Primary hepatic neuroendocrine tumor with multiple liver metastases: A case report with review of the literature

Abstract

We herein present a case involving a 41-year-old woman in whom ultrasound examination revealed multiple liver hemangiomas more than 3 years ago. Follow-up ultrasound examination revealed that the masses had significantly increased; the largest was located in the right lobe (about 8.2 cm × 7.4 cm × 6.0 cm). Abdominal multidetector computed tomography revealed multiple well-circumscribed, heterogeneous, hypodense masses (largest, 6.4 cm × 6.3 cm × 5.0 cm) with significant contrast enhancement during the arterial and portal phases and with contrast wash-out and peripheral enhancement during the delayed phases. Magnetic resonance images demonstrated multiple well-circumscribed, heterogeneous, hypointense hepatic masses with significant contrast enhancement (largest, 6.4 cm × 6.5 cm × 5.1 cm); multiple enlarged porta lymph nodes; and multiple slightly enlarged retroperitoneal lymph nodes. Histological and immunohistochemical examination of the right mass biopsy specimen suggested a malignant neoplasm that had originated from a neuroendocrine cell type (grade 2 well-differentiated neuroendocrine carcinoma). After performing a systemic examination to exclude metastasis from an extrahepatic primary site, we considered that the masses had arisen from a primary hepatic neuroendocrine tumor with multiple liver metastases. The patient underwent transcatheter arterial chemoembolization using a combination of oxaliplatin (150 mg) mixed with one bottle of gelatin sponge particles (560-710 μm) and lipiodol (6 mL). Primary neuroendocrine tumors of the liver are extremely rare. This case is interesting because of the rarity of this neoplasm and previous misdiagnosis as multiple liver hemangiomas. Previously reported cases in the literature are also reviewed.

Case Report of Contrast-Enhanced Ultrasound Features of Primary Hepatic Neuroendocrine Tumor

“… 14 , 15 The most common symptoms found in this study were right upper quadrant palpable mass and abdominal pain in both our cases and previous reports. 16 No patient with carcinoid syndrome was found, which agreed with a report that carcinoid syndrome was usually caused by metastatic neuroendocrine tumors. 17 …”

Comparative Clinical Analysis of Gastroenteropancreatic Neuroendocrine Carcinomas with Liver Metastasis and Primary Hepatic Neuroendocrine Carcinomas

“…Diagnosis of PHNECs is considered challenging in view of the common initial presentation of GEP NECs as metastatic liver lesion. Hepatic neuroendocrine cell may originate from intrahepatic bile duct epithelial cells, heterotopic pancreatic cells, or adrenal tissue [ 4 , 5 ]. PHNECs can secrete a variety of polypeptides and biogenic amines, including 5-HT, pancreatic polypeptides, gastrin, prostaglandin, and calcitonin.…”

Misdiagnosis of Primary Hepatic Neuroendocrine Carcinoma: A Case Report and Literature Review

“…Highly differentiated NETs of grades G1 and G2 are considered benign, and poorly differentiated NETs of grade G3 are named neuroendocrine carcinoma. Studies in the literature have reported that 45% of NETs originate from the small intestine, 20% from the rectum, 17% from the appendix, 11% from the colon, and 7% from the stomach [4] . The liver is the most common metastatic organ of NETs, but PHNEC is rare, accounting for only 0.3-4.0% of all NETs [1] .…”

“…Primary hepatic neuroendocrine tumors (PHNETs) frequently stem from intrahepatic bile duct epithelial cells, adrenal tissue, or ectopic pancreatic cells [5] . PHNEC usually occurs in the right lobe of the liver.…”