Diagnostic Ultrasound - Abdomen and Pelvis
Serous Cystadenoma of Pancreas TERMINOLOGY Abbreviations • Serous cystadenoma (SCA) Synonyms • Pancreatic serous cystic neoplasm (SCN), glycogen-rich cystadenoma, microcystic adenoma of pancreas Definitions • Benign epithelial neoplasm arising from centroacinar cells of the exocrine pancreas, and composed of small cysts containing proteinaceous fluid separated by fibrovascular connective tissue septa. IMAGING General Features • Best diagnostic clue ○ Solitary, honeycomb or sponge-like mass with central radiating scar • Location ○ Commonly in the body and tail; 30% in pancreatic head • Size ○ Variable sizes; mean: 4.9 cm ○ Giant SCA ( >10 cm) are rare • Morphology ○ Lobulated, well-demarcated, cystic mass ○ 2 morphologic types based on WHO subclassification: – Serous microcystic adenomas: Honeycomb (20-40% of cases) or polycystic – Serous oligocystic adenoma/macrocystic variant (< 10% of cases): Usually unilocular or fewer larger cysts (> 2cm) Ultrasonographic Findings • Grayscale ultrasound ○ Well-demarcated, lobulated, heterogeneous mass with posterior acoustic enhancement ○ Generally → solid echogenic appearance due to interfaces between cysts – Slightly echogenic, solid-appearing mass (many interfaces between numerous small cysts) – Multicystic mass with septa and solid-appearing component □ Anechoic cystic areas usually in periphery □ Central echogenic area = central scar (present in 30% of cases); ± calcification – Macrocystic variant: Anechoic cyst ± a few septa ○ Pancreatic and common bile duct dilatation not typical • Color Doppler ○ Increased vascularity within septa CT Findings • Microcystic form: Classic honeycomb pattern ○ Thin wall with enhancing septa delineating small cysts – Cluster of > 6 cysts; each typically < 1 cm – Coalescing septa may form characteristic central stellate scar ± calcification – May mimic solid mass if cystic locules are small and enhancing septa predominate ○ Polycystic pattern: Multiple cysts ≤2 cm separated by enhancing fibrous septa ± calcification • Macrocystic serous cystadenoma: Usually unilocular ○ One/ few locules; thin nonenhancing imperceptible wall MR Findings • Can help identify cystic locules in tumors that appear solid on US and CT • T1WI: Hypointense tumor, central scar and calcification ○ Rarely may see intratumoral hemorrhage → varied signal intensity • T2WI: Hyperintense cystic components, hypointense septa, central scar, and calcification • T1WI C+: Delayed enhancement of septa and central scar • MRCP: No communication to the pancreatic duct Other Modality Findings • Endoscopic ultrasound (EUS) ○ Higher spatial resolution than transabdominal ultrasound → often diagnostic for microcystic form: – Well-delineated honeycomb appearance with central stellate scar (microcystic type) – Poorly developed cyst wall – Thin internal septa; hypervascular on Doppler ○ Can be used to guide fine needle aspiration (FNA) of cyst fluid for indeterminate cases eg macrocystic variant – Low viscosity, amylase, and CEA levels (< 5ng/mL) Imaging Recommendations • Best imaging tool ○ Contrast enhanced CT or MR ○ EUS: Improves characterization of lesion morphology and can guide cyst aspiration/biopsy – Invasive technique – Presumptive diagnosis based on typical microcystic features – EUS-FNA when EUS appearance is nonspecific • Protocol advice ○ In patients with thin body habitus, higher frequency transabdominal US transducer help to depict small cysts within the mass ○ Careful examination for subtle pancreatic calcification DIFFERENTIAL DIAGNOSIS Pancreatic Pseudocyst • Most common cystic pancreatic lesion • Collection of pancreatic fluid encapsulated by fibrous tissue • Shows well-defined capsule vs. imperceptible wall of SCA • Usually unilocular, no septa, solid component, or central calcification • Pancreatitis present or history of previous pancreatitis Mucinous Cystadenoma of Pancreas • Multiloculated cystic mass with echogenic internal septa • May be indistinguishable from macrocystic SCA by imaging • Most commonly located in tail of pancreas • Thicker wall with calcification that tends to be peripheral • Internal solid component suggests malignant tumor Intraductal Papillary Mucinous Neoplasm (IPMN) • Low grade malignancy arises from main pancreatic duct (MPD) or side branch pancreatic duct (SBD) Diagnoses: Pancreas 371
Serous Cystadenoma of Pancreas Diagnoses: Pancreas • SBD type lesion simulates serous microcystic adenoma due to presence of dilated small branch ducts ○ Grape-like cluster of small cysts ○ Communicates with the MPD ○ May be multiple • Can show pancreatic ductal dilatation Cystic Neuroendocrine Tumor • Solid mass with no pancreatic ductal dilatation • May show cystic components due to degeneration or central necrosis which can contain hemorrhagic debris • Intratumoral hemorrhage rare with SCA Ductal Pancreatic Carcinoma • More common tumor than serous cystadenoma • Rarely shows necrosis, or appears cystic due to fibrosis • Lack of tumoral calcification • Shows pancreatic &/or common bile ductal dilatation; vascular encasement ± regional/distant metastases Solid Pseudopapillary Neoplasm • Rare solid and cystic tumor with thick capsule and areas of necrosis and hemorrhage • Intratumoral hemorrhage rare with SCA • Typically in young women PATHOLOGY General Features • Associated abnormalities ○ von Hippel-Lindau disease: May have multiple SCAs • In general no malignant potential Gross Pathologic & Surgical Features • Well-circumscribed, round/ovoid, cystic mass with lobulated margin due to bulging cysts • Macroscopic cut section ○ Spongy appearance due to many small cysts (1-20 mm) ○ Fluid in cysts : Typically clear with no mucoid plugs – Rarely hemorrhagic in nature ○ Thin septa radiating from central scar ± dystrophic calcification Microscopic Features • Cysts lined by small cuboidal epithelial cells with clear cytoplasm and minimal mucin ○ Glycogen-rich; no cytologic atypia nor mitotic figures • Positive staining for epithelial membrane antigen & cytokeratin of low and high molecular weights • Fibrovascular septa • Adjacent pancreatic tissue: Normal or focally atrophic CLINICAL ISSUES Presentation • Most common signs/symptoms ○ Typically asymptomatic or vague epigastric pain ○ May present with nausea, vomiting, weight loss, palpable mass, jaundice ○ Other signs/symptoms of mass effect on adjacent structures (stomach & bowel) Demographics • Age ○ Middle & elderly age group (more common) ○ Mean age 61.5 years • Gender ○ M:F = 1:4 • Epidemiology ○ Cystic pancreatic neoplasms are rare ○ Accounts for 20% of all cystic pancreatic lesions; only 1% of all pancreatic neoplasms Natural History & Prognosis • Clinical practice assumes benign, slow-growing course ○ Most remain static over time with slow growth rate (~ 0.12 cm/year) and no complications • Rare tumors may behave aggressively or become symptomatic (usually large lesions and location in the head of the pancreas) ○ Tumors > 4cm grow faster (~ 2 cm/year) ○ Potential complications: CBD obstruction or obstructive chronic pancreatitis, bowel obstruction; invasion of surrounding structures or vessels ○ Very rarely metastases to lymph nodes or distant organs • Prognosis ○ Excellent: Classically thought to have no malignant potential ○ However, rare aggressive subtypes can be locally aggressive or malignant (0.8%) ○ Good prognosis after complete excision with negative surgical margins, in symptomatic or aggressive cases ○ May recur after incomplete resection Treatment • Nonoperative management if asymptomatic, small, and confidently diagnosed ○ Imaging surveillance at 6-12 month intervals until stability demonstrated over 2 year period • Symptomatic & large tumors → complete surgical excision ○ No imaging surveillance necessary if negative surgical margins DIAGNOSTIC CHECKLIST Consider • Differentiate from other cystic pancreatic lesions such as pseudocysts or cystic neoplasms that have malignant potential Image Interpretation Pearls • Well-demarcated, lobulated cystic lesion composed of innumerable small cysts (1-20 mm) separated by thin hypervascular septa SELECTED REFERENCES 1. But DY et al: To fine needle aspiration or not? An endosonographer's approach to pancreatic cystic lesions. Endosc Ultrasound. 3(2):82-90, 2014 2. Dewhurst CE et al: Cystic tumors of the pancreas: imaging and management. Radiol Clin North Am. 50(3):467-86, 2012 3. Kucera JN et al: Cystic lesions of the pancreas: radiologic-endosonographic correlation. Radiographics. 32(7):E283-301, 2012 4. Choi JY et al: Typical and atypical manifestations of serous cystadenoma of the pancreas: imaging findings with pathologic correlation. AJR Am J Roentgenol. 193(1):136-42, 2009 372
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Serous Cystadenoma of Pancreas<br />
Diagnoses: Pancreas<br />
• SBD type lesion simulates serous microcystic adenoma due<br />
to presence of dilated small branch ducts<br />
○ Grape-like cluster of small cysts<br />
○ Communicates with the MPD<br />
○ May be multiple<br />
• Can show pancreatic ductal dilatation<br />
Cystic Neuroendocrine Tumor<br />
• Solid mass with no pancreatic ductal dilatation<br />
• May show cystic components due to degeneration or<br />
central necrosis which can contain hemorrhagic debris<br />
• Intratumoral hemorrhage rare with SCA<br />
Ductal Pancreatic Carcinoma<br />
• More common tumor than serous cystadenoma<br />
• Rarely shows necrosis, or appears cystic due to fibrosis<br />
• Lack of tumoral calcification<br />
• Shows pancreatic &/or common bile ductal dilatation;<br />
vascular encasement ± regional/distant metastases<br />
Solid Pseudopapillary Neoplasm<br />
• Rare solid <strong>and</strong> cystic tumor with thick capsule <strong>and</strong> areas of<br />
necrosis <strong>and</strong> hemorrhage<br />
• Intratumoral hemorrhage rare with SCA<br />
• Typically in young women<br />
PATHOLOGY<br />
General Features<br />
• Associated abnormalities<br />
○ von Hippel-Lindau disease: May have multiple SCAs<br />
• In general no malignant potential<br />
Gross Pathologic & Surgical Features<br />
• Well-circumscribed, round/ovoid, cystic mass with lobulated<br />
margin due to bulging cysts<br />
• Macroscopic cut section<br />
○ Spongy appearance due to many small cysts (1-20 mm)<br />
○ Fluid in cysts : Typically clear with no mucoid plugs<br />
– Rarely hemorrhagic in nature<br />
○ Thin septa radiating from central scar ± dystrophic<br />
calcification<br />
Microscopic Features<br />
• Cysts lined by small cuboidal epithelial cells with clear<br />
cytoplasm <strong>and</strong> minimal mucin<br />
○ Glycogen-rich; no cytologic atypia nor mitotic figures<br />
• Positive staining for epithelial membrane antigen &<br />
cytokeratin of low <strong>and</strong> high molecular weights<br />
• Fibrovascular septa<br />
• Adjacent pancreatic tissue: Normal or focally atrophic<br />
CLINICAL ISSUES<br />
Presentation<br />
• Most common signs/symptoms<br />
○ Typically asymptomatic or vague epigastric pain<br />
○ May present with nausea, vomiting, weight loss, palpable<br />
mass, jaundice<br />
○ Other signs/symptoms of mass effect on adjacent<br />
structures (stomach & bowel)<br />
Demographics<br />
• Age<br />
○ Middle & elderly age group (more common)<br />
○ Mean age 61.5 years<br />
• Gender<br />
○ M:F = 1:4<br />
• Epidemiology<br />
○ Cystic pancreatic neoplasms are rare<br />
○ Accounts for 20% of all cystic pancreatic lesions; only 1%<br />
of all pancreatic neoplasms<br />
Natural History & Prognosis<br />
• Clinical practice assumes benign, slow-growing course<br />
○ Most remain static over time with slow growth rate (~<br />
0.12 cm/year) <strong>and</strong> no complications<br />
• Rare tumors may behave aggressively or become<br />
symptomatic (usually large lesions <strong>and</strong> location in the head<br />
of the pancreas)<br />
○ Tumors > 4cm grow faster (~ 2 cm/year)<br />
○ Potential complications: CBD obstruction or obstructive<br />
chronic pancreatitis, bowel obstruction; invasion of<br />
surrounding structures or vessels<br />
○ Very rarely metastases to lymph nodes or distant organs<br />
• Prognosis<br />
○ Excellent: Classically thought to have no malignant<br />
potential<br />
○ However, rare aggressive subtypes can be locally<br />
aggressive or malignant (0.8%)<br />
○ Good prognosis after complete excision with negative<br />
surgical margins, in symptomatic or aggressive cases<br />
○ May recur after incomplete resection<br />
Treatment<br />
• Nonoperative management if asymptomatic, small, <strong>and</strong><br />
confidently diagnosed<br />
○ Imaging surveillance at 6-12 month intervals until<br />
stability demonstrated over 2 year period<br />
• Symptomatic & large tumors → complete surgical excision<br />
○ No imaging surveillance necessary if negative surgical<br />
margins<br />
DIAGNOSTIC CHECKLIST<br />
Consider<br />
• Differentiate from other cystic pancreatic lesions such as<br />
pseudocysts or cystic neoplasms that have malignant<br />
potential<br />
Image Interpretation Pearls<br />
• Well-demarcated, lobulated cystic lesion composed of<br />
innumerable small cysts (1-20 mm) separated by thin<br />
hypervascular septa<br />
SELECTED REFERENCES<br />
1. But DY et al: To fine needle aspiration or not? An endosonographer's<br />
approach to pancreatic cystic lesions. Endosc <strong>Ultrasound</strong>. 3(2):82-90, 2014<br />
2. Dewhurst CE et al: Cystic tumors of the pancreas: imaging <strong>and</strong> management.<br />
Radiol Clin North Am. 50(3):467-86, 2012<br />
3. Kucera JN et al: Cystic lesions of the pancreas: radiologic-endosonographic<br />
correlation. Radiographics. 32(7):E283-301, 2012<br />
4. Choi JY et al: Typical <strong>and</strong> atypical manifestations of serous cystadenoma of<br />
the pancreas: imaging findings with pathologic correlation. AJR Am J<br />
Roentgenol. 193(1):136-42, 2009<br />
372