Endocrine Abstracts

Introduction: Somatostatin analogues (SSA) have been used as first line treatment to control the symtoms in hormonally active neuroendocrine tumors (NET), for over three decades. Primary hepatic neuroendocrine tumors (PHNETs) are rare neoplasms. Despite increased incidence of PHNETs over time, these tumors remain a rarity. According to the previously reported cases, primary neuroendocrine carcinoma of the liver is usually multicentric, often mimicking liver metastases. The demonstration of the hepatic origin of a neuroendocrine carcinoma is often arduous.

Case presentation: A 59-year-old woman underwent ultrasound examination 3 years ago, that revealed two liver nodular tumors (the largest having approximately 4 cm), with a complex structure. Abdominal IRM images confirmed multiple liver nodular tumors with intense vascularization, suggesting liver metastases. The patient was asymptomatic. In order to determinate the primary site of the tumor, the patient underwent multiple imagistic investigations and endoscopies. Among the blood tests (5-hydroxy indole acetic acid, carcinoembryonic antigen, chromogranin A, serotonin), only chromogranin A presented significantly high values. Further, a liver aspiration biopsy was performed. The histopathological examination result completed with the immunohistochemistry techniques suggested a well differentiated neuroendocrine carcinoma of an intermediary stage, with a proliferation marker ki67 of 4%, most likely a metastasis. For a full diagnosis, Octreoscan (11In-pantreotide scintigraphy) was performed. The whole body scintigraphy at 10 minutes completed with the SPECT/CT at 4 hours established a primary liver site with secondary spleen metastases, along with liver and renal adenopathies. Treatment with somatostatin analogues (SANDOSTATIN LAR 30 mg/month), was commenced, with favourable outcome. After 18 months of treatment, serum chromogranin values are still normal, while the volume of the tumors decreased significantly (by repeated MRI).

Conclusion: Despite that the liver represents the common site for metastases from other gastrointestinal neuroendocrine carcinomas or other tumors, primary hepatic neuroendocrine tumors are rare, but can occur. The diagnosis of primary neuroendocrine tumors of the liver is difficult, as radiological appearances on ultrasound, CT scan and MRI can mimic other pathologies.

Keywords: Neuroendocrine tumors; liver metastases.