| Summary: | Adrenal cystic lymphangiomas are unusual entities with a very low prevalence that are typically discovered incidentally. We describe a 45-year-old male known for hypertension that was referred to our endocrinology department for right adrenal incidentaloma. Seven months prior referral, he presented right hypochondrium pain, vomiting and nausea and imaging studies included a computer tomography that revealed an irregular-shaped right adrenal lesion (42 × 23 mm), with scattered calcifications in the medial branch and heterogenous contrast enhancement and contrast-enhanced computer tomography showed an absolute wash-out of 28% and hyperattenuating areas (>90 HU). Physical examination was unremarkable. Hormonal studies were all within normal range. Due to the findings of imaging studies and lack of biochemical findings a presumptive diagnosis of non-producing adrenal carcinoma was made. Laparoscopic of right adrenalectomy was carried out. Macroscopic appearance of the resected right adrenal surface showed a 3.0 × 2.5 cm whitish-grayish multicystic lesion with solid areas that replaced all adrenal medulla and almost all the cortex. Microscopic examination revealed multiple cystic channels lined with endothelial cells without atypia or mitotic figures. On immunohistochemical examination, anti-calretinin, anti-D2-40 and anti-Factor VIII were all positive confirming the diagnosis of adrenal cystic lymphangioma. It is a rare pathology that should be considered as part of the differential diagnosis during the evaluation of adrenal gland masses.
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