Tommaso Claudio Mineo, MD

Here we examine recent advances in the knowledge of this severe and heterogeneous malignancy, and we analyze the clinical significance of prognostic factors.

The rarity and heterogeneity of these tumors limits research and obviates the possibility of large randomized clinical trials. Surgery remains the mainstay of treatment, but often radiotherapy and/or chemotherapy is required. The role of neoadjuvant therapy is evolving. In the not too distant future, new therapies tailored to the molecular profile of the tumor will be available.