Mesenchymal tumors and tumor-like lesions of the liver can present a diagnostic challenge due to their overlapping spindle cell morphology, varying stromal composition, and inflammatory backgrounds. Histologic pattern recognition is critical in distinguishing these entities, with key features including storiform or fascicular spindle cell arrangements, myxoid or collagenous stroma, perivascular growth, cystic or nested epithelial components, and distinctive vascular patterns such as the staghorn vasculature in solitary fibrous tumors. The presence of inflammatory infiltrates, adipocytic differentiation, calcifications, or atypical mitotic figures further refines differential diagnoses. Immunohistochemical markers such as ALK, STAT6, HMB-45, and β-catenin provide additional specificity, guiding classification alongside molecular alterations like ALK and ROS1 rearrangements, NAB2::STAT6 fusion, MDM2 amplification, and TSC1/TSC2 mutations. While most of these lesions follow an indolent course, some exhibit malignant potential, local recurrence, or metastatic behavior, necessitating precise histologic assessment. In addition, metastatic mesenchymal tumors to the liver, such as gastrointestinal stromal tumors or sarcomas, must be carefully distinguished from primary hepatic lesions. Surgical resection remains the primary treatment, but the integration of molecular diagnostics has enabled targeted therapies, such as ALK and mTOR inhibitors, for selected cases. The convergence of histopathologic pattern recognition, immunophenotyping, and molecular profiling is essential in accurately classifying these rare hepatic lesions and optimizing patient management.

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Mesenchymal (Nonvascular) Tumors and Tumor-Like Lesions

  • Xiaoyan Liao

摘要

Mesenchymal tumors and tumor-like lesions of the liver can present a diagnostic challenge due to their overlapping spindle cell morphology, varying stromal composition, and inflammatory backgrounds. Histologic pattern recognition is critical in distinguishing these entities, with key features including storiform or fascicular spindle cell arrangements, myxoid or collagenous stroma, perivascular growth, cystic or nested epithelial components, and distinctive vascular patterns such as the staghorn vasculature in solitary fibrous tumors. The presence of inflammatory infiltrates, adipocytic differentiation, calcifications, or atypical mitotic figures further refines differential diagnoses. Immunohistochemical markers such as ALK, STAT6, HMB-45, and β-catenin provide additional specificity, guiding classification alongside molecular alterations like ALK and ROS1 rearrangements, NAB2::STAT6 fusion, MDM2 amplification, and TSC1/TSC2 mutations. While most of these lesions follow an indolent course, some exhibit malignant potential, local recurrence, or metastatic behavior, necessitating precise histologic assessment. In addition, metastatic mesenchymal tumors to the liver, such as gastrointestinal stromal tumors or sarcomas, must be carefully distinguished from primary hepatic lesions. Surgical resection remains the primary treatment, but the integration of molecular diagnostics has enabled targeted therapies, such as ALK and mTOR inhibitors, for selected cases. The convergence of histopathologic pattern recognition, immunophenotyping, and molecular profiling is essential in accurately classifying these rare hepatic lesions and optimizing patient management.