The diagnostic dilemma of adrenal endothelial tumors: analysis of 21 cases and systematic review of the literature

Abstract

Purpose Adrenal endothelial tumors are mainly represented by adrenal cavernous hemangiomas (ACHs) and adrenal cystic lymphangiomas (ACLs). Their radiological features often overlap with malignant tumors, therefore ruling out malignancy becomes mandatory. We analyzed clinical, radiological, and histopathological data to identify specific characteristics of these tumors. Methods We reviewed 21 patients with ACHs (n = 12), ACLs (n = 8) or adrenal cysts (n = 1) confirmed by histopathology. We selected 82 papers from PubMed to provide a systematic review of the literature. Results In our cohort, median age at diagnosis was 58 years, with sex evenly distributed. All tumors were unilateral (median size = 44 mm), with 8 cases of increasing tumor size. All tumors exhibited non-contrast CT density > 10 Hounsfield Unit (HU). Calcifications were found in 5 cases. Hormonal studies revealed 11 non-functioning tumors and 2 cortisol-secreting tumors. Elevated urinary metanephrines were found in 2 cases. Immunostaining showed CD31/CD34/factor VIII expression in ACHs (n = 5, 24%) and podoplanin expression in ACLs (n = 6, 29%). Literature review revealed 71 reported cases of ACHs and 104 reported cases of ACLs. Median age at diagnosis was 46 years, with a slightly female prevalence (63%). Median tumor size was 48 mm. 84 cases were symptomatic, with life-threatening hemorrhage reported in only 3 patients. Calcifications were found in 23% of cases. Surgical approaches varied, with open and laparoscopic adrenalectomy performed in 55 and 42 patients respectively. Conclusions ACHs and ACLs represent a diagnostic dilemma in clinical practice due to their rarity and their misleading imaging features.