Primary Hepatic Neuroendocrine Carcinoma Research Articles

A case of primary hepatic neuroendocrine carcinoma diagnosed by the needle biopsy

A case of primary hepatic neuroendocrine carcinoma diagnosed by the needle biopsy

Hyperammonemic encephalopathy in a patient with primary hepatic neuroendocrine carcinoma

A 53-year-old male patient was admitted to our hospital with abdominal pain in the right upper quadrant. There was no change in laboratory investigations other than a slight increase in serum levels of alkaline phosphatase (ALP), alanine aminotransferase (ALT), and gamma glutamyl transferase (GGT). Computed tomography (CT) of the abdomen showed multiple hepatic nodular lesions in the liver. Tru-cut biopsy of the lesions was reported as well-differentiated neuroendocrine carcinoma. The patient received sandostatin treatment. After a few days, the patient was hospitalized in the intensive care unit with disturbance of consciousness and clinical features suggestive of encephalopathy. Serum ammonia level was found highly elevated. After the treatment with L-ornithine-L-aspartate, a remarkable improvement in the level of patient's sensorium occurred as well as a reduction in serum ammonia level within a few days. Transarterial chemoembolization (TACE) was performed one week later. The patient's condition began to worsen along with increase in serum ammonia level and he died because of hyperammonemic encephalopathy. There are case reports of hyperammonemia with some malignancies such as multiple myeloma, plasma cell leukemia, and leiomyosarcoma, or in some patients who have received chemotherapy. This case may suggest an association between hyperammonemia and neuroendocrine tumors.

Primary hepatic neuroendocrine carcinoma in a baboon (Papiosp.)

Primary neuroendocrine carcinomas of the liver have rarely been reported in humans and domestic animals, but not in non-human primates. We describe the morphologic and immunohistochemical features of a primary hepatic neuroendocrine carcinoma found in a 29-year-old female baboon. The neoplasm was characterized by multiple solid nodules that were multifocally distributed in the liver. Metastases were not observed. Histologically, the neoplasm was composed of cords and nests of epithelial cells arranged in a neuroendocrine pattern, occasionally forming glandular and rosette-like structures. On immunohistochemical evaluation, the neoplastic cells were immunopositive for pancytokeratin, chromogranin A, neuron-specific endolase, and synaptophysin and were negative for vimentin, S100 protein, glucagon, and insulin.

Imaging appearance of primary hepatic neuroendocrine carcinomas

Objective To investigate the CT and MRI findings of primary hepatic neuroendocrine carcinomas.Methods Imaging findings of 6 patients with pathologically confirmed hepatic neuroendocrine carcinomas were retrospectively analyzed.Four patients underwent plain CT and contrast enhanced CT examinations.The other 2 patients underwent plain MRI and contrast enhanced MRI.Results One out of the 6 cases manifested multicentric tumor which appeared as one large tumor surrounded with multiple small nodules peripherally,and the tumors of the other 5 cases were solitary.On plain CT images,all lesions were well-defined and hypoattenuating with central areas of even lower density except punctuate calcifications at tumor periphery in 1 case.On pre-contrast MRI,the cases manifested heterogeneous low signal intensity on T1 wI and slightly high intensity on T2 WI.On dynamic contrast enhanced CT and MRI,Tumors manifested slight to mild enhancement in arterial phase,slight enhancement in portal venous phase or delayed phase with no enhancement in the center,accompanied by dislocation of surrounding vessels.There Was no lymphadenopathy in peritoneal cavity and retroperitoniurm. Conclusion CT and MRI scanning can demonstrate specific features of primary hepatic neuroendocrine carcinoma,which may help the diagnosis and differential diagnosis of the tumor. Key words: Liver neoplasms; Carcinoma,neuroendoerine; Tomography,X-ray computed; Magnetic resonance imaging

Neuroendocrine carcinoma of the liver associated with dermatomyositis: Autopsy case and review of the literature

Reported herein is an autopsy case of primary hepatic neuroendocrine carcinoma associated with dermatomyositis. A 71-year-old Japanese man, who was diagnosed with dermatomyositis 5 months before death, had multiple tumors within a non-cirrhotic liver. Histopathologically, the tumors were composed of small- and medium-sized round cells with clear cytoplasm arranged in nests, sheets or rosettes. Immunohistochemically, the tumor cells were positive for chromogranin A, neuron-specific enolase and CD56 and were negative for synaptophysin. This tumor was diagnosed as a primary hepatic neuroendocrine carcinoma with metastasis to the lung, gallbladder and lymph nodes around the pancreas and aorta; no primary lesions were detected in any other organ. The tumor cells were also positive for cytokeratin 7, cytokeratin 19 and epithelial membrane antigen but were negative for anti-hepatocyte antibody and AFP. These findings suggest that the tumor originated in intrahepatic bile duct epithelium. Various cancers have been reported in patients with dermatomyositis, but only seven cases of dermatomyositis associated with primary liver cancer have been reported. To the best of the authors' knowledge, this is the first report of dermatomyositis associated with primary hepatic neuroendocrine carcinoma.

Primary hepatic neuroendocrine carcinoma: an underdiagnosed entity

Primary hepatic neuroendocrine tumors are rare. We report a case of a primary neuroendocrine carcinoma of the liver with a review of the literature. In earlier reports, all of these tumors were named hepatic carcinoids. We now recognize that they belong to a heterogeneous group of neoplasms, with microscopic appearance not necessarily predictive of biologic behavior. To our knowledge, only 55 cases have been reported in the English literature. This entity has probably been underreported because of the assumption that it represents a metastatic lesion. A search for an occult primary neuroendocrine tumor outside the liver must always be pursued.

Primary hepatic neuroendocrine carcinoma in a child morphologic, immunocytochemical, and molecular biologic studies

Primary neuroendocrine tumor of the liver is uncommon, and virtually all reported patients with the tumor have been adults. Most of the tumors were carcinoids. The authors report an 8-year-old girl with primary hepatic neuroendocrine carcinoma. Histologic, ultrastructural, and immunocytochemical studies and Southern blot analysis of tumor N-myc DNA were performed in the patient. Histologically, the tumor revealed a characteristic organoid pattern with a spectrum of differentiation varying from well-differentiated carcinoid-like areas to poorly differentiated pleomorphic areas. Ultrastructural features included neurosecretory granules and interdigitating cytoplasmic extensions. The tumor cells showed immunoreactivity to neuron-specific enolase (NSE), S-100 protein, chromogranin, and synaptophysin. No evidence of amplification of tumor N-myc DNA was present. However, the molecular weight of the tumor N-myc DNA (1.8 kb) was significantly lower than the normal control (from normal liver tissue) (2.0 kb). This report documents the occurrence of primary hepatic neuroendocrine carcinoma in a child. Thorough studies and complete clinical evaluation are essential to the establishment of diagnosis. The result of N-myc DNA analysis probably is attributable to deletion of part of the tumor N-myc gene. The clinical implication of this finding is unknown, and additional investigation is warranted.