title: Robot-assisted cortical-sparing adrenalectomy in a patient with Von Hippel-Lindau disease and bilateral pheochromocytomas separated by 9 years.
abstract: Von Hippel-Lindau disease is a heritable syndrome that confers an increased risk of developing various benign and malignant tumors to those with a germline mutation of the tumor suppressor gene. We present a case of a male patient who initially presented at age 9 with headaches, fevers, and fatigue. He was found to have a left pheochromocytoma which was successfully managed with open total adrenalectomy. He presented again at age 18 with a second pheochromocytoma in the right adrenal gland. DNA analysis revealed a de novo Val84Leu mutation in the Von Hippel-Lindau gene, not seen in either parent. The challenge presented was that of balancing the obvious benefits of cortical- sparing adrenalectomy with the risk of tumor recurrence in spared tissue. Ultimately, management consisted of a robot-assisted laparoscopic partial right adrenalectomy with successful preservation of adrenocortical function.
authors.affiliation: Department of Surgery, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21231, USA.
authors.forename: Jamii
authors.initials: J
authors.lastname: St Julien
authors.forename: Douglas
authors.initials: D
authors.lastname: Ball
authors.forename: Richard
authors.initials: R
authors.lastname: Schulick
mesh_terms: D000293:Adolescent; D000302:Adrenal Cortex; D000310:Adrenal Gland Neoplasms / Q000209:etiology* / Q000601:surgery*; D000315:Adrenalectomy / Q000379:methods*; D002648:Child; D006801:Humans; D008297:Male; D016609:Neoplasms, Second Primary / Q000209:etiology* / Q000601:surgery*; D010673:Pheochromocytoma / Q000209:etiology* / Q000601:surgery*; D012371:Robotics; D013997:Time Factors; D006623:von Hippel-Lindau Disease / Q000150:complications*