Robot-assisted cortical-sparing adrenalectomy in a patient with Von Hippel-Lindau disease and bilateral pheochromocytomas separated by 9 years

Jamii St. Julien, Douglas Ball, Richard Schulick

Research output: Contribution to journalArticlepeer-review

30 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)473-477
Number of pages5
JournalJournal of Laparoendoscopic and Advanced Surgical Techniques
Volume16
Issue number5
DOIs
StatePublished - Oct 1 2006

ASJC Scopus subject areas

  • Surgery

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