Laparoscopic Left Hepatectomy for Slowly Progressing Primary Hepatic Carcinosarcoma Associated With Von Hippel-Lindau Disease.

Von Hippel-Lindau disease (VHL) is a hereditary multiorgan cancer syndrome; however, liver tumors are rarely encountered. Herein, we present a case of slow-growing primary hepatic carcinosarcoma associated with VHL that was curatively treated laparoscopically. A 37-year-old woman has regularly visited the neurosurgery department of our hospital with VHL-associated cerebellar and medullary hemangioblastomas. Computed tomography performed 6 years earlier revealed a 20-mm solitary hypervascular tumor with cystic components in liver segment 3. The tumor size was stable for a while but had been steadily growing over the past few years and was 55 mm at the time of referral to our department. Because increased uptake on 18F-FDG-PET suggested malignancy, such as hemangioblastoma or neuroendocrine tumor, laparoscopic left hepatectomy was performed without percutaneous biopsy, considering the potential risk of tumor cell seeding. Histopathological examination revealed carcinosarcoma, and no recurrence was noted at the 1-year follow-up.