Primary Hepatic Neuroendocrine Carcinoma

Primary Hepatic Neuroendocrine Carcinoma with Metastasis to the Mesentery: A Case Report

“…Based on the abovementioned clinical-imaging findings, it is difficult to distinguish hepatic NECs from other hepatic carcinomas, such as HCC and cholangiocarcinoma. Consequently, pathological examination through the performance of a preoperative liver tumor biopsy is essential for diagnosis [9‒52]. In our case, the patient underwent a biopsy of the liver lesion for histological diagnosis, since the characteristics in the imaging studies were not sufficient to make the diagnosis conclusively.…”

“…Presentation: there is no staging for NENs of the liver, so they are classified according to the location of the disease; localized (located within the primary organ, in this case the liver), locally advanced or regional (nodal disease), and advanced or metastatic (disease in distant nodes and organs) [9, 11‒52]. The size of the tumor is 1.5–27 cm, presenting as single (76.3%) and multiple in 23.7% of cases, located mainly in the right lobe in 48.4% of cases, and as bilobular in 18.5%.…”

“…The size of the tumor is 1.5–27 cm, presenting as single (76.3%) and multiple in 23.7% of cases, located mainly in the right lobe in 48.4% of cases, and as bilobular in 18.5%. Knox et al [54] reported extrahepatic involvement in 18.6% of cases, such as bone (60%), lymph node (60%), and lung (40%) [9‒55]. The form of tumor presentation was two liver tumors that occupied both lobes of approximately 20 cm with metastasis to the mesentery.…”

“…This number includes both primary hepatic NETs and NECs. Primary hepatic neuroendocrine carcinomas (PHNECs) are extremely rare, with only about 90 cases having been reported in the English-language literature [10‒50] (Table 1).…”

“…PHNECs are a diagnosis of exclusion since they are far less common than hepatic metastasis of NECs hence an extrahepatic primary NEC must always be excluded first through studies including endoscopy and colonoscopy (to rule out GI origin), CT, MRI, and somatostatin PET-CT (to determine the extent of the disease and its origin), as was done in our patient [51, 52]. Since the radiological and laboratory findings of PHNECs are not specific, definitive diagnosis of PHNECs needs pathologic evaluation of a surgically resected specimen [9‒53].…”