Intraductal papillary bile duct adenocarcinoma and gastrointestinal stromal tumor in a case of neurofibromatosis type 1

Figure 1 General appearance. A and B: A patient diagnosed with neurofibromatosis type 1 presented with café au lait spots on the skin. 300 mm × 225 mm (300 × 300 DPI).

Figure 2 Computed tomography. A: A low-attenuated multilocular mass with septation, and a mural nodule containing a soft tissue-enhancing lesion in the right hepatic lobe (white arrow); B: Diffuse intrahepatic duct (IHD) dilatation, especially in the right hepatic lobe, with a suspicious soft tissue lesion connected to the IHD. A 1.7-cm-sized, well-demarcated enhancing mass is observed in the third portion of the duodenum (white arrow). 300 mm × 225 mm (300 × 300 DPI).

Figure 3 Magnetic resonance cholangiopancreatography. A: A lobulated cystic mass in the right hepatic lobe with multifocal intramural enhancing nodules (white arrow); B: The connection of the cystic mass with the intrahepatic duct indicated a malignant transformation (white arrow). 300 mm × 225 mm (300 × 300 DPI).

Figure 4 Histologic findings of gastrointestinal stromal tumor. A: A mass with rounded border, resected from the third portion of the duodenum. (HE, 40 × magnification); B: The resected duodenal mass contained many spindle cells. (HE, 100 ×); C: Positive immunohistochemistry for c-kit suggested GIST. (c-kit immunostain, 100 ×). 300 mm × 225 mm (300 × 300 DPI). GIST: Gastrointestinal stromal tumor.

Figure 5 Histologic findings of intraductal papillary neoplasm of bile duct. A: The resected hepatic mass was a multilocular cystic neoplasm with septation and a mural nodule; B: It had communication to segmental bile duct, although it was not connected to main bile duct (white arrow: Main bile duct); C: In the dilated bile duct, normal epithelial lining with abrupt papillary epithelial portions suggests IPNB. (HE, 100 ×); D: Multifocal stromal invasion multifocal stromal invasion with a tubulopapillary mucin component, suggestive of invasive adenocarcinoma with high-grade dyplasia. (HE, 40 ×). 300 mm × 225 mm (300 × 300 DPI).