ECE2022 Poster Presentations Adrenal and Cardiovascular Endocrinology (87 abstracts)
Pre-, peri- and post-operative characteristics of biochemically silent pheochromocytomas: a case series
Athanasios Fountas 1 , Georgia Kanti 1 , Spyridoula Glikofridi 1 , Athanasia Kalantzi 1 , Irini Giagourta 1 , Athina Markou 1 , Maria Christou 2 , Stelios Tigas 2 , Georgia Ntali 3 , Chrysanthi Aggeli 4 , Giorgos Zografos 4 , Eleftheria Saoulidou 5 , Antonia Dimakopoulou 5 , Theodora Kounadi 1 & Labrini Papanastasiou 1
1General Hospital of Athens ’G. Gennimatas’ Unit of Endocrinology and Diabetes Center, Athens, Greece; 2University Hospital of Ioannina, Department of Endocrinology, Ioannina, Greece; 3‘Evangelismos’ General Hospital of Athens, Department of Endocrinology, Diabetes and Metabolism, Greece; 4’G. Gennimatas’ General Hospital of Athens, Third Department of Surgery, Athens, Greece; 5’G. Gennimatas’ General Hospital of Athens, Department of Anesthesiology, Athens, Greece
Introduction: Pheochromocytomas (PCs) and paragangliomas are rare tumours occurring in about 0.6 cases per 100,000 person-years. Biochemically silent PCs with normal catecholamine levels due to lack of catecholamine secretion or subtle secretion within the established normal levels are even rarer. Up to date, biochemically silent PCs are poorly investigated.
Aim: To assess the pre-, peri- and post-operative characteristics of patients with biochemically silent PCs
Design: Observational multicentre retrospective study between 2014-2021 in three tertiary hospitals.
Patients and methods: Records of patients diagnosed with biochemically silent PC and followed-up at each participating centre were reviewed and clinical, laboratory and imaging data, as well as treatment outcomes were recorded.
Results: Ten patients (5 men) with biochemically silent PC [median age 52.5 years (24-72) and BMI 25.26 Kg/m2 (16-34.2)] were included. The adrenal masses were incidentally discovered in all patients except one who presented with paroxysmal hypertension, palpitations and postural hypotension. Twenty-four-hour urine metanephrine and normetanephrine levels were found in the low-normal, normal and high-normal range in 4, 4 and 2 patients and in 1, 6 and 3 patients, respectively. Tumours were unilateral in all cases (7 on the right side) with a median size of 46 mm (17-125). High tumour density on pre-contrast CT imaging [median density 35 HU (22-45)] or high signal intensity on T2-weighted MRI scan was found in all cases; cystic and/or necrotic component was evident in 7 cases. 131I-MIBG scan was performed in 4 cases and it was positive in all of them. Alpha-adrenergic receptors blockade with phenoxybenzamine at a median total daily dose of 70 mg (20-100) was offered in 5 patients. Intra-operatively, 4 patients developed hypertension requiring glyceryl trinitrate administration (2 of them had no pre-operative treatment with phenoxybenzamine) and 8 developed hypotension; vasoconstrictors were required in 5 cases (3 of them did not receive pre-operative alpha-adrenergic receptors blockade). One patient, not pre-operatively treated with phenoxybenzamine, developed Takotsubo cardiomyopathy requiring admission in the ICU. On histology, PASS score was ≥4 (2-9) in all but one cases. During a median 24-month (12-88) period of follow-up, one patient had disease progression (a right retroperitoneal paraaortic metastasis detected 88 months after surgery).
Conclusions: Patients with biochemical silent PCs were in the vast majority asymptomatic and had lesions suspicious for PC on CT or MRI scan. Despite these tumours being biochemically silent, hemodynamic instability could manifest during surgical management. A multidisciplinary approach with experienced endocrinologist, surgeon and anaesthesiologist is mandatory.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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