious features that might be difficult to identify on CT. This was found to be a MCN with low-grade dysplasia at surgery.


TERMINOLOGY


Synonyms




• Mucinous cystic neoplasm (MCN), mucinous macrocystic neoplasm or adenoma, mucinous cystadenoma or cystadenocarcinoma


Definitions




• Thick-walled, unilocular or multilocular pancreatic tumor composed of large, mucin-containing cysts
image Some sources suggest presence of ovarian stroma within lesion is necessary for diagnosis

• MCN and intraductal papillary mucinous neoplasm (IPMN) are together classified as pancreatic mucinous tumors


IMAGING


General Features




• Best diagnostic clue
image Large, unilocular or multilocular encapsulated cystic mass with septations and thick wall in pancreatic tail

• Location
image Tail of pancreas (more common)

image Presents as single lesion (not multifocal like IPMN)

• Size
image 2-12 cm in diameter
– MCNs usually larger than serous cystadenoma or IPMN

image Size is predictor of biologic behavior
– Lesions < 40 mm found to have low risk of malignancy in recent series

• Morphology
image Typically round/ovoid, but may be ill defined/irregular


Radiographic Findings




• ERCP
image Displacement and narrowing of main pancreatic duct adjacent to tumor

image Can differentiate IPMN (which communicate with main pancreatic duct) from MCN (which do not communicate)


CT Findings




• Unilocular or multilocular encapsulated cyst in pancreatic body/tail with frequent internal septations
image “Macrocystic” pattern: Few (< 6) macrocystic locules that are relatively large (> 2 cm)

• Usually of simple fluid attenuation, but may be mildly hyperdense due to hemorrhage or protein

• Frequent peripheral curvilinear calcifications or calcifications in septations (16% of cases)

• Presence of thick, irregular wall, internal mural nodularity, or thick septations suggest invasive malignancy
image Upstream pancreatic ductal dilatation or atrophy suggest invasive malignancy

• No apparent communication with main pancreatic duct
image In rare instances, some series have suggested possible communication with pancreatic duct in very small minority of lesions


MR Findings




• Typically show simple fluid signal (high T2; low T1), but may be slightly less T2 hyperintense due to mucin content
image May show areas of T1 hyperintensity due to internal hemorrhage, proteinaceous content, or mucin

• Internal septations (typically T2 hypointense) easier to perceive on MR compared to CT

• Calcifications often not visible on MR: If visible, are low signal on all pulse sequences

• Thick, enhancing septations, wall thickening, and mural nodularity on T1WI C+ suggest invasive malignancy

• MRCP: No communication with pancreatic duct

• DWI: No role in differentiating mucinous from nonmucinous lesions or benign from malignant


Ultrasonographic Findings




• Grayscale ultrasound
image Multiloculated cystic mass with echogenic internal septa
– Often associated with thick wall ± mural nodularity

image Can also appear as unilocular anechoic mass


Angiographic Findings




• Conventional
image Predominantly avascular mass

image Cyst wall and solid component
– Show small areas of vascular blush and neovascularity

image Displacement of surrounding arteries and veins by cysts


Imaging Recommendations




• CECT or MR


DIFFERENTIAL DIAGNOSIS


Pancreatic Pseudocyst




• Usually known history of pancreatitis or alcoholism ± imaging stigmata of chronic pancreatitis (pancreatic calcifications, ductal beading, etc.)

• Loculated cyst with adjacent peripancreatic fat stranding/inflammation

• Evolves over time from acute peripancreatic fluid collection into loculated pseudocyst

• Communication with pancreatic duct is frequent (70% of cases) and may be visible on ERCP or MRCP

• Lab data: Increased amylase in cyst and serum

• When occurring in pancreatic tail, may simulate unilocular mucinous cystic neoplasm


Pancreatic Serous Cystadenoma




• Well-circumscribed, lobulated cystic mass most often occurring in pancreatic head

• Classic appearance (“microcystic” or “sponge” lesion): Many small cysts separated by thin septa and with central scar demonstrating calcification
image Calcification more common in serous than mucinous pancreatic neoplasms (38% vs. 16%)

• Macrocystic, oligocystic, and unilocular variants of serous cystadenoma difficult to distinguish from MCN
image Thick wall and mural nodularity unusual with serous cystadenoma


Pancreatic IPMN




• Mucin-producing neoplasms which are classified into 3 types with different risks of malignancy
image Side-branch IPMN: Arise in pancreatic duct side branch and carry risk of invasive malignancy of 17%

image Main pancreatic duct (MPD): Arise in main pancreatic duct and carry high risk of malignancy (58%)

image Combined type IPMN: Features of both side branch and main duct IPMN with prognosis similar to main duct IPMN

• Side-branch or combined type IPMN: Cyst (± nodularity, septations, calcifications) that communicates with pancreatic duct


Cystic Pancreatic Neuroendocrine Tumor




• Cystic neuroendocrine tumors more likely to be non-insulin producing and nonsyndromic

• Cystic lesion without pancreatic ductal dilatation or atrophy
image Differentiate from IPMN/MCN by presence of peripheral hyperenhancement on arterial phase CECT or MR

Nov 16, 2016 | Posted by in GASTROINTESTINAL IMAGING | Comments Off on Mucinous Cystic Pancreatic Tumor

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