Diagnostic Ultrasound - Abdomen and Pelvis
Solid Pseudopapillary Neoplasm TERMINOLOGY Abbreviations • Solid pseudopapillary neoplasm (SPN) Synonyms • Solid and papillary epithelial neoplasm (SPEN); papillary cystic epithelial neoplasm; papillary cystic tumor; solid and cystic tumor of pancreas, Franz or Hamoudi tumor Definitions • Epithelial tumor of exocrine pancreas with low-grade malignant potential and solid and cystic features IMAGING General Features • Best diagnostic clue ○ Large, encapsulated, round, complex cystic pancreatic mass with no associated ductal dilatation • Location ○ Commonly in pancreatic tail • Size ○ Usually large (average: 10 cm; range: 2.5-20 cm) • Morphology ○ Typically well-defined, large, solid and cystic mass ○ Lesions < 3 cm show solid, homogeneous appearance ○ May contain dystrophic calcifications ○ Aggressive features are uncommon, but can be seen – Perivascular invasion, ductal dilation; metastases usually to liver, but also lymph nodes and peritoneum Ultrasonographic Findings • Well-defined, heterogeneous mass with solid and cystic components ○ Hypoechoic center due to tumor necrosis/hemorrhage ○ Cystic portion may show fluid-debris level • Color Doppler: Hypovascular, due to areas of necrosis • Endoscopic ultrasound (EUS): More sensitive for small mass ○ Can guide fine-needle aspiration biopsy CT Findings • Heterogeneous density with mixed solid/cystic areas • Enhancing capsule and soft tissue projections • Hyperdensity on unenhanced CT from hemorrhage; low attenuation from nonenhancing areas of necrosis MR Findings • Well-demarcated mass with central areas of heterogeneously bright T1 and T2 signal ○ Low T1 SI: Capsule and solid components that enhance on post-contrast T1WI ○ High T1 SI: Intratumoral hemorrhage ○ High T2 signal: Necrosis and hemorrhage ○ Low T2 signal: Thick fibrous capsule Imaging Recommendations • Best imaging tool ○ Multiplanar CECT or MR • Protocol advice ○ Unenhanced CT and MR can best demonstrate intratumoral hemorrhage ○ Post-contrast imaging should include arterial phase DIFFERENTIAL DIAGNOSIS Mucinous Cystic Pancreatic Tumor • No hemorrhage; commonly seen in middle-aged women Nonfunctioning Neuroendocrine Tumor • Cystic components typically do not show T1 hyperintensity; peripheral portions are more hypervascular Pancreatic Serous Cystadenoma • No large solid component; usually located in head of pancreas; more common in elderly women Pancreatic Ductal Carcinoma • Rarely necrotic or hemorrhagic; typically shows pancreatic &/or common bile duct obstruction; older adults PATHOLOGY General Features • Large solitary tumor with variable mixture of solid, hemorrhagic, and necrotic components • Low malignant potential Gross Pathologic & Surgical Features • Thick, fibrous, hypervascular capsule surrounding soft tumor, usually with no mass effect/ductal obstruction Microscopic Features • Solid nests of homogeneous, epithelioid cells with areas of separation into pseudopapillary aggregates due to degeneration CLINICAL ISSUES Presentation • Usually asymptomatic or nonspecific abdominal pain • May have palpable abdominal mass Demographics • ~ 90% female; < 35 years of age • African Americans or non-Caucasian groups Natural History & Prognosis • Usually benign, but with low malignant potential • Prognosis: Excellent after surgical resection; usually curative (95% 5-year survival) • < 10% metastasize (usually to liver) or recur Treatment • Complete surgical excision DIAGNOSTIC CHECKLIST Image Interpretation Pearls • Encapsulated pancreatic tail mass with solid, cystic, and hemorrhagic components in young non-Caucasian female SELECTED REFERENCES 1. Ganeshan DM et al: Solid pseudo-papillary tumors of the pancreas: current update. Abdom Imaging. 38(6):1373-82, 2013 2. Reddy S et al: Surgical management of solid-pseudopapillary neoplasms of the pancreas (Franz or Hamoudi tumors): a large single-institutional series. J Am Coll Surg. 208(5):950-7; discussion 957-9, 2009 3. Choi JY et al: Solid pseudopapillary tumor of the pancreas: typical and atypical manifestations. AJR Am J Roentgenol. 187(2):W178-86, 2006 Diagnoses: Pancreas 389
Solid Pseudopapillary Neoplasm Diagnoses: Pancreas (Left) Axial T2 MR shows a small, well-defined T2-bright lesion in the pancreatic body . Notice that the signal is less intense than that of fluid st in the gallbladder and spinal canal . (Right) Corresponding axial T1 C+ FS MR shows a small hypoenhancing mass in the body of the pancreas mimicking, pancreatic adenocarcinoma except for the notable lack of pancreatic ductal dilation or surrounding infiltration . (Left) Axial CECT shows a heterogeneous mass with solid ſt and cystic st components in the pancreatic body in a 19-year-old woman. (Right) Corresponding coronal CECT shows the large, predominantly cystic mass with an enhancing rim and peripheral soft tissue ſt. (Left) Corresponding endoscopic US in the same patient shows a heterogeneous appearance with solid ſt and small anechoic cystic areas and a larger complex cystic component . (Right) Endoscopic ultrasound-guided biopsy shows the needle st within a peripheral soft tissue nodular component ſt of the large solid and cystic pancreatic mass , which was a proven SPN. 390
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Solid Pseudopapillary Neoplasm<br />
TERMINOLOGY<br />
Abbreviations<br />
• Solid pseudopapillary neoplasm (SPN)<br />
Synonyms<br />
• Solid <strong>and</strong> papillary epithelial neoplasm (SPEN); papillary<br />
cystic epithelial neoplasm; papillary cystic tumor; solid <strong>and</strong><br />
cystic tumor of pancreas, Franz or Hamoudi tumor<br />
Definitions<br />
• Epithelial tumor of exocrine pancreas with low-grade<br />
malignant potential <strong>and</strong> solid <strong>and</strong> cystic features<br />
IMAGING<br />
General Features<br />
• Best diagnostic clue<br />
○ Large, encapsulated, round, complex cystic pancreatic<br />
mass with no associated ductal dilatation<br />
• Location<br />
○ Commonly in pancreatic tail<br />
• Size<br />
○ Usually large (average: 10 cm; range: 2.5-20 cm)<br />
• Morphology<br />
○ Typically well-defined, large, solid <strong>and</strong> cystic mass<br />
○ Lesions < 3 cm show solid, homogeneous appearance<br />
○ May contain dystrophic calcifications<br />
○ Aggressive features are uncommon, but can be seen<br />
– Perivascular invasion, ductal dilation; metastases<br />
usually to liver, but also lymph nodes <strong>and</strong> peritoneum<br />
Ultrasonographic Findings<br />
• Well-defined, heterogeneous mass with solid <strong>and</strong> cystic<br />
components<br />
○ Hypoechoic center due to tumor necrosis/hemorrhage<br />
○ Cystic portion may show fluid-debris level<br />
• Color Doppler: Hypovascular, due to areas of necrosis<br />
• Endoscopic ultrasound (EUS): More sensitive for small mass<br />
○ Can guide fine-needle aspiration biopsy<br />
CT Findings<br />
• Heterogeneous density with mixed solid/cystic areas<br />
• Enhancing capsule <strong>and</strong> soft tissue projections<br />
• Hyperdensity on unenhanced CT from hemorrhage; low<br />
attenuation from nonenhancing areas of necrosis<br />
MR Findings<br />
• Well-demarcated mass with central areas of<br />
heterogeneously bright T1 <strong>and</strong> T2 signal<br />
○ Low T1 SI: Capsule <strong>and</strong> solid components that enhance<br />
on post-contrast T1WI<br />
○ High T1 SI: Intratumoral hemorrhage<br />
○ High T2 signal: Necrosis <strong>and</strong> hemorrhage<br />
○ Low T2 signal: Thick fibrous capsule<br />
Imaging Recommendations<br />
• Best imaging tool<br />
○ Multiplanar CECT or MR<br />
• Protocol advice<br />
○ Unenhanced CT <strong>and</strong> MR can best demonstrate<br />
intratumoral hemorrhage<br />
○ Post-contrast imaging should include arterial phase<br />
DIFFERENTIAL DIAGNOSIS<br />
Mucinous Cystic Pancreatic Tumor<br />
• No hemorrhage; commonly seen in middle-aged women<br />
Nonfunctioning Neuroendocrine Tumor<br />
• Cystic components typically do not show T1 hyperintensity;<br />
peripheral portions are more hypervascular<br />
Pancreatic Serous Cystadenoma<br />
• No large solid component; usually located in head of<br />
pancreas; more common in elderly women<br />
Pancreatic Ductal Carcinoma<br />
• Rarely necrotic or hemorrhagic; typically shows pancreatic<br />
&/or common bile duct obstruction; older adults<br />
PATHOLOGY<br />
General Features<br />
• Large solitary tumor with variable mixture of solid,<br />
hemorrhagic, <strong>and</strong> necrotic components<br />
• Low malignant potential<br />
Gross Pathologic & Surgical Features<br />
• Thick, fibrous, hypervascular capsule surrounding soft<br />
tumor, usually with no mass effect/ductal obstruction<br />
Microscopic Features<br />
• Solid nests of homogeneous, epithelioid cells with areas of<br />
separation into pseudopapillary aggregates due to<br />
degeneration<br />
CLINICAL ISSUES<br />
Presentation<br />
• Usually asymptomatic or nonspecific abdominal pain<br />
• May have palpable abdominal mass<br />
Demographics<br />
• ~ 90% female; < 35 years of age<br />
• African Americans or non-Caucasian groups<br />
Natural History & Prognosis<br />
• Usually benign, but with low malignant potential<br />
• Prognosis: Excellent after surgical resection; usually<br />
curative (95% 5-year survival)<br />
• < 10% metastasize (usually to liver) or recur<br />
Treatment<br />
• Complete surgical excision<br />
DIAGNOSTIC CHECKLIST<br />
Image Interpretation Pearls<br />
• Encapsulated pancreatic tail mass with solid, cystic, <strong>and</strong><br />
hemorrhagic components in young non-Caucasian female<br />
SELECTED REFERENCES<br />
1. Ganeshan DM et al: Solid pseudo-papillary tumors of the pancreas: current<br />
update. Abdom Imaging. 38(6):1373-82, 2013<br />
2. Reddy S et al: Surgical management of solid-pseudopapillary neoplasms of<br />
the pancreas (Franz or Hamoudi tumors): a large single-institutional series. J<br />
Am Coll Surg. 208(5):950-7; discussion 957-9, 2009<br />
3. Choi JY et al: Solid pseudopapillary tumor of the pancreas: typical <strong>and</strong><br />
atypical manifestations. AJR Am J Roentgenol. 187(2):W178-86, 2006<br />
Diagnoses: Pancreas<br />
389