RESUMEN
CONTEXT: Classic Kaposi sarcoma (KS), also known as Mediterranean KS, affects immunocompetent patients and is usually limited to the skin, without profound organ involvement. We report an exceptional case of a primary adrenal classic KS. CASE DESCRIPTION: A left adrenal incidentaloma was fortuitously discovered on a computed tomography scan performed for chest pain in a 60-year-old man. Magnetic resonance imaging showed a heterogeneous left adrenal nodule enhanced by gadolinium injection. Adrenalectomy revealed a massive spindle cell infiltrate of the adrenal gland that was positive for CD31, CD34, and herpes virus 8 (HHV8) on immunohistochemistry, allowing for the diagnosis of KS. The explorations revealed no immunodeficiency or other involvement of KS. Four months later, another nodular lesion appeared on the right adrenal gland, and 2.5 years later, two nodular angiomatous KS lesions had appeared on the right foot. The evolution was indolent, and no complementary treatment of KS was required at 3 years after the diagnosis. CONCLUSIONS: Adrenal involvement of KS is rare, eventually observed in AIDS-KS. The present case is, to the best of our knowledge, the first report of primary isolated adrenal classic KS. KS should be considered in the etiology of adrenal incidentaloma, especially if the patient has epidemiological risk factors for HHV8 infection, mainly, but not exclusively, in the context of immunodeficiency.