Primary hepatic carcinoid: a case report and literature review

Abstract

Carcinoids are tumors derived from neuroendocrine cells and often produce functional peptide hormones. Approximately 54.5% arise in the gastrointestinal tract and frequently metastasize to the liver. Primary hepatic carcinoid tumors (PHCT) are extremely rare; only 95 cases have been reported. A 65-year-old man came to our attention due to occasional ultrasound findings in absence of clinical manifestations. His previous medical history, since 2003, included an echotomography of the dishomogeneous parenchymal area but no focal lesions. A computed tomography scan performed in 2005 showed an enhanced pseudonodular-like lesion of about 2 cm. Cholangio-magnetic resonance imaging identified the lesion as a possible cholangiocarcinoma. No positive findings were obtained with positron emission tomography. Histology suggested a secondary localization in the liver caused by a low-grade malignant neuroendocrine tumor. Immunohistochemistry was positive for anti chromogranin antibodies, Ki67 antibodies and synaptophysin. Octreoscan scintigraphy indicated intense activity in the lesion. Endoscopic investigations were performed to exclude the presence of extrahepatic neoplasms. Diagnosis of PHCT was established. The patient underwent left hepatectomy, followed by hormone therapy with sandostatine LAR. Two months after surgery he had a lymph nodal relapse along the celiac trunk and caudate lobe, which was histologically confirmed. The postoperative clinical course was uneventful, with a negative follow-up for hematochemical, clinical and radiological investigations at 18 mo post-surgery. Diagnosis of PHCT is based principally on the histopathological confirmation of a carcinoid tumor and the exclusion of a non-hepatic primary tumor. Surgical resection is the recommended primary treatment for PHCT. Recurrence rate and survival rate in patients treated with resection were 18% and 74%, respectively.

Figure 1

Abdominal CT scan (July 2005) showing a low-density pseudonodular area (arrow) of 2 cm with biliary dilatation.

Figure 2

Abdominal MRI (August 2005) showing a pseudonodular mass (arrow) measuring about 5 cm × 3 cm of the hepatic segments II-III. At the bottom of the lesion, the biliary tree appears dilated.

Figure 3

111 In-pentetreotide (octreotide) scintigraphy. A: Before hepatectomy: marked hyperactivity of the lesion is observed in the left hepatic lobe and interaortal adenopathy is observed; B: After hepatectomy; abnormal fluid accumulation of ligand in the epigastric region and dishomogeneous hepatic distribution.

Figure 4

Abdominal CT scan. Results of left hepatectomy show an extended nodular mass measuring about 2.5 cm × 1.5 cm arranged on the back part of the caudate lobe, indicating a lymph node localization of disease.

Figure 5

Histological and immunohistochemistry. Proliferation of average sized monomorphic epithelial cells collected in strings and glandular structures (A). Positive immunohistochemistry staining for CgA (B) and NSE (C).