ADENOCARCINOID OF THE LIVER ARISING WITHIN AN AREA OF HAMARTOMA WITH PREDOMINANT BILE-DUCT COMPONENT

Malignant transformation in bile duct hamartomas has been previously r eported in very rare instances. Here, we describe a unique case of a n euroendocrine tumor of the liver arising within an area of unusually l arge hamartoma with predominant bile duct component, hitherto unreport ed and distinct from the conventional von Meyenburg complex. The tumor was apparently secreting gastrin and chromogranin, with associated ga strinoma syndrome over several years. The histologic picture was remin iscent of a moderately differentiated adenocarcinoid, with positive mu cin staining in a signet ring pattern. Tumor cells showed positive sta ining for neuron-specific enolase, chromogranin A, gastrin, and seroto nin. Staining for pancreatic hormone peptides was negative. Resection of the tumor was apparently curative, with complete resolution of the patient's symptoms.