Synopsis
Approach to Cystic Pancreatic Neoplasms using MRI Phenotype. Radiologists are only approximately 60% accurate in providing the correct diagnosis of cystic pancreatic neoplasms pre-operatively. This image rich talk will cover the typical and atypical MRI characteristics for the seven most common cystic pancreatic neoplasms (IPMN, mucinous cystic neoplasm, serous cystadenoma, pseudocyst, cystic neuroendocrine tumor, necrotic adenocarcinoma and solid pseudopapillary tumors), and emphasize distinct MRI features that can help narrow the differential diagnosis. Useful clinical information, such as patient history and demographics will be covered. Common pitfalls will also be discussed. Background
With increased cross-sectional imaging,
many cystic pancreatic lesions are found incidentally. For example, 13.5% of asymptomatic patients
undergoing abdominal MRI will have incidentally detected cystic pancreatic lesions,
usually small (7 mm), solitary (60%), and with an increasing incidence with age
(Lee et al). Many of these small lesions
are presumed branch duct IPMNs, although some may represent non-neoplastic cysts
(mucinous, lymphoepithelial, or epithelial).
Larger
and more complex lesions often are surgically resected, and radiologists are
only 60-63% accurate in providing the correct diagnosis pre-operatively
(Correa-Gallego et al, Del Chiaro et al).
Increasing our awareness of these lesions and their MRI phenotype can
decrease unnecessary EUS and possibly morbid pancreatic surgery.
This image-rich talk will focus on these
potentially surgical lesions, and how to use their MRI phenotypes to narrow the
differential diagnosis.
The seven cystic
pancreatic lesions to be covered can be divided into two broad categories -
primarily cystic lesions and solid lesions undergoing cystic change. Primarily cystic lesions include intraductal papillary mucinous neoplasm
(IPMN), mucinous cystic neoplasm (MCN), pseudocyst and serous cystadenoma (SCA). Solid lesions undergoing cystic change include cystic neuroendocrine tumor (NET), necrotic adenocarcinoma, and solid pseudopapillary tumor (SPT).
Patient demographics
The patient's clinical history and demographics are important factors in narrowing the differential diagnosis of cystic pancreatic neoplasms. Gender and age are most helpful for MCN (95% female, middle-aged), SPT (88% female, young) and SCA (75% female, older). A convincing history of pancreatitis can suggest a diagnosis of pseudocyst.
Cyst fluid analysis
Cyst fluid analysis obtained during EUS cyst fluid aspiration can narrow the differential. A cyst fluid CEA > 192 ng/mL yields a 73% sensitivity and 84% specificity for mucinous lesions such as MCN and IPMN (Brugge et al). Elevated amylase suggests communication with the pancreatic duct, such as IPMN and pseudocyst. Recent studies have shown that cyst fluid VEGF-A levels > 8500 pg/mL yield a 100% sensitivity and 97% specificity for SCA (Yip-Schneider et al).
MRI phenotype
1. Branch Duct IPMN
- Lesion morphology: tubular / ovoid >
round; communication with duct; 40% multifocal (“field defect”); main duct
involvement is under called in 20% of cases (Correa-Gallego et al)
- 2012 International Consensus Guidelines
(Tanaka et al): Worrisome
features include size > 3 cm, thickened/enhancing walls, main duct 5-9 mm,
non-enhancing mural nodule, abrupt change in duct caliber
- High
risk stigmata of malignancy: obstructive jaundice, enhancing solid component,
main duct > 10 mm
- Small FOV DWI can help w/ detection of
invasive component
- Additional: Older, slight male
predilection, EUS CEA > 192, elevated amylase
2. Mucinous Cystic Neoplasm (MCN)
- Lesion characteristics: thin wall,
clean claw sign with pancreas; +/- enhancing septations or locules; typically
not T1 hyperintense unless post-biopsy; +/- peripheral calcification on CT; can
be unilocular (look for nice claw sign to ddx from pseudocyst); predilection
for body/tail. Size > 4 cm and
enhancing soft tissue component suggests carcinomatous transformation
- Additional: Middle-aged, female (95%) as lined by ovarian stroma,
EUS CEA > 192, no elevation in amylase
3. Pseudocyst *most common cystic lesion of the pancreas
- Lesion characteristics: T1 hyperintense
internal debris / hemorrhage (sometimes with a fluid-fluid level); no internal
enhancement; thick T2 hypointense wall which becomes more discrete over time; dissection
of inflammatory tissue along fascial planes; evidence of acute/chronic
pancreatitis elsewhere in gland; obliteration of venous structures
- Most helpful clue is history and prior
imaging showing an evolution of pancreatitis
4. Serous Cystadenoma (SCA)
- Lesion characteristics: Lobulated /
bosselated border; typically microcystic honeycomb appearance with thin
enhancing septations, but also can be oligocystic (10%), mixed
micro/macrocystic, and solid variant; can obstruct duct; only 30% with
pathognomonic central scar (enhancing or T2 hypointense calcification). -
- DWI may be able to differentiate
between mucinous cysts and SCA with 100% sensitivity and 97% specificity (Schraibman et al)
- EUS: VEGF-A > 8500 is 97% specific; also cystic fluid
metabolites glucose and kynurenine are markedly elevated in SCA compared to
MCNs
- Can occur in VHL patients – look for
other VHL lesions such as RCC
- Benign – NO malignant potential (30
cases reported in literature, but most pathologists do not believe that SCA has
malignant potential) so accurate pre-operative diagnosis prevents surgery
5. Cystic components found in 17% of
resected NET
- Lesion characteristics: well
circumscribed; rim of well-vascularized tissue; centrally can be completely
cystic or have low level internal enhancement amongst degeneration
- Usually non-functional, lower grade /
better prognosis than solid nonfunctioning NET
- More correlated with Multiple Endocrine
Neoplasia (MEN) than patients with solid NET
6. Necrotic Adenocarcinoma
- Lesion characteristics: infiltrative
mass w/ cystic component from necrosis, side-branch obstruction, or adjacent pseudocyst; usually
causes ductal obstruction
- Main DDX: invasive adenocarcinoma in
the setting of an IPMN, necrotic collection
7. Solid Pseudopapillary Tumor (SPT)
- Lesion characteristics: well
circumscribed, large (avg size 9 cm), solid / papillary / hemorrhagic (T1
hyperintense) / pseudocystic mass in pancreatic tail (60%) of young (29 yo)
female (88%)
- Low grade malignant potential,
excellent prognosis
- Main DDX: MCN w/ invasive component, cystic
NET --> all three are surgical lesions so may not change management
Common Pitfalls
1. Calling
a pseudocyst because “history of pancreatitis”, final pathology reveals MCN
Teaching
point: History of pancreatitis can be a red herring, look at remaining gland
for any signs of prior pancreatitis. A thin
wall and clean claw sign with no additional signs of acute/chronic pancreatitis
suggests MCN. Non-enhancing T1
hyperintensity, a thickened rind and evolution over several exams suggests a
pseudocyst.
2. Calling
a lobulated oligocystic lesion w/ central confluent enhancement an IPMN with
enhancing nodule, when it is actually oligocystic or mixed micro/macrocystic
SCA
Teaching
point: If lobulated / bosselated border and possible central scar, recommend
EUS w/ VEGF-A – can prevent unnecessary surgery.
3. Calling
an oligocystic lesion an MCN (surgical lesion) when it is an IPMN (not
necessarily surgical lesion depending on size).
Teaching
point: If solitary lesion in middle aged female, favor MCN.
If additional small cysts, tubular, or connection to duct, favor
IPMN. If still overlap, consider EUS.
4. Assuming
men cannot get SPT (12% will be in men) or MCN (at least 5% will be men)
Teaching
point: If it looks like a possible SPT or MCN, put it in the differential
because different pathologic stains will be performed on EUS-FNA.
5. Calling
a small-moderate sized lesion an SPT because the patient is young and female,
without looking as carefully at the lesion, which could possibly be a cystic
NET.
Teaching
point: Smaller SPTs are being found more frequently / incidentally with
increased imaging (not necessarily large).
SPTs have areas of hemorrhage in an overall solid mass; cystic NET tends
to have a peripheral rind of enhancement and are associated with MEN syndrome.
6. Calling
a main duct IPMN w/ solid components when it is chronic pancreatitis or vice versa.
Teaching
point: Chronic pancreatitis can overlap with main duct IPMN – look carefully at
their history, any prior CTs with calcification, EUS findings suggesting
calcifications and fibrosis, main duct irregularity. Check diffusion for focal mass.
7. Calling
pancreatitis and walled-off necrosis in setting of necrotic adenocarcinoma
causing inflammation
Teaching
point: Pancreatic adenocarcinoma can cause pancreatitis and necrotic pancreatic
adenocarcinoma can mimic a complex pseudocyst / walled off necrosis. Look
carefully at any cases of unexplained pancreatitis, for an obstructing or
necrotic mass.
8. Calling
a branch-duct IPMN when it is pathologically confirmed to be a benign lesion
Teaching
point: 5% of
resected pancreatic cystic lesions will be benign cysts (lymphoepithelial
cysts, squamoid cysts, mucinous non-neoplastic cysts, non-classified cysts),
but currently there are no specific / defining features to differentiate.
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