Composite Hemangioendothelioma (CH) is an extremely rare vascular neoplasm which develops predominantly in the skin, soft-tissue, and musculoskeletal system. Very few cases have been described in viscera (kidney, spleen), but never in the adrenal. CH is thought to have an intermediate biological potential, with benign, low-grade malignant, and malignant vascular components. An even more rare (and more aggressive) subtype of CH is this with neuroendocrine differentiation (CH-ND). To the best of our knowledge we present the first reported case of an adrenal CH-ND with a follow-up of 1,5 year after complete resection with the posterior retroperitoneoscopic procedure.

A 39-year old otherwise healthy woman was referred due to an 8.7 cm mixed mass of the left adrenal with multiple cystic and solid components in the computed-tomography scan (pictures shown). She underwent a complete left adrenal gland removal with the 3-keyhole prone retroperitoneoscopic approach. We present the Operative Video which shows the complete resection of the mass. The patient was discharged the day after surgery.

Pathology showed an adrenal CH of the neuroendocrine subtype, characterized by a diffuse expression of endothelial markers. The Ki67 index was low (2%). Histology pictures are provided. The case was discussed in the oncological multidisciplinary meeting and a 6-month follow-up with imaging was decided. At 1,5 year of follow-up there are not any findings of recurrence or of metastasis.

This is the first described case of an adrenal CH-ND. The complete and oncologic removal of a large (>8.5cm) CH is feasible with the minimally invasive posterior retroperitoneoscopy.