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Bilateral Pheochromocytoma Afer Kidney Transplantation Valeria de Miguel, MD 1 Andrea Paissan, MD1 Gabriela Scheinfeld, MD1 Francisco Umaña Riveros, MD1 Patricio Garcia Marchiñena, MD2

Alberto Jurado, MD2 José Alfie, MD3 Mariana Isola, MD4 Jorge Ferraris, MD5 Fainstein Day Patricia, MD PhD1

1 Endocrinology Unit, 2 Urology Unit, 3 Internal Medicine Unit, 3 Pathology Unit , 5Nephrology Pediatric Unit, Hospital Italiano de Buenos Aires. Argentina

Nothing to disclose: VDM, AP, GS, FUR, PGM, AJ, JA, MI, JF, PFD.

Background

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The prevalence of hypertension is very high in kidney trasplantation patients and secondary causes should be excluded. Neurofibromatosis type 1 (NF1) is an autosomal dominat genetic disease with a prevalence of 1/3000. Pheochromocytoma (PHEO) occurs in about 5% of patients with NF1 mutations.

Clinical Case

We present a 25-year-old man with a history of NF1 and end-stage renal disease due to renal dysplasia at birth who underwent a second kidney transplantation in 2010. Inmunosupresive therapy: Deltisone, Micofenolate and Tacrolimus. He had a family history of NF1: Mother, sister, grandmother and aunt. He maintained controlled blood pressure and normal kidney function until 2014: His blood pressure readings were around 200/110 mmHg despite maximun doses of five anhypertensive drugs including diuretics (Figure 1). He reported episodes sweating, headaches and palpitations. He was admitted to our hospital for resistant hypertension and renal failure. Clinical examination revealed hypertension (210/110 mm Hg), tachycardia (110 BPM), café-au-lait spots, skin neurofibromas and genu varum. Allograft nephropathy and renovascular disease were excluded. CT Scans revealed enlargement of the adrenal glands (Figure 2). The 24- hours urinary metanephrines were highly elevated (Table 1).

Biochemestry Results

Creatinine (0.7-1.6 mg)

1.63

Urinary Total Metanephrines (150-1200 mcg/24 h)

20.055

Urinary Metanephrine (50-400 mcg/24 h)

5380

Urinary Normetanephrine (100-800 mcg/24 h)

14.675

24-hour Ambulatory Blood Pressure Monitoring (ABPM)

ABPM on Alpha-Beta Blockade treatment

Doxazosine 2 mg c/8 h. Nebivolol 5 mg c/24 h Amlodipine 5 mg c/24 h

Nebivolol, Amlodipine Indapamide, Furosemide, espironolactone, Enalapril

2016

2010 2014

CT Scans Follow up:

•  Clinicians should be aware of PHEO diagnosis in genetic predisposition diseases.

•  In the workup of elevated blood pressure, it´s important to considered PHEO as well as other causes of secondary hipertension related to kidney transplant and NF1.

•  PRA is a safe technique with excellent surgical outcomes and low complication rates, that should be specially considered for patients with earlier major abdominal operation or bilateral affection of adrenal glands.

•  Some studies suggested that tumors/cancer occurs more frequently in kidney transplantation patients than in general population.

•  There are only a few cases reported in the literature of PHEO after solid organ transplantation.

Figure 1

Right Adrenal 40x38 mm, Left Adrenal 41 x 36 mm

Figure 2 Blood pressure was controlled with doxazosin, nebivolol and amlodipine (Figure 3). The patient underwent a successful simultaneous bilateral Posterior Retroperitoneoscopic Adrenalectomy (PRA). Pathology report confirmed PHEO diagnosis. He is under prednisone and fludrocortisone therapy. Four months after surgery he is asymptomatic and normotensive without antihypertensive therapy. Renal function and urinary metanephrines were within the normal range

Figure 3

Follow up

Conclusions

References: 1.  Pert and Else. “Genetic Predisposition to Endocrine Tumors: Diagnoses, surveillance

and challenge in care”. Seminars in Oncology (43) 2016. 582 2.  Constantinides V,et al. Systematic review and meta-analysis of retroperitoneoscopic

versus laparoscopic adrenalectomy. Br J Surg 2012, 99: 1639-48 3.  Kasike et al. “Cancer after Kidney Transplantation in United States”. American Journal

of Tranplantation, 2004. 4

131 MIBG Scintigraphy

Table 1