PMC4861888_01
65-year-old male with pancreatic differentiated MANEC within the left hepatic lobe. Frontal radiograph of the abdomen demonstrates a large opacity (open arrows) in the right upper quadrant with mass effect on the adjacent air-filled bowel and stomach.
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Abdominal radiographs demonstrated a mass in the upper abdomen with mass effect on the surrounding bowel (Fig. 1).
65-year-old male with pancreatic differentiated MANEC within the left hepatic lobe. Axial (A) and coronal (B) CT images of the abdomen show a 13.5 x 11.8 x 10.5-cm, heterogeneously enhancing mass in the left hepatic lobe with central hypodensity (open arrow). No calcifications or satellite lesions are seen. Incidental simple hepatic cysts are nearby.
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A subsequent CT of the abdomen and pelvis, enhanced with intravenous and oral contrast and imaged during the portal venous phase, revealed a lobulated 13.5 x 11.8 x 10.5-cm, heterogeneously enhancing mass in the left hepatic lobe, with central hypoattenuating material thought to represent necrosis (Fig. 2).
65-year-old male with pancreatic differentiated MANEC within the left hepatic lobe. Axial T1 (A) and T2 (B) weighted images show a large lobulated tumor in the left hepatic lobe with a T1- hypointense, T2-hyperintense scar (open arrow) with multiple surrounding simple cysts (stars) and intratumoral foci of hemorrhage at the periphery.
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Outside of a 7mm hypodensity in the pancreatic head, the pancreas was unremarkable, with no suspicious mass (Fig. 3).
65-year-old male with pancreatic differentiated MANEC within the left hepatic lobe. Axial diffusion-weighted imaging (A) demonstrates high signal throughout the tumor. The corresponding ADC sequence (B) shows high signal intensity within the central scar due to T2 "shine-through" and confirms restricted diffusion in the surrounding tumor parenchyma.
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T1 and T2 sequences, with a T2-hyperintense central scar (Fig. 4). The tumor was heterogeneously hyperenhancing in the arterial phase, with relative washout in the portal venous phase.
65-year-old male with pancreatic differentiated MANEC within the left hepatic lobe. Contrast-enhanced axial LAVA FS images during the arterial phase (A), at 5 min (B), and at 10 min (C) demonstrate arterial enhancement of the MANEC followed by slight washout relative to the liver. The central scar demonstrates delayed enhancement (C).
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A central scar was noted that demonstrated progressive accumulation of the gadolinium-based contrast agent (GBCA) on 10-minute delayed and hepatobiliary (60-minute) phases (Figure 5, Figure 6).
65-year-old male with pancreatic differentiated MANEC within the left hepatic lobe. Contrast-enhanced coronal LAVA FS image at 60 minutes (hepatobiliary phase) demonstrates no retention of the hepatobiliary agent in the tumor parenchyma. Progressive enhancement of the central scar is seen, but not due to biliary excretion of the hepatobiliary agent; rather, the dense cellular matrix of the scar traps the gadolinium chelate.
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A central scar was noted that demonstrated progressive accumulation of the gadolinium-based contrast agent (GBCA) on 10-minute delayed and hepatobiliary (60-minute) phases (Figure 5, Figure 6).
65-year-old male with pancreatic differentiated MANEC within the left hepatic lobe. Whole-body planar images 24 hours after the administration of 5.98 mCi of indium-111 demonstrate physiologic uptake within the liver, spleen, kidneys, and bowel with no evidence of radiotracer uptake within the hepatic tumor.
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This study revealed no tracer uptake in the hepatic mass, pancreas, bowel, or elsewhere in the body (Fig. 7).
65-year-old male with pancreatic differentiated MANEC within the left hepatic lobe. (A) The large lobulated tumor within the left hepatic lobe, status post lobectomy. (B) The transected tumor with a central stellate scar.
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The patient proceeded to left hepatic lobectomy, with specimens sent to two separate tertiary referral centers for consultation (see Fig. 8 for gross specimen).
65-year-old male with pancreatic differentiated MANEC within the left hepatic lobe. Immunohistochemical stains of the MANEC demonstrate weak, diffuse staining with chromogranin (A) and patchy staining with synaptophysin (B), both of which are neuroendocrine markers. The WHO requirement for the diagnosis of MANEC requires at least 30% acinar and neuroendocrine differentiation.
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Additional immunohistochemical stains demonstrated a moderately poorly differentiated carcinoma with mixed neuroendocrine and acinar cell differentiation, with stronger chromogranin consistent with predominant neuroendocrine differentiation (Fig. 9).