Endocrine Abstracts

Introduction: Lymphangioma is a rare benign malformation of the lymphatic system that typically affects the neck and head during childhood. Adrenal glands are an uncommon and rare location for lymphangioma. Herein, we report an unusual clinical presentation of an adrenal lymphangioma revealed by Cushing syndrome.

Case report: A 69-year-old female was referred to our department for exploration of an adrenal incidentaloma. Her medical history was positive for dyslipidemia and depressive symptoms. She was admitted in the emergency department for a five-month history of diffuse abdominal pain. Clinical and biochemical findings were normal. Abdominal sonography was performed, revealing a large heterogeneous mass measuring 9×8 cm, located in the right adrenal gland. Then, an endocrine assessment was considered. On admission, physical examination revealed fragile skin, easy bruisability and normal blood pressure. Her laboratory results were within reference ranges. The endocrinlogical workup revealed a plasma adrenocorticotropic hormone (ACTH) at 8.17 pg/ml (normal <45 pg/ml), morning serum cortisol of 220 ng/ml and unsuppressed cortisol after 1 mg overnight dexamethasone suppression test. Low-dose dexamethasone suppression testing showed unsuppressed cortisol at 23.5 ng/ml. These results were compatible with ACTH independent Cushing syndrome. Serum metanephrines, normetanephrines and androgens levels were normal. On abdominal computed tomography (CT), there was evidence of a right giant heterogeneous mass with central and peripheral calcifications measuring 10×8 cm, suggestive of an adrenocortical carcinoma. Abdominal MRI confirmed CT scan’ findings, showing large necrotic tissue. Fluorodeoxyglucose (FDG) Positron Emission Tomography (PET) scan did not demonstrate any metastasis. The patient underwent right adrenalectomy, under hydrocortisone coverage, through a right subcostal incision. Lumbo-aortic lymph node dissection was also performed. Unexpectedly, the histopathological examination was consistent with the diagnosis of a benign adrenal Lymphangioma. The postoperative period was uneventful, with pituitary–adrenal recovery.

Conclusion: Adrenal lymphangiomas remain extremely rare.However, they must be included in the differential diagnosis of adrenal mass.Clincial and radiological features are variable, leading to a misdiagnosis.Pathological examination is essential for the diagnosis.