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Diagnostic Ultrasound - Abdomen and Pelvis

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Pancreatic Neuroendocrine Tumor<br />

TERMINOLOGY<br />

Synonyms<br />

• Pancreatic/gastroenteropancreatic neuroendocrine tumor<br />

(NET); pancreatic endocrine tumor (pET)<br />

• Historical terms: Islet cell tumor; carcinoid<br />

Definitions<br />

• Solid epithelial neoplasm believed to arise from pluripotent<br />

pancreatic ductal cells with capacity to differentiate along<br />

neuroendocrine lines<br />

IMAGING<br />

General Features<br />

• Best diagnostic clue<br />

○ Well-circumscribed, round hypervascular mass(es); no<br />

pancreatic ductal dilation<br />

○ Calcification, necrosis, <strong>and</strong> cystic change in larger tumors<br />

• Location<br />

○ 85% within pancreas: Usually body/tail; 15% ectopic<br />

– Duodenum, stomach, lymph nodes, ovaries<br />

○ May be multiple<br />

• Size<br />

○ Variable: Range from < 1 cm to > 20 cm; typically 1-5 cm<br />

– Functioning tumors: Smaller at time of presentation<br />

– Nonfunctioning tumors: Usually larger at diagnosis<br />

• Morphology: Functioning vs. nonfunctioning subtypes<br />

○ Functioning tumors: Small, round, enhancing mass<br />

– May be subtle<br />

○ Nonfunctioning: Large, well-demarcated, lobulated mass<br />

with heterogeneous enhancement pattern<br />

– Areas of cystic change/necrosis <strong>and</strong> calcification<br />

– Displaces, rather than invades, adjacent structures<br />

Transabdominal <strong>Ultrasound</strong> (TA US)<br />

• Well-defined, round, hypoechoic mass; can appear<br />

isoechoic (focal contour asymmetry)<br />

• Large tumors may be echogenic ± calcification &/or central<br />

hypoechogenicity (necrosis)<br />

○ Intratumoral calcification suggests malignancy<br />

○ 60-90% have adenopathy <strong>and</strong> liver metastases at clinical<br />

presentation<br />

– Hyperechoic, hypoechoic, or target lesions<br />

• Color Doppler: Demonstrates intratumoral flow<br />

• Reported sensitivity for detection of small tumors ~ 25-60%<br />

○ Limited role in detection for functional NETs given<br />

typical small tumor size <strong>and</strong> body habitus limitations<br />

Endoscopic <strong>Ultrasound</strong> (EUS)<br />

• ↑ sensitivity for detection of small tumors (up to 94%)<br />

• Can guide biopsy <strong>and</strong> sample cyst fluid<br />

Intraoperative <strong>Ultrasound</strong> (IOUS)<br />

• Highest sensitivity for small lesions (75-100%)<br />

CT Findings<br />

• CECT<br />

○ Smoothly marginated <strong>and</strong> hypervascular (arterial/PV<br />

phases)<br />

○ Large lesions: Heterogeneous due to nonenhancing<br />

cystic/necrotic areas ± calcification<br />

○ Cystic variant: Hypervascular rim, central necrosis<br />

(distinguishing feature vs. other cystic pancreatic lesions)<br />

○ Rarely appears infiltrative (poorly differentiated NET)<br />

○ Liver <strong>and</strong> nodal metastases: Hypervascular, often with<br />

ring-like enhancement in liver lesions<br />

MR Findings<br />

• T1WI ± FS: Hypointense to normal pancreas<br />

○ Unenhanced, FS T1WI has highest sensitivity (75%)<br />

• T2WI ± FS: Usually hyperintense to normal pancreas<br />

• T1 C+: Hypervascular<br />

○ Small tumors: Homogeneous enhancement pattern<br />

○ Large tumors: More heterogeneous with nonenhancing<br />

cystic, necrotic/hemorrhagic areas <strong>and</strong> hyperenhancing<br />

tumor<br />

○ Liver <strong>and</strong> nodal metastases: Prominent enhancement,<br />

ring like in liver<br />

Nuclear Medicine Findings<br />

• Octreotide scintigraphy<br />

○ Limitations: Uptake by nontarget sites, ↑ image<br />

acquisition time, poor image resolution<br />

• PET/CT with Ga-68-(DFO)-octreotide<br />

○ Advantages: Rapid excretion from nontarget sites, ↓<br />

acquisition time, CT localization → ↑ precision<br />

• Useful for metastatic disease or if primary lesion<br />

undetected by other imaging modalities<br />

○ Low sensitivity for insulinomas (↓ expression of<br />

somatostatin receptors)<br />

Imaging Recommendations<br />

• Best imaging tool<br />

○ Contrast-enhanced CT or MR<br />

○ Endoscopic US: ↑ detection rate for small tumors<br />

compared to transabdominal US<br />

– Invasive technique<br />

– Performed for localization if CT/MR is negative with<br />

high clinical suspicion or to guide biopsy<br />

○ Even if initial CECT/MR/US are negative, if positive<br />

biochemical evidence exists, further imaging studies are<br />

essential<br />

– Consider somatostatin receptor functional imaging<br />

○ Intraoperative ultrasound helps ensure complete<br />

detection/resection of small tumors<br />

• Protocol advice<br />

○ CT: Dual phase pancreatic protocol should be performed<br />

(late arterial <strong>and</strong> portal venous phases)<br />

– Arterial phase increases conspicuity of small lesions<br />

○ MR: Include T1/T2 ± FS <strong>and</strong> C+ T1W FS dynamic imaging<br />

DIFFERENTIAL DIAGNOSIS<br />

Mucinous Cystic Pancreatic Tumor<br />

• Multiloculated cystic mass with septations<br />

• Can appear similar to cystic NET variant<br />

• Usually with larger cystic component <strong>and</strong> no thick<br />

enhancing rind<br />

Solid Pseudopapillary Neoplasm<br />

• Rare solid <strong>and</strong> cystic mass than can appear similar to a<br />

nonfunctioning NET<br />

• Occurs in overlapping younger age demographic<br />

Diagnoses: Pancreas<br />

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