21 years of evolution of primary hepatic neuroendocrine neoplasm in a patient with primary sclerosing cholangitis: A case report

INTRODUCTION AND IMPORTANCE: Primary hepatic neuroendocrine neoplasms(PHNEN) are among the rarest primitive neuroendocrine neoplasms. Main prognostic factor is histological. We report an unusual case of a PHNEN with 21 years of evolution in the setting of a primary sclerosing cholangitis(PSC). CASE PRESENTATION: A 40 year old man presented in 2001 with clinical signs of obstructive jaundice. CT-scan and MRI showed a 4 cm large hypervascular proximal hepatic mass evoking hepatocellular carcinoma(HCC) or cholangiocarcinoma. Exploratory laparotomy found an aspect of advanced chronic liver disease of the left lobe. Extemporaneous biopsy of a suspicious nodule showed signs of cholangitis. Left lobectomy was performed and postoperatively the patient received ursodeoxycholic-acid and biliary stenting. After 11 years of follow-up, jaundice reappeared with a stable hepatic lesion.A percutaneous liver biopsy was done. Pathology showed a G1 neuroendocrine tumor. Endoscopy, imagery and Octreoscan were normal, supporting the diagnosis of PHNEN. PSC was diagnosed on tumor-free parenchyma. The patient is on liver transplantation waiting list. CLINICAL DISCUSSION: PHNENs are exceptional. Pathology findings, endoscopy and imagery are necessary to rule out an extra hepatic NEN with liver metastasis. While G1 NEN are known for their slow evolution, this 21 year latency is extremely rare. The presence of PSC adds to the complexity of our case. Surgical resection is recommended when possible. CONCLUSION: This case showcases the extreme latency of some PHNEN as well as possible overlap with PSC. Surgery is the most recognized treatment. Liver transplantation seems to be necessary for us, as the rest of the liver shows signs of PSC.