V11-02: Bilateral adrenalectomy and multiple tumorectomy in Von Hippel–Lindau desease

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INTRODUCTION

The von Hippel - Lindau disease is an autosomal dominant disease that courses with multifocal renal cell carcinoma in 65% of the affected individuals. The risk of developing pheochromocytomas in these patients reaches 20%.

METHODS

We present the case of a 50 year old man, obese with past history of hypertension; diagnosed by MRI performed prior to bariatric surgery, with a bilateral adrenal tumor (Left 7x7cm and right 5x4cm) and a double right renal tumor (posterior middle valve 3x2cm and lower pole 1.5cm). Given the high plasma and urinary levels of norepinephrine and the positive metaiodobenzylguanidine scintigraphy (MYBG), diagnosis of bilateral pheochromocytoma was confirmed. Simultaneous left nephron sparing surgery and adrenalectomy by retroperitoneoscopy approach after trasnperitoneal left adrenalectomy were proposed.

RESULTS

Following an alpha and beta-adrenergic blockade, a left transperitoneal laparoscopic adrenalectomy was performed without incident. (120 minutes - Estimated blood loss: 150 cc). One month later a retroperitoneal right adrenalectomy with off-clamp tumorectomy of the two right kidney tumors was undertaken. The pathology showed bilateral pheochromocytoma; while the right kidney tumorectomy report clear cell carcinoma, Fuhrman I, pT1a, with negative surgical margin in both tumours. The genetic studies confirmed VHL disease. The patient remained normotensive without medical treatment.

CONCLUSION

Simultaneous laparoscopic nephron sparing surgery and adrenalectomy is a suitable option for patients with Von Hippel-Lindau Disease affected by synchronic pheocromocytomas and renal tumors. _x000D_ Trans and Retroperitoneal approaches are both safe and effective procedures presenting low morbidity, and may both be used as an alternative, depending on the position of the renal tumors.

Funding: None