Hepatic resection and transplantation for primary carcinoid tumors of the liver

Abstract

Objective: To discuss the diagnosis and management of primary carcinoid tumors of the liver in light of our experience and a literature review.

Summary background data: Carcinoid tumors of the liver are rare and pose a diagnostic and management dilemma. This series is the largest reported and the only one to include liver transplantation as a treatment option.

Methods: Between March 1994 and May 2002, we treated 8 patients (4 male, 4 female) with primary hepatic carcinoid tumors. Carcinoid syndrome complicated only 1 of the cases. Treatment was by liver resection in 6 patients and orthotopic liver transplantation in 2.

Results: The diagnosis was confirmed histologically with light microscopy and immunohistochemistry in the absence of an alternative primary site. Six patients remain alive and disease free after follow-up of more than 3 years: 39, 43, 45, 50, 50, and 95 months. Two patients are recently postoperative.

Conclusions: Active exclusion of an extrahepatic primary site is essential for the diagnosis of primary carcinoid of the liver. The mainstay of treatment should be liver resection, although liver transplantation may be considered in patients with widespread hepatic involvement. A radical surgical approach is warranted as this disease carries a better prognosis than for other primary hepatic tumors and for secondary hepatic carcinoids.

FIGURE 1. Flow diagram representing the suggested diagnostic pathway for primary hepatic carcinoid tumor.

FIGURE 2. A somatostatin scan shows an intense uptake in the region of a large tumor in the left liver (patient 2).

FIGURE 3. Coronal (A) and transverse (B) section magnetic resonance imaging demonstrates a 12 × 10-cm tumor enveloping both left and right branches of the portal vein, hepatic artery, and hepatic ducts (patient 4), for which an orthotopic liver transplant was performed.

FIGURE 4. Transverse (A) and coronal oblique (B) section magnetic resonance imaging demonstrating a 15-cm diameter tumor, primarily involving the right liver, but with significant extension into the left liver. There is evident biliary obstruction (patient 7). Hepatic resection was possible by a modified right hemihepatectomy technique.

FIGURE 5. A: Photomicrograph showing typical morphology of low-grade endocrine tumor, showing trabecular arrangement of cells with regular round nuclei, peripheral pallisading, and highly vascular stroma. (patient 2; original magnification ×200). B: Photomicrograph of an area of tumor in which the cells show larger, pleomorphic nuclei and distinctive perinuclear eosinophilic inclusions (patient 3; original magnification ×400).