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Title: False negative pheochromocytoma and paragangioloma: diagnostic challenges and implications on management.
Authors: Bozin, Michael
Johns-Putra, Lydia
Issue Date: 2015
Conference Name: Royal Australasian College of Surgeons 84th Annual Scientific Congress
Conference Date: May 4-8, 2015
Conference Place: Perth, Western Australia
Abstract: Purpose: Pheochromocytoma and paraganglionomas (PPGL) are rare tumors of catecholamine producing chromaffi n cells. Sensitivity of plasma metanephrines is close to 100% in order to rule out PPGL. We describe a case of a metanephrine ‘false-negative’ extra-adrenal paraganglionoma and its implications on investigation and management. Methodology: We describe a case of a 25-year-old female with an incidental fi nding of a right 5-cm adrenal lesion found on ultrasound with mild hypertension. Investigation with urinary catecholamine and plasma metanephrines were analyzed using tandem mass spectrometry. The patient underwent laparotomy in which the lesion was excised, sent for histological assessment and discussed at our uro-pathology multi-discipline meeting. Results: Biochemical analysis of 24-hour urine catecholamines revealed borderline elevated adrenalin (93 nmol/L). Noradrenaline and dopamine was within normal limits at 466 nmol/L and 2807 nmol/L respectively. Plasma metanephrine and normetanephrine were also within normal limits (142 and 309 pmol/L respectively). Laparotomy revealed a 5-cm para-caval mass adhered to the right renal vein requiring nephrectomy. Histopathology revealed extra-adrenal benign PPGL. Conclusion: Metanephrine-negative PPGL is extremely rare. Causes are small tumors in asymptomatic patients, dopamine secreting tumors, and patients with SDHB/SDHD mutation. Silent secreting lesions, if they can be resected safely and their long-term consequences are the primary concern. Proportionately high dopamine compared to other catecholamines may suggest a dopamine secreting PPGL. We suggest that false negative PPGL should be investigated for 3-methoxytyramine (dopamine metabolite) preoperatively to reduce the risk of missing a true PPGL.
Internal ID Number: 00682
Health Subject: DIAGNOSIS
Type: Conference
Appears in Collections:Research Output

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