Abstract
Atypical spindle cell lipomatous tumour (ASCLT) is a benign neoplasm that presents a variable proportion of atypical spindle and adipocytic cells, frequently expressing CD34, and embedded in myxoid or collagenous matrix. An important feature is a constant lack of either MDM2 or CDK4 amplification. It typically arises in the extremities. The retroperitoneum is a rare site of involvement. We report a case of a retroperitoneal ASCLT in a 62-year-old male. A differential diagnosis of ASCLT from the other mesenchymal, spindle-cell, and lipomatous tumours is crucial for optimal treatment and significantly influences the prognosis. A diagnosis should be warranted by the immunohistochemistry and molecular findings.