Abstract and Introduction
Objective: Cystic adrenal mass is a rare imaging presentation of pheochromocytoma. We aimed to describe the clinical, biochemical and imaging characteristics of patients with cystic pheochromocytoma.
Design: Single-centre, retrospective study, 2000–2020.
Patients: Consecutive patients with cystic pheochromocytoma were identified from our institutional pathology and adrenal tumour database.
Results: Of the 638 patients with pheochromocytomas, 21 (3.2%) had cystic pheochromocytomas (median age: 57 years, 57% women). Most pheochromocytomas were discovered incidentally (57%) or due to symptoms of catecholamine excess (24%). The median tumour size was 6.4 cm. On imaging, cystic pheochromocytomas were round or oval (90%), heterogeneous lesions (86%) with a thick solid rim (median rim thickness 13.9 mm, unenhanced computed tomography (CT) attenuation 40 Hounsfield units (HU), venous-phase CT attenuation 83 HU), and a median cystic component of 40% (unenhanced CT attenuation 17.6 HU, venous-phase CT attenuation 20.4 HU), and rarely with calcifications (15%). All 20 patients with biochemical testing had functioning tumours (adrenergic in 80%, noradrenergic in 20%). Total urinary metanephrine excretion correlated with the volume of the solid component (R 2 = .75, p < .0001) but not the cystic component (R 2 = .04, p = .4386). All patients underwent adrenalectomy (48% laparoscopic, 52% open), and the median duration of hospital stay was 4 days.
Conclusions: Cystic pheochromocytomas are rare, large tumours with a phenotypic appearance that can masquerade as other adrenal cystic lesions. The degree of biochemical abnormality in cystic pheochromocytomas is associated with the volume of the solid component. All patients with adrenal cysts that have a solid component or an unenhanced attenuation >10 HU should undergo biochemical testing for pheochromocytoma.
Pheochromocytomas are rare catecholamine-secreting neuroendocrine tumours arising from the chromaffin cells in the adrenal medulla. In a population-based study of 1287 patients with adrenal tumours, pheochromocytomas represented 1.1%. Most pheochromocytomas are discovered incidentally on imaging performed for reasons other than adrenal mass and are typically solid tumours with unenhanced attenuation on computed tomography (CT) of >20 Hounsfield units (HU).[3,4] While the presence of focal cystic changes in pheochromocytomas is common, presentation with a macroscopic cystic component is rare. These cases may pose a diagnostic challenge risking inadequate preparation for surgery due to misdiagnosis as an adrenal cyst. Due to its rarity, evidence regarding cystic pheochromocytomas is limited to case reports or small pathology-based series of up to six patients.[6–10]
An accurate preoperative diagnosis of pheochromocytoma is important to assure the best perioperative outcomes. Our objectives were to (1) characterize the clinical, biochemical, imaging characteristics and outcomes of patients with cystic pheochromocytoma and (2) determine the association of the solid and cystic component volumes of pheochromocytoma with the degree of catecholamine excess.
Clin Endocrinol. 2023;98(1):32-40. © 2023 Blackwell Publishing