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Endocrine Abstracts (2024) 99 EP1059 | DOI: 10.1530/endoabs.99.EP1059

1Carol Davila University of Medicine and Pharmacy, Endocrinology IV, Bucureşti; 2"Ana Aslan" National Institute of Geriatrics and Gerontology, Endocrinology, Bucharest, Romania; 3National Institute of Endocrinology, Endocrinology IV, Bucureşti; 4Carol Davila University of Medicine and Pharmacy, Endocrinology, Bucureşti


Introduction: Pheochromocytoma is a rare tumor, representing a cause of secondary endocrine hypertension. Traditionally, prior to the widespread availability of imaging investigations, pheochromocytoma was diagnosed based on the triad: headache, palpitations and sweating. Diagnosis is crucial, as 40-50% of pheochromocytoma patients exhibit genetic mutations associated with multiple syndromes, such as MEN2 syndrome, succinate dehydrogenase enzyme mutations, neurofibromatosis type 1 and von Hippel-Lindau syndrome. Management inevitably involves surgery, preceded by appropriate α-blockade. Lifelong monitoring is necessary due to the risk of recurrence and malignancy.

Case description: We present the case of a 50-year-old Caucasian woman, smoker of 30 pack-years, diagnosed with hypertension in 2022 for which she is currently undergoing treatment with Perindopril/Indapamide 5/1.25 mg. The patient describes three hypertensive crises associated with stressful events and hyperhidrosis for the past 3 months. In november 2023 she underwent an abdominal CT scan that revealed a nodularly transformed right adrenal gland, with dimensions of 90/66/81.2 mm, featuring an iodophilic tissue component, cystic areas and several central necrotic areas, suggestive for a giant right adrenal pheochromocytoma. Upon examination, the patient has hyperpigmentation on the anterior thorax, intermittent headache and a blood pressure of 150/98 mmHg. Calcitonin is within normal range. Plasma metanephrines and normetanephrines present values of 1770 pg/ml (>23 UNL) and 4860 pg/ml (>31 UNL), respectively. Markers specific of neuroendocrine tumors are elevated, such as chromogranin A (981 ng/ml; >9.5 UNL) and neuronal specific enolase (19.8 mg/l; >1.5 UNL). Urinary metanephrines and normetanephrines have elevated values of 2600 mg/24 h (>8.5 UNL) and 3900 mg/24 h (>7 UNL), respectively. Urinary 5-HIAA has normal values. The mean blood pressure after ambulatory blood pressure monitoring is 122/80.2 mmHg. Based on these results, the patient underwent surgery after receiving treatment with phenoxybenzamine for 3 weeks. The histopathology report is pending as well as the RET gene analysis.

Discussion: This case describes the management of a patient with giant oligosymptomatic pheochromocytoma. The particularity of the case is given by the large dimensions of the tumor (9 cm) and the unusual clinical picture of the patient.

Volume 99

26th European Congress of Endocrinology

Stockholm, Sweden
11 May 2024 - 14 May 2024

European Society of Endocrinology 

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