[Sarcomatoid variant of adrenocortical carcinoma]

Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis and limited response to current therapies. The sarcomatoid subtype represents the rarest and most aggressive form of ACC, with very few cases reported in the literature. We present the case of a 62-year-old male with a history of an untreated left adrenal incidentaloma, who was diagnosed four years later with advanced-stage sarcomatoid ACC. Despite surgical and oncologic treatment, the disease progressed rapidly with systemic involvement and limited survival. The histopathological diagnosis is particularly challenging, as this subtype is characterized by the coexistence of epithelial and fusocelular/pleomorphic components with sarcomatoid differentiation, which may hinder its distinction from other primary or metastatic neoplasms. This case underscores the importance of appropriate initial evaluation of adrenal incidentalomas and highlights the highly malignant behavior of this rare variant, emphasizing the need for early diagnosis and timely intervention.