Pheochromocytomas and paragangliomas in von Hippel-Lindau disease: A role for laparoscopic and cortical-sparing surgery

Mercedeh Baghai, Geoffrey B. Thompson, William F. Young, Clive S. Grant, Virginia V. Michels, Jon A. Van Heerden

Research output: Contribution to journalArticlepeer-review

67 Scopus citations


Hypothesis: Von Hippel-Lindau disease (VHL) is an autosomal-dominant disorder characterized by benign and malignant tumors involving the central nervous system, kidneys, pancreas, adrenal glands, and paraganglia. Appropriate management of pheochromocytomas and paragangliomas in VHL is evolving as we better understand the genetics and natural course of the disease and master advanced surgical techniques for adrenalectomy. Design: Retrospective chart review. Setting: Tertiary referral center. Patients: A total of 109 patients identified at the Mayo Clinic, Rochester, Minn, with VHL (60 males and 49 females) between January 1, 1975, and June 30, 2000. Seventeen patients (16%) had an identifiable adrenal mass and 3 patients had paragangliomas. Follow-up was complete in all but 2 patients. Main Outcome Measures: Clinical presentation, preoperative evaluation, surgical management, and outcome. Results: Three patients with paragangliomas and 13 of 17 patients with adrenal masses underwent surgical resection. Median age at time of diagnosis was 30 years (range, 16-47 years); 8 (40%) were asymptomatic. Frationated urinary catecholamine and metanephrine concentrations were normal in one third of patients. Computed tomographic scanning identified 20 (83%) of 2 tumors. Adrenalectomies were performed as unilateral or bilateral, open or laparoscopic, and, finally, total or cortical-sparing. Seven (50%) of the patients underwent other concurrent abdominal procedures. There were no deaths, with an overall operative morbidity of 2 patients (14%). Only the 2 patients in whom bilateral total adrenalectomies were performed became corticosteroid dependent. No recurrences have been noted to date. Conclusions: A multidisciplinary approach is imperative for proper examination and monitoring of patients with VHL. Evaluation should begin early in life and always before elective surgery and childbirth. All adrenal masses in patients with VHL should be thoroughly evaluated and most should be resected. Early intervention and advanced surgical techniques better allow for corticalsparing and laparoscopic procedures. With low recurrence rates, corticosteroid independence can be maintained for prolonged periods.

Original languageEnglish (US)
Pages (from-to)682-689
Number of pages8
JournalArchives of Surgery
Issue number6
StatePublished - Jun 18 2002

ASJC Scopus subject areas

  • Surgery


Dive into the research topics of 'Pheochromocytomas and paragangliomas in von Hippel-Lindau disease: A role for laparoscopic and cortical-sparing surgery'. Together they form a unique fingerprint.

Cite this