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doi:10.1111/jgh.12626
E D U C AT I O N A N D I M A G I N G
Hepatobiliary and pancreatic: Coexisting cancers: Hepatic neuroendocrine carcinomas with gall bladder adenocarcinoma
Figure 1
Figure 2
A woman, aged 67, was investigated because of intermittent pain in the epigastrium for one month. There was no relevant past history and no abnormalities were detected on physical examination. Routine blood tests, including liver function tests, were within the reference range but she had a mild elevation of CA 19-9 (55 U/ml). Serological tests for hepatitis B and C were negative. A contrast- enhanced computed tomography (CT) scan of the upper abdomen showed irregular thickening of the gallbladder wall (Figure 1, left). She also had low-density tumors with ring enhancement in segments 4 and 5 of the liver (Figure 1, right). These lesions had a maximum diameter of 5.0 cm. She was thought to have gallbladder adenocarcinoma with liver metastases and was treated by an extended resection that included gallbladder, mid-segments of the liver and regional lymph nodes. The gallbladder wall was diffusely thickened while the hepatic tumors were greyishwhite, hard and well-circumscribed. Histological evaluation of the galbladder specimen revealed adenocarcinoma while the hepatic tumors had sheets of neoplastic cells with round to oval hyperchromatic nuclei and eosinophilic cytoplasm (Figure 2, left). Immunohistochemical studies revealed strong expression for Syn (Figure 2, right) and CD6 supporting the diagnosis of an hepatic neuroendocrine tumor (neuroendocrine carcinoma).
Under most circumstances, liver tumors associated with known or strongly suspected primary tumors are liver metastases. Occasionally, however, known or suspected primary tumors may coexist with a second tumor causing liver metastasis or with a primary liver tumor. In the above case, a neuroendocrine tumor involving the liver coexisted with a primary adenocarcinoma of the gallbladder. The possibility of the coexistence of two tumors might have been suspected because of ring enhancement of the liver tumors on a contrast-enhanced CT scan. This radiological finding is common in patients with neuroendocrine tumors but uncommon in patients with typical liver metastases. Other investigations that might have differentiated the tumors include magnetic resonance imaging, somatostatin- receptor scintigraphy and positron emission tomography (PET)-CT using a variety of tracers to identify neuroendocrine tumours. In the above case, a post-operative PET-CT failed to identify any extrahepatic tumors, thus raising the possibility of a primary neuroendocrine tumor of the liver. Primary hepatic tumors of this type appear to be rare with fewer than 150 cases in the medical literature.
Journal of Gastroenterology and Hepatology 29 (2014) 1343 © 2014 Journal of Gastroenterology and Hepatology Foundation and Wiley Publishing Asia Pty Ltd
Contributed by J Lu, X-Z Xiong & N-S Cheng Department of Bile Duct Surgery, West China Hospital, Sichuan University, Chengdu, China
1343
doi:10.1111/jgh.12626
E D U C AT I O N A N D I M A G I N G
Hepatobiliary and pancreatic: Coexisting cancers: Hepatic neuroendocrine carcinomas with gall bladder adenocarcinoma
Figure 1
Figure 2
A woman, aged 67, was investigated because of intermittent pain in the epigastrium for one month. There was no relevant past history and no abnormalities were detected on physical examination. Routine blood tests, including liver function tests, were within the reference range but she had a mild elevation of CA 19-9 (55 U/ml). Serological tests for hepatitis B and C were negative. A contrast- enhanced computed tomography (CT) scan of the upper abdomen showed irregular thickening of the gallbladder wall (Figure 1, left). She also had low-density tumors with ring enhancement in segments 4 and 5 of the liver (Figure 1, right). These lesions had a maximum diameter of 5.0 cm. She was thought to have gallbladder adenocarcinoma with liver metastases and was treated by an extended resection that included gallbladder, mid-segments of the liver and regional lymph nodes. The gallbladder wall was diffusely thickened while the hepatic tumors were greyishwhite, hard and well-circumscribed. Histological evaluation of the galbladder specimen revealed adenocarcinoma while the hepatic tumors had sheets of neoplastic cells with round to oval hyperchromatic nuclei and eosinophilic cytoplasm (Figure 2, left). Immunohistochemical studies revealed strong expression for Syn (Figure 2, right) and CD6 supporting the diagnosis of an hepatic neuroendocrine tumor (neuroendocrine carcinoma).
Under most circumstances, liver tumors associated with known or strongly suspected primary tumors are liver metastases. Occasionally, however, known or suspected primary tumors may coexist with a second tumor causing liver metastasis or with a primary liver tumor. In the above case, a neuroendocrine tumor involving the liver coexisted with a primary adenocarcinoma of the gallbladder. The possibility of the coexistence of two tumors might have been suspected because of ring enhancement of the liver tumors on a contrast-enhanced CT scan. This radiological finding is common in patients with neuroendocrine tumors but uncommon in patients with typical liver metastases. Other investigations that might have differentiated the tumors include magnetic resonance imaging, somatostatin- receptor scintigraphy and positron emission tomography (PET)-CT using a variety of tracers to identify neuroendocrine tumours. In the above case, a post-operative PET-CT failed to identify any extrahepatic tumors, thus raising the possibility of a primary neuroendocrine tumor of the liver. Primary hepatic tumors of this type appear to be rare with fewer than 150 cases in the medical literature.
Journal of Gastroenterology and Hepatology 29 (2014) 1343 © 2014 Journal of Gastroenterology and Hepatology Foundation and Wiley Publishing Asia Pty Ltd
Contributed by J Lu, X-Z Xiong & N-S Cheng Department of Bile Duct Surgery, West China Hospital, Sichuan University, Chengdu, China
1343
