Primary endodermal sinus (yolk sac) tumor of the falciform ligament

Extragonadal yolk sac tumors (YSTs) are uncommon and YSTs of the liver are exceedingly rare, with only three reported cases in the literature. A case is described of primary YST of the falciform ligament extending into the left lobe of the liver in a 14-month-old boy. This is the first reported case of primary YST arising within the falciform ligament. The patient underwent an exploratory laparotomy after presenting with hemoperitoneum. An extremely friable and necrotic tumor was found extending from the falciform ligament into the liver. The tumor was debulked and the patient received 5 months of chemotherapy employing a modified Einhorn regimen. After a partial response to chemotherapy the patient had a second-look laparotomy, at which time a left hepatic lobectomy and en bloc resection of the falciform ligament was performed in order to remove residual tumor. At the present time the patient has no signs of metastases and is alive and well 2 years after his presentation.