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. 2015:14:85-8.
doi: 10.1016/j.ijscr.2015.07.015. Epub 2015 Jul 28.

Pheochromocytoma as a rare cause of arterial hypertension in a patient with autosomal dominant polycystic kidney disease: A diagnostic and therapeutic dilemma

Amelia J Hessheimer  1 , Oscar Vidal  2 , Mauro Valentini  2 , Juan Carlos García-Valdecasas  2
Affiliations

Pheochromocytoma as a rare cause of arterial hypertension in a patient with autosomal dominant polycystic kidney disease: A diagnostic and therapeutic dilemma

Amelia J Hessheimer et al. Int J Surg Case Rep. 2015.

Abstract

Introduction: Individuals with autosomal dominant polycystic kidney disease (ADPKD) frequently suffer arterial hypertension even prior to significant loss of renal function, a clinical situation that obscures detection of modifiable secondary causes of hypertension.

Presentation of case: A 50-year-old man with ADPKD and polycystic liver and resistant hypertension is diagnosed with a 4-cm right adrenal mass. Cross-sectional MRI is indicative of pheochromocytoma versus adrenocortical carcinoma or metastasis, though there are no typical PCC symptoms and plasma and urine metanephrines are within normal ranges. Since malignancy cannot be excluded, right adrenalectomy is performed. Considering that the enlarged liver poses an obstacle for transperitoneal open and laparoscopic approaches, a retroperitoneoscopic approach is used. Surgical pathology reveals a 4.5-cm pheochromocytoma; the patient no longer requires antihypertensive therapy.

Discussion & conclusion: Pheochromocytoma is a rare but treatable cause of hypertension in ADPKD; given the anatomical complexities these patients present, careful preoperative planning and surgical technique are essential to a favorable outcome.

Keywords: Adrenalectomy; Hypertension; Pheochromocytoma; Polycystic kidney; Polycystic liver; Retroperitoneoscopy.

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Figures

Fig. 1
Fig. 1
Contrast-enhanced cross-sectional imaging of the patient’s abdomen demonstrates a 40 × 27 mm lesion in the right adrenal gland (white arrows). The lesion has heterogeneous contrast uptake and increased signal intensity on T2-weighted MRI (A) and is suggestive of pheochromocytoma versus adrenocortical carcinoma or metastatic lesion of unknown primary. The lesion is situated posterior to the enlarged polycystic liver and inferior vena cava (B) and anterior and superior to the superior pole of the polycystic right kidney (C, D).
See this image and copyright information in PMC

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