Primary Malignant Mesenchymal Tumors of the Liver

Abstract

Primary malignant mesenchymal tumors represent a rare subset of hepatic lesions. These lesions pose unique diagnostic challenges for a pathologist because they are infrequently seen in general practice and can be easily mistaken for their benign counterparts or mimickers. In the current era of personalized medicine, the precise pathological diagnosis of these lesions is critically important to guide targeted therapy. In this article, we first briefly review the key clinical, pathological, immunohistochemical, and molecular characteristics of both common and uncommon primary malignant mesenchymal tumors of the liver. We then outline a practical diagnostic approach based on the unique features of each entity. This approach may help the general pathologist and pathology trainees navigate through these complex uncommon lesions and arrive at a specific diagnosis.