A long-term survivor of intrahepatic cholangiocarcinoma with paraaortic lymph node metastasis

Abstract

of hepatic tumors was minimal over 7 years (see Fig. 1), very unlikely as metastatic tumors. Additionally, we failed to find any possible primary site for somatostatinoma, even though extensive searches for the primary sites were performed using upper and lower GI endoscopy, CT scan of the head, neck, and trunk, and 67Ga scintigraphy of the whole body. Therefore, it is reasonable to assume that the tumor originated from the liver. Hepatic tumors observed in patients with VRD, although very rare, include plexiform neurofibromas, angiosarcoma, and malignant schwannoma.2 Primary hepatic somatostatinoma, with or even without VRD, is very rare and has been reported only by Ohwada et al.3 It is known that somatostatin-containing cells are present in the intrahepatic biliary tree.4 Thus, intrahepatic tumors have arisen from these endocrine cells, possible affected by a genetic disorder present in patients with VRD.