EYES2019 7th ESE Young Endocrinologists and Scientists (EYES) Meeting Poster Presentations (46 abstracts)
Pheochromocytoma, unmasking the chameleon
Clara Cunha 1 , Cátia Ferrinho 1 , Francisco Sousa Santos 1 , Eugénia Silva 1 , Luís Viana Fernandes 2 , Catarina Saraiva 1 , Manuela Oliveira 1 & João Sequeira Duarte 1
1Department of Endocrinology and Diabetes, Hospital Egas Moniz, Lisbon, Portugal; 2Department of General Surgery, Hospital Egas Moniz, Lisbon, Portugal.
Background: Pheochromocytoma is a rare catecholamine secreting tumor originating usually from adrenal medulla and representing approximately 5% of adrenal incidentalomas. Currently 10% of all pheochromocytomas are discovered incidentally during imaging studies for unrelated disorders.
Case presentation: A thirty five year old woman with no relevant medical history presented at the emergency department with symptoms of left flank pain and vomiting. Abdominal ultrasonography revealed renal microlithiasis and a right adrenal mass. The abdominal computed tomography confirmed a heterogeneous right adrenal mass with 118×105 mm with cystic and hemorrhagic component consistent with right adrenal pheochromocytoma. The hypertension was controlled with verapamil 40 mg twice daily. Biochemistry tests revealed markedly raised plasma normetanephrines (12746 μg/24 h, reference range 162–527), slightly increased metanephrines (518 μg/24 h, reference range 64–302), elevated chromogranin A (31 nom/l reference range <3) and 3-methoxytyramine (2227 μg/24 h, reference range 103–434). The metaiodobenzylguanidine (MIBG) scintigraphy only identified hyperfixation in the right adrenal gland. After alfa and beta blockade patient underwent laparoscopic right adrenalectomy, and the histopathologic features were consistent with pheochromocytoma. Following surgery her hypertension has resolved and the value of metanephrines and normetanephrines four weeks after the surgery was normal. The result of genetic testing (VHL, MAX, SDHB, SDHC, SDHD) was negative.
Conclusions: Pheochromocytoma is a rare catecholamine-producing tumor requiring a high index of suspicion for early diagnosis. Our case report highlights the importance of considering pheochromocytoma in the workup of patients with adrenal incidentalomas, whose incidence is increasing due to the better availability and accessibility of imaging procedures.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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