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Neuroendocrine Tumors of the Pancreas in von Hippel–Lindau Disease: Spectrum of Appearances at CT and MR Imaging with Histopathologic Comparison¹

¹ From the Department of Diagnostic Radiology (H.B.M., P.L.C.), Surgery Branch (S.K.L., H.R.A., D.L.B.), Urologic Oncology Branch (M.M.W., W.M.L., G.M.G., P.L.C.), Genetic Epidemiology Branch (G.M.G.), and Surgical Neurology Branch (I.A.L.), National Cancer Institute, National Institutes of Health, 10 Center Dr, MSC 1182, Bldg 10, Rm 1C660, Bethesda, MD 20892-1182. Received July 30, 2001; revision requested September 25; final revision received November 8; accepted May 14, 2002. Address correspondence to P.L.C. (e-mail: pchoyke@nih.gov).

View larger version (16K): [in a new window]   Figure 1. Graph illustrates findings of tumor size versus DT in the current study.

View larger version (106K): [in a new window]   Figure 2a. Transverse CT and MR images obtained in a 35-year-old woman with VHL syndrome and pancreatic NET. (a) Contrast-enhanced CT scan demonstrates a 2.0 x 1.8-cm mass in the pancreatic head (arrows) that is homogeneously enhancing. (b) T1-weighted spoiled gradient-echo MR image with fat suppression (150/1.9; flip angle, 70°) obtained by using a phased-array coil at the same tomographic location 7 months after a shows the same mass. The mass (arrows) is well defined, low in signal intensity, and has increased slightly in size (2.2 x 2.0 cm). (c) Transverse T2-weighted fast spin-echo MR image (5,700/126) shows that the mass (arrows) is moderately high in signal intensity. (d) Early and (e) delayed postcontrast T1-weighted spoiled gradient-echo MR images with fat suppression (150/1.9; flip angle, 70°) show an intense homogeneous enhancement pattern in the mass (arrows) that remains higher in signal intensity than does background pancreatic tissue. This finding reflects the abundant blood supply of the tumor. (f) Histopathologic specimen from the resected tumor shows classic trabecular architecture (short arrow) and small NET cells (long arrow) with eosinophilic cytoplasm. (Hematoxylin-eosin stain; original magnification, x400.)

View larger version (115K): [in a new window]   Figure 2b. Transverse CT and MR images obtained in a 35-year-old woman with VHL syndrome and pancreatic NET. (a) Contrast-enhanced CT scan demonstrates a 2.0 x 1.8-cm mass in the pancreatic head (arrows) that is homogeneously enhancing. (b) T1-weighted spoiled gradient-echo MR image with fat suppression (150/1.9; flip angle, 70°) obtained by using a phased-array coil at the same tomographic location 7 months after a shows the same mass. The mass (arrows) is well defined, low in signal intensity, and has increased slightly in size (2.2 x 2.0 cm). (c) Transverse T2-weighted fast spin-echo MR image (5,700/126) shows that the mass (arrows) is moderately high in signal intensity. (d) Early and (e) delayed postcontrast T1-weighted spoiled gradient-echo MR images with fat suppression (150/1.9; flip angle, 70°) show an intense homogeneous enhancement pattern in the mass (arrows) that remains higher in signal intensity than does background pancreatic tissue. This finding reflects the abundant blood supply of the tumor. (f) Histopathologic specimen from the resected tumor shows classic trabecular architecture (short arrow) and small NET cells (long arrow) with eosinophilic cytoplasm. (Hematoxylin-eosin stain; original magnification, x400.)

View larger version (115K): [in a new window]   Figure 2c. Transverse CT and MR images obtained in a 35-year-old woman with VHL syndrome and pancreatic NET. (a) Contrast-enhanced CT scan demonstrates a 2.0 x 1.8-cm mass in the pancreatic head (arrows) that is homogeneously enhancing. (b) T1-weighted spoiled gradient-echo MR image with fat suppression (150/1.9; flip angle, 70°) obtained by using a phased-array coil at the same tomographic location 7 months after a shows the same mass. The mass (arrows) is well defined, low in signal intensity, and has increased slightly in size (2.2 x 2.0 cm). (c) Transverse T2-weighted fast spin-echo MR image (5,700/126) shows that the mass (arrows) is moderately high in signal intensity. (d) Early and (e) delayed postcontrast T1-weighted spoiled gradient-echo MR images with fat suppression (150/1.9; flip angle, 70°) show an intense homogeneous enhancement pattern in the mass (arrows) that remains higher in signal intensity than does background pancreatic tissue. This finding reflects the abundant blood supply of the tumor. (f) Histopathologic specimen from the resected tumor shows classic trabecular architecture (short arrow) and small NET cells (long arrow) with eosinophilic cytoplasm. (Hematoxylin-eosin stain; original magnification, x400.)

View larger version (110K): [in a new window]   Figure 2d. Transverse CT and MR images obtained in a 35-year-old woman with VHL syndrome and pancreatic NET. (a) Contrast-enhanced CT scan demonstrates a 2.0 x 1.8-cm mass in the pancreatic head (arrows) that is homogeneously enhancing. (b) T1-weighted spoiled gradient-echo MR image with fat suppression (150/1.9; flip angle, 70°) obtained by using a phased-array coil at the same tomographic location 7 months after a shows the same mass. The mass (arrows) is well defined, low in signal intensity, and has increased slightly in size (2.2 x 2.0 cm). (c) Transverse T2-weighted fast spin-echo MR image (5,700/126) shows that the mass (arrows) is moderately high in signal intensity. (d) Early and (e) delayed postcontrast T1-weighted spoiled gradient-echo MR images with fat suppression (150/1.9; flip angle, 70°) show an intense homogeneous enhancement pattern in the mass (arrows) that remains higher in signal intensity than does background pancreatic tissue. This finding reflects the abundant blood supply of the tumor. (f) Histopathologic specimen from the resected tumor shows classic trabecular architecture (short arrow) and small NET cells (long arrow) with eosinophilic cytoplasm. (Hematoxylin-eosin stain; original magnification, x400.)

View larger version (98K): [in a new window]   Figure 2e. Transverse CT and MR images obtained in a 35-year-old woman with VHL syndrome and pancreatic NET. (a) Contrast-enhanced CT scan demonstrates a 2.0 x 1.8-cm mass in the pancreatic head (arrows) that is homogeneously enhancing. (b) T1-weighted spoiled gradient-echo MR image with fat suppression (150/1.9; flip angle, 70°) obtained by using a phased-array coil at the same tomographic location 7 months after a shows the same mass. The mass (arrows) is well defined, low in signal intensity, and has increased slightly in size (2.2 x 2.0 cm). (c) Transverse T2-weighted fast spin-echo MR image (5,700/126) shows that the mass (arrows) is moderately high in signal intensity. (d) Early and (e) delayed postcontrast T1-weighted spoiled gradient-echo MR images with fat suppression (150/1.9; flip angle, 70°) show an intense homogeneous enhancement pattern in the mass (arrows) that remains higher in signal intensity than does background pancreatic tissue. This finding reflects the abundant blood supply of the tumor. (f) Histopathologic specimen from the resected tumor shows classic trabecular architecture (short arrow) and small NET cells (long arrow) with eosinophilic cytoplasm. (Hematoxylin-eosin stain; original magnification, x400.)

View larger version (121K): [in a new window]   Figure 2f. Transverse CT and MR images obtained in a 35-year-old woman with VHL syndrome and pancreatic NET. (a) Contrast-enhanced CT scan demonstrates a 2.0 x 1.8-cm mass in the pancreatic head (arrows) that is homogeneously enhancing. (b) T1-weighted spoiled gradient-echo MR image with fat suppression (150/1.9; flip angle, 70°) obtained by using a phased-array coil at the same tomographic location 7 months after a shows the same mass. The mass (arrows) is well defined, low in signal intensity, and has increased slightly in size (2.2 x 2.0 cm). (c) Transverse T2-weighted fast spin-echo MR image (5,700/126) shows that the mass (arrows) is moderately high in signal intensity. (d) Early and (e) delayed postcontrast T1-weighted spoiled gradient-echo MR images with fat suppression (150/1.9; flip angle, 70°) show an intense homogeneous enhancement pattern in the mass (arrows) that remains higher in signal intensity than does background pancreatic tissue. This finding reflects the abundant blood supply of the tumor. (f) Histopathologic specimen from the resected tumor shows classic trabecular architecture (short arrow) and small NET cells (long arrow) with eosinophilic cytoplasm. (Hematoxylin-eosin stain; original magnification, x400.)

View larger version (115K): [in a new window]   Figure 3a. Transverse contrast-enhanced CT scans obtained in a 21-year-old woman with VHL syndrome and an enhancing uncinate-process mass that is growing slowly. (a) CT scan shows a 1.3 x 1.5-cm homogeneously enhancing mass in the uncinate process (arrows). (b) CT scan obtained 2 years after a shows only slight growth of the mass (1.5 x 1.7 cm; DT, 638 days).

View larger version (114K): [in a new window]   Figure 3b. Transverse contrast-enhanced CT scans obtained in a 21-year-old woman with VHL syndrome and an enhancing uncinate-process mass that is growing slowly. (a) CT scan shows a 1.3 x 1.5-cm homogeneously enhancing mass in the uncinate process (arrows). (b) CT scan obtained 2 years after a shows only slight growth of the mass (1.5 x 1.7 cm; DT, 638 days).

View larger version (101K): [in a new window]   Figure 4a. Images obtained in a 39-year-old woman with VHL syndrome and "occult" NET. (a) Transverse contrast-enhanced CT scan acquired during the equilibrium phase shows no identifiable mass in the pancreatic head. Acquisition of this scan was delayed from the time of injection. (b) Transverse contrast-enhanced arterial phase T1-weighted spoiled gradient-echo MR image with fat suppression (150/1.9; flip angle, 70°) shows homogeneous enhancement of the 2.0-cm mass (arrows). (c) Histopathologic specimen from the resected tumor shows solid architecture, small vessels (short arrows), and cells with prominent clear cytoplasm (long arrow). (Hematoxylin-eosin stain; original magnification, x400.) At gross examination, this tumor demonstrated prominent yellow coloring secondary to abundant lipid content.

View larger version (88K): [in a new window]   Figure 4b. Images obtained in a 39-year-old woman with VHL syndrome and "occult" NET. (a) Transverse contrast-enhanced CT scan acquired during the equilibrium phase shows no identifiable mass in the pancreatic head. Acquisition of this scan was delayed from the time of injection. (b) Transverse contrast-enhanced arterial phase T1-weighted spoiled gradient-echo MR image with fat suppression (150/1.9; flip angle, 70°) shows homogeneous enhancement of the 2.0-cm mass (arrows). (c) Histopathologic specimen from the resected tumor shows solid architecture, small vessels (short arrows), and cells with prominent clear cytoplasm (long arrow). (Hematoxylin-eosin stain; original magnification, x400.) At gross examination, this tumor demonstrated prominent yellow coloring secondary to abundant lipid content.

View larger version (148K): [in a new window]   Figure 4c. Images obtained in a 39-year-old woman with VHL syndrome and "occult" NET. (a) Transverse contrast-enhanced CT scan acquired during the equilibrium phase shows no identifiable mass in the pancreatic head. Acquisition of this scan was delayed from the time of injection. (b) Transverse contrast-enhanced arterial phase T1-weighted spoiled gradient-echo MR image with fat suppression (150/1.9; flip angle, 70°) shows homogeneous enhancement of the 2.0-cm mass (arrows). (c) Histopathologic specimen from the resected tumor shows solid architecture, small vessels (short arrows), and cells with prominent clear cytoplasm (long arrow). (Hematoxylin-eosin stain; original magnification, x400.) At gross examination, this tumor demonstrated prominent yellow coloring secondary to abundant lipid content.

View larger version (119K): [in a new window]   Figure 5. Transverse contrast-enhanced CT scan obtained in a 31-year-old man with VHL syndrome who had a pancreatic mass and hepatic metastases at presentation. The scan shows an ill-defined 6.0 x 4.0-cm mass (long arrows) at the midbody of the pancreas that resulted in encasement of the superior mesenteric artery and vein and splenic artery. The mass is enhancing heterogeneously. Multiple hypervascular enhancing focal hepatic metastases (short solid arrow indicates one) are also seen. Note the left adrenal mass (pheochromocytoma) (open arrow). The patient underwent surgical removal of the pheochromocytoma and hepatic biopsy for hepatic metastasis.

View larger version (129K): [in a new window]   Figure 6a. Transverse T1-weighted spoiled gradient-echo MR images with fat suppression obtained in a 32-year-old woman with VHL syndrome that manifested as a large mass in the pancreatic tail and as hepatic metastases. (a) Precontrast MR image (150/1.9; flip angle, 70°) demonstrates an ill-defined heterogeneous mass with low signal intensity (short arrows) that measures 4.5 x 3.0 cm and is located in the pancreatic tail, slightly medial and posterior to the posterior gastric wall. Two focal low-signal-intensity masses (long arrows) are seen in the right and left lobes of the liver. (b) Postcontrast MR image (150/1.9; flip angle, 70°) obtained 90 seconds after injection and at the same tomographic location as a shows the mass (short arrows) enhancing heterogeneously. The tumor heterogeneity seen on the pre- and postcontrast images is consistent with necrosis. Hypervascular hepatic metastases (long arrows) are also noted. On the 4-month follow-up MR image (not shown), the tumor showed a size increase that reflected a higher growth rate (DT, 277 days). The patient underwent distal pancreatectomy and radio-frequency thermal ablation of hepatic metastases. Histopathologic examination of the resected tumor showed features similar to those described for the tumors smaller than 3.0 cm. However, malignant microscopic infiltration of adjacent organs and hepatic metastases were identified.

View larger version (132K): [in a new window]   Figure 6b. Transverse T1-weighted spoiled gradient-echo MR images with fat suppression obtained in a 32-year-old woman with VHL syndrome that manifested as a large mass in the pancreatic tail and as hepatic metastases. (a) Precontrast MR image (150/1.9; flip angle, 70°) demonstrates an ill-defined heterogeneous mass with low signal intensity (short arrows) that measures 4.5 x 3.0 cm and is located in the pancreatic tail, slightly medial and posterior to the posterior gastric wall. Two focal low-signal-intensity masses (long arrows) are seen in the right and left lobes of the liver. (b) Postcontrast MR image (150/1.9; flip angle, 70°) obtained 90 seconds after injection and at the same tomographic location as a shows the mass (short arrows) enhancing heterogeneously. The tumor heterogeneity seen on the pre- and postcontrast images is consistent with necrosis. Hypervascular hepatic metastases (long arrows) are also noted. On the 4-month follow-up MR image (not shown), the tumor showed a size increase that reflected a higher growth rate (DT, 277 days). The patient underwent distal pancreatectomy and radio-frequency thermal ablation of hepatic metastases. Histopathologic examination of the resected tumor showed features similar to those described for the tumors smaller than 3.0 cm. However, malignant microscopic infiltration of adjacent organs and hepatic metastases were identified.

View larger version (120K): [in a new window]   Figure 7a. Transverse CT and MR images obtained in a 23-year-old woman with VHL syndrome that manifested as a 4.0-cm mass in the pancreatic tail. (a) Transverse contrast-enhanced CT scan shows a 4.0 x 3.2-cm mass with ill-defined margins (thick arrows) in the pancreatic tail. The mass enhances heterogeneously, with multiple areas of calcification (thin arrow). (b) Early postcontrast T1-weighted spoiled gradient-echo MR image with fat suppression (150/1.9; flip angle, 70°) shows the mass (thick arrows) enhancing heterogeneously, with a central signal void in the calcification (thin arrow). The patient underwent distal pancreatectomy.

View larger version (132K): [in a new window]   Figure 7b. Transverse CT and MR images obtained in a 23-year-old woman with VHL syndrome that manifested as a 4.0-cm mass in the pancreatic tail. (a) Transverse contrast-enhanced CT scan shows a 4.0 x 3.2-cm mass with ill-defined margins (thick arrows) in the pancreatic tail. The mass enhances heterogeneously, with multiple areas of calcification (thin arrow). (b) Early postcontrast T1-weighted spoiled gradient-echo MR image with fat suppression (150/1.9; flip angle, 70°) shows the mass (thick arrows) enhancing heterogeneously, with a central signal void in the calcification (thin arrow). The patient underwent distal pancreatectomy.

View larger version (23K): [in a new window]   Figure 8. Drawing illustrates the location and size of pancreatic NETs in the current study. Dotted lesions are tumors associated with hepatic metastases.