Laparoscopic Adrenal-sparing Approach for Children with Bilateral Pheochromocytoma in Von Hippel-Lindau Disease
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In brief
In brief
Retrospective case series of 8 pediatric VHL patients demonstrates that laparoscopic partial adrenalectomy is a safe, effective approach for bilateral pheochromocytomas, preserving adrenal function without steroid replacement. With 14 of 16 procedures completed as partial adrenalectomies and minimal recurrence over 5-year follow-up, this technique should be considered first-line when technically feasible.
Written by the GCMD Library team from the article.
Introduction
Von Hippel-Lindau disease (VHL) is a rare cause of hereditary bilateral Pheochromocytomas (PHEO). Traditionally, treatment has been total adrenalectomy due to a lifetime risk of developing new tumors. Limited data exists on the surgical management of bilateral PHEO in children with VHL. We reviewed our experience with laparoscopic partial adrenalectomy for bilateral PHEO.
Methods
A retrospective review was performed of patients undergoing adrenalectomy for PHEO in children with VHL from 2004-2019.
Results
Eight children with VHL diagnosed with bilateral PHEO underwent 16 adrenalectomies (10 synchronous, 5 metachronous, 1 for recurrence). Median age at diagnosis was 13 [range 8-17] years with a median tumor size of 2.3 [range 0.5-7.7] cm. Of 16 adrenalectomies, all were performed laparoscopically, 14 were partial adrenalectomies; 2 patients required a contralateral total adrenalectomy due to size and diffuse multinodularity. There were no postoperative complications. No patients required corticosteroid replacement at the end of the study period. Two patients had new ipsilateral tumors identified after a median follow up of 5 [range 4-6] years with one undergoing repeat partial adrenalectomy. There were no mortalities in the study period.
Conclusion
Partial adrenalectomy for bilateral PHEO in patients with VHL is safe and does not compromise outcomes. When technically feasible, laparoscopic partial adrenalectomy should be considered as a primary surgical approach for children with VHL.
Level of Evidence
Level IV - Case series with no comparison group