Primary hepatic carcinoid tumor in children

Abstract

Primary carcinoid tumors of the liver are rare, with fewer than 60 cases currently reported in the English literature. We present the evaluation and management of a solid hepatic tumor in a 14-year-old boy. Intraoperative biopsy was indeterminant for malignant potential, and the patient underwent complete resection by left hepatic lobectomy. Final histopathologic evaluation of the mass revealed a carcinoid tumor. Extensive endoscopic and radiologic workup revealed no other primary source. The patient recovered well from surgery and is currently free of disease 32 months after initial resection. Review of the literature suggests that primary hepatic carcinoid tumors are particularly rare in children. As the liver is frequently a site for carcinoid metastasis from the gastrointestinal tract, any patient with a suspected primary hepatic carcinoid tumor must undergo an extensive search for an extrahepatic primary site. These tumors are typically indolent but may metastasize. In addition, medical therapy is of limited benefit in reducing tumor bulk. The mainstay for treatment of primary hepatic carcinoid tumors is surgical resection, and these tumors carry a more favorable prognosis than other primary hepatic malignancies and metastatic carcinoid. Follow-up is long-term, as these tumors can recur many years after initial resection.