Abstract
Malignant psammomatous melanotic schwannoma (MPMS) is a rare type of tumour, occasionally reported to occur with mediastinal involvement. Histopathologic similarities with melanoma may lead to a wrong diagnosis, but distinguishing between types of tumours is mandatory for adequate management and prognosis. MPMS may be aggressive and manifest unpredictable behavior, with a poor midterm prognosis despite benign histopathologic features. We discuss the challenges that come with a diagnosis of MPMS, and the rationale for our treatment strategy, in this first report regarding MPMS involving the left heart ventricle.