Single-stage laparoscopic adrenalectomy for pheochromocytoma and enucleation of a pancreatic neuroendocrine tumor in Von Hippel-Lindau disease: A case report

Abstract. Von Hippel-Lindau (VHL) disease is an inherited syndrome with autosomal-dominant transmission, characterized by central nervous system and retinal hemangioblastomas, visceral cysts and tumors. Optimal surgical treatment, including its timing, remains a controversial topic. The present study reports the case of a 67-year-old female patient with adrenal and pancreatic manifestations of VHL. A laparoscopic cortex-sparing left adrenalectomy for a 4-cm pheochromocytoma and pancreatic enucleation for pancreatic polypeptidoma of the pancreas tail were performed during the same operative procedure. The total operative time was 240 min. There were no operative complications, and the surgery was completed laparoscopically with minimal blood loss. A prolonged hospital stay was necessary to treat a grade C postoperative pancreatic fistula. The histopathological result was an adrenal pheochromocytoma and a well-differentiated neuroendocrine tumor, secreting pancreatic polypeptides. In conclusion, organ-sparing laparoscopic surgery is an important option for treating simultaneous lesions in several abdominal solid viscera, such as VHL disease, and the present case study represents, to the best of the authors' knowledge, the first report of single-stage laparoscopic adrenalectomy and pancreatic tumor enucleation.