Diagnostic Ultrasound - Abdomen and Pelvis
Mucinous Cystic Pancreatic Tumor TERMINOLOGY Abbreviations • Mucinous cystic pancreatic tumor (MCN) Synonyms • Mucinous cystic neoplasm of pancreas • Mucinous cystadenoma/cystadenocarcinoma • Macrocystic cystadenoma/cystadenocarcinoma • Macrocystic adenoma Definitions • Septated cystic neoplasm composed of mucin-producing epithelium and distinctive ovarian-type stroma, ranging in grade from potentially malignant to invasive carcinoma IMAGING General Features • Best diagnostic clue ○ Thick-walled, multilocular cystic mass with internal septations and possibly mural nodularity • Location ○ Body and tail of pancreas (more common) • Size ○ Range from 2 cm to > 10 cm in diameter ○ Mean size 8.7 cm Ultrasonographic Findings • Grayscale ultrasound ○ Well-circumscribed, anechoic, or hypoechoic mass, commonly in pancreatic body or tail ○ Unilocular or multilocular with echogenic septations – Cyst contents may be anechoic, echogenic with debris, ± solid component ○ No communication with pancreatic ductal system ○ May contain calcification ○ Mural nodularity suggests malignancy – When malignant: Possible adenopathy ± thick-walled cystic liver lesions • Color Doppler ○ Hypovascular mass ○ May encase splenic vein or displace surrounding vessels • Findings on US are nonspecific and further evaluation with CT or MR is necessary CT Findings • CECT ○ Well circumscribed and smoothly marginated ○ Unilocular or multilocular low-attenuation cystic lesion – When multiloculated, typically contains fewer than 6 cystic components – Each > 2 cm in size ○ May show peripheral curvilinear or septal calcification ○ Enhancement of cyst wall, internal septations and any mural nodules ○ Features favoring malignancy – Solid mural nodules – Thick septations – Wall thickening MR Findings • T1WI ○ Variable signal intensity based on cyst content – May be hypointense, isointense, or hyperintense on FS T1, depending on proteinaceous content ○ Hypointense focal calcifications • T2WI ○ T2 hyperintense cysts with mixed signal of internal septa ○ T2 hypointense capsule and calcifications • T1WI C+ ○ Enhancement of fibrous cyst wall on more delayed postcontrast sequences ○ Features suggesting malignancy – Enhancing septations and solid components • MRCP ○ Pancreatic duct may be displaced or narrowed by mass ○ Confirms absence of communication with pancreatic duct Other Modality Findings • Endoscopic ultrasound (EUS) ○ High spatial resolution; can depict internal septations, mural nodules, wall thickness ○ Can guide aspiration of fluid and biopsy of solid components, increasing overall accuracy for diagnosis over CT and MR – Cyst contents □ High CEA (< 5 ng/mL virtually excludes mucinous lesion) □ Low amylase (though can be increased) □ When malignant high CA 19.9 level □ Positive mucin stain Imaging Recommendations • Best imaging tool ○ CT or MR – Provides accurate characterization of cyst morphology ○ EUS – Invasive technique – Often performed in conjunction with cyst fluid aspiration for definitive diagnosis • Protocol advice ○ CT or MR should be performed with contrast enhancement – Dual-phase pancreatic protocol (late arterial and portal venous phases) – Improves sensitivity for depicting morphological features, including internal septation and mural nodularity ○ MRCP – To characterize relationship between lesion and pancreatic duct DIFFERENTIAL DIAGNOSIS Pseudocyst • Unilocular anechoic or hypoechoic cyst with no septations or solid components • May show communication to pancreatic duct • Peripancreatic fat plane infiltration • Clinical history of pancreatitis Diagnoses: Pancreas 367
Mucinous Cystic Pancreatic Tumor Diagnoses: Pancreas Intraductal Papillary Mucinous Neoplasm • Branch duct type: Grape-like clusters of small cysts • Communicates with pancreatic duct • Typically in head of pancreas or uncinate process Macrocystic Variant of Serous Cystadenoma • Unilocular cystic lesion usually in pancreatic head • Typically shows thinner, nonenhancing imperceptible wall Solid Pseudopapillary Tumor (SPN) • Large solid and heterogeneously cystic mass commonly in tail of pancreas • Intratumoral hemorrhage is more typical of SPN; rarely seen with MCN • Typically in young women Cystic Pancreatic Neuroendocrine Tumor • Hypervascular mass with nonenhancing cystic/necrotic components • May be multiple • May see metastatic disease in liver PATHOLOGY Staging, Grading, & Classification • Tumors in this group include ○ Benign mucinous cystadenoma (72%) ○ Borderline mucinous cystic tumor (10.5%) ○ Mucinous cystic tumor with carcinoma in situ (5.5%) ○ Mucinous cystadenocarcinoma (12%) • Any tumor in this group has potential to transform into invasive carcinoma Gross Pathologic & Surgical Features • Large mass with thick fibrous capsule • Mucin-containing cystic cavity ○ May be filled with thick mucoid material/clear/green/blood-tinged fluid • No communication with pancreatic duct • Solid papillary projections may protrude into tumor Microscopic Features • Cysts lined by columnar mucin-producing epithelium supported by ovarian-type stroma ○ Ovarian-type stroma distinguishes MCN from intraductal papillary mucinous neoplasm (IPMN) with stroma ductal in origin • Epithelium ranges in grade from benign to carcinoma ○ Can have benign configurations adjacent to areas of invasive carcinoma in same lesion ○ Biopsy to determine benign vs. malignant disease is therefore unreliable CLINICAL ISSUES Presentation • Most common signs/symptoms ○ Often asymptomatic ○ May present with epigastric pain, palpable mass, or fullness Demographics • Age ○ Mean age: 50 years ○ Range: 20-82 years • Gender ○ M:F = 1:20 • Epidemiology ○ 10% of cystic pancreatic tumors ○ 1% of pancreatic neoplasms Natural History & Prognosis • Invariably transforms into cystadenocarcinoma • Resection in absence of invasive carcinoma usually curative • 0% risk of recurrence • 5-year survival rate for invasive MCN 75% Treatment • All tumors in this class are considered surgical lesions ○ Typically seen in younger patient population and nonoperative management would require years of highcost, high-resolution imaging ○ Additionally, imaging and biopsy are unreliable in excluding invasive elements ○ Even benign lesions have future potential for malignant conversion • Tumors < 4 cm without mural nodule ○ May perform laparoscopic procedure ○ Parenchyma-sparing resections (e.g., middle pancreatectomy) and distal pancreatectomy with spleen preservation should be considered • Observation may be considered in elderly frail patients if unfit for surgery DIAGNOSTIC CHECKLIST Consider • Differentiate from other cystic pancreatic lesions • EUS and cyst aspiration for further evaluation Image Interpretation Pearls • Large, round, solitary, encapsulated uni- or multiloculated cystic mass with enhancing wall and internal septations SELECTED REFERENCES 1. Khashab MA et al: Should we do EUS/FNA on patients with pancreatic cysts? The incremental diagnostic yield of EUS over CT/MRI for prediction of cystic neoplasms. Pancreas. 42(4):717-21, 2013 2. Sahani DV et al: Diagnosis and management of cystic pancreatic lesions. AJR Am J Roentgenol. 200(2):343-54, 2013 3. Dewhurst CE et al: Cystic tumors of the pancreas: imaging and management. Radiol Clin North Am. 50(3):467-86, 2012 4. Tanaka M et al: International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas. Pancreatology. 12(3):183- 97, 2012 5. Sakorafas GH et al: Primary pancreatic cystic neoplasms revisited: part II. Mucinous cystic neoplasms. Surg Oncol. 20(2):e93-101, 2011 6. Reddy RP et al: Pancreatic mucinous cystic neoplasm defined by ovarian stroma: demographics, clinical features, and prevalence of cancer. Clin Gastroenterol Hepatol. 2(11):1026-31, 2004 368
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Mucinous Cystic Pancreatic Tumor<br />
Diagnoses: Pancreas<br />
Intraductal Papillary Mucinous Neoplasm<br />
• Branch duct type: Grape-like clusters of small cysts<br />
• Communicates with pancreatic duct<br />
• Typically in head of pancreas or uncinate process<br />
Macrocystic Variant of Serous Cystadenoma<br />
• Unilocular cystic lesion usually in pancreatic head<br />
• Typically shows thinner, nonenhancing imperceptible wall<br />
Solid Pseudopapillary Tumor (SPN)<br />
• Large solid <strong>and</strong> heterogeneously cystic mass commonly in<br />
tail of pancreas<br />
• Intratumoral hemorrhage is more typical of SPN; rarely<br />
seen with MCN<br />
• Typically in young women<br />
Cystic Pancreatic Neuroendocrine Tumor<br />
• Hypervascular mass with nonenhancing cystic/necrotic<br />
components<br />
• May be multiple<br />
• May see metastatic disease in liver<br />
PATHOLOGY<br />
Staging, Grading, & Classification<br />
• Tumors in this group include<br />
○ Benign mucinous cystadenoma (72%)<br />
○ Borderline mucinous cystic tumor (10.5%)<br />
○ Mucinous cystic tumor with carcinoma in situ (5.5%)<br />
○ Mucinous cystadenocarcinoma (12%)<br />
• Any tumor in this group has potential to transform into<br />
invasive carcinoma<br />
Gross Pathologic & Surgical Features<br />
• Large mass with thick fibrous capsule<br />
• Mucin-containing cystic cavity<br />
○ May be filled with thick mucoid<br />
material/clear/green/blood-tinged fluid<br />
• No communication with pancreatic duct<br />
• Solid papillary projections may protrude into tumor<br />
Microscopic Features<br />
• Cysts lined by columnar mucin-producing epithelium<br />
supported by ovarian-type stroma<br />
○ Ovarian-type stroma distinguishes MCN from intraductal<br />
papillary mucinous neoplasm (IPMN) with stroma ductal<br />
in origin<br />
• Epithelium ranges in grade from benign to carcinoma<br />
○ Can have benign configurations adjacent to areas of<br />
invasive carcinoma in same lesion<br />
○ Biopsy to determine benign vs. malignant disease is<br />
therefore unreliable<br />
CLINICAL ISSUES<br />
Presentation<br />
• Most common signs/symptoms<br />
○ Often asymptomatic<br />
○ May present with epigastric pain, palpable mass, or<br />
fullness<br />
Demographics<br />
• Age<br />
○ Mean age: 50 years<br />
○ Range: 20-82 years<br />
• Gender<br />
○ M:F = 1:20<br />
• Epidemiology<br />
○ 10% of cystic pancreatic tumors<br />
○ 1% of pancreatic neoplasms<br />
Natural History & Prognosis<br />
• Invariably transforms into cystadenocarcinoma<br />
• Resection in absence of invasive carcinoma usually curative<br />
• 0% risk of recurrence<br />
• 5-year survival rate for invasive MCN 75%<br />
Treatment<br />
• All tumors in this class are considered surgical lesions<br />
○ Typically seen in younger patient population <strong>and</strong><br />
nonoperative management would require years of highcost,<br />
high-resolution imaging<br />
○ Additionally, imaging <strong>and</strong> biopsy are unreliable in<br />
excluding invasive elements<br />
○ Even benign lesions have future potential for malignant<br />
conversion<br />
• Tumors < 4 cm without mural nodule<br />
○ May perform laparoscopic procedure<br />
○ Parenchyma-sparing resections (e.g., middle<br />
pancreatectomy) <strong>and</strong> distal pancreatectomy with spleen<br />
preservation should be considered<br />
• Observation may be considered in elderly frail patients if<br />
unfit for surgery<br />
DIAGNOSTIC CHECKLIST<br />
Consider<br />
• Differentiate from other cystic pancreatic lesions<br />
• EUS <strong>and</strong> cyst aspiration for further evaluation<br />
Image Interpretation Pearls<br />
• Large, round, solitary, encapsulated uni- or multiloculated<br />
cystic mass with enhancing wall <strong>and</strong> internal septations<br />
SELECTED REFERENCES<br />
1. Khashab MA et al: Should we do EUS/FNA on patients with pancreatic cysts?<br />
The incremental diagnostic yield of EUS over CT/MRI for prediction of cystic<br />
neoplasms. Pancreas. 42(4):717-21, 2013<br />
2. Sahani DV et al: Diagnosis <strong>and</strong> management of cystic pancreatic lesions. AJR<br />
Am J Roentgenol. 200(2):343-54, 2013<br />
3. 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 />
4. Tanaka M et al: International consensus guidelines 2012 for the<br />
management of IPMN <strong>and</strong> MCN of the pancreas. Pancreatology. 12(3):183-<br />
97, 2012<br />
5. Sakorafas GH et al: Primary pancreatic cystic neoplasms revisited: part II.<br />
Mucinous cystic neoplasms. Surg Oncol. 20(2):e93-101, 2011<br />
6. Reddy RP et al: Pancreatic mucinous cystic neoplasm defined by ovarian<br />
stroma: demographics, clinical features, <strong>and</strong> prevalence of cancer. Clin<br />
Gastroenterol Hepatol. 2(11):1026-31, 2004<br />
368