Vascular and perivascular tumors of the liver encompass a spectrum of benign and malignant neoplasms, often presenting diagnostic challenges due to overlapping histologic and radiologic features. While cavernous hemangiomas are the most common benign hepatic vascular tumors, rarer entities such as hepatic small vessel neoplasm (HSVN), angiosarcoma, and epithelioid hemangioendothelioma (EHE) require careful histopathologic and molecular analysis. Malignant vascular tumors, particularly angiosarcoma, exhibit aggressive behavior and can mimic benign lesions, necessitating immunohistochemical and molecular profiling for accurate diagnosis. EHE, characterized by WWTR1::CAMTA1 or YAP1::TFE3 fusions, presents as an infiltrative neoplasm with a distinctive myxohyaline stroma. HSVN, a recently recognized entity, demonstrates GNAQ or GNA14 mutations and may be mistaken for angiosarcoma. Perivascular epithelioid cell tumors (PEComas), including hepatic angiomyolipoma, can exhibit epithelioid morphology and mimic hepatocellular neoplasms, requiring melanocytic and smooth muscle markers for confirmation. The role of biopsy remains critical, particularly for lesions with ambiguous imaging characteristics. This chapter discusses the histopathologic, immunophenotypic, and molecular features of hepatic vascular and perivascular neoplasms, emphasizing differential diagnoses and diagnostic pitfalls. Advancements in molecular diagnostics are enhancing classification and risk stratification, aiding in the differentiation of benign from malignant vascular tumors. A comprehensive approach integrating clinical, radiologic, histologic, and molecular findings is essential for optimal diagnosis and management of these neoplasms.

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Vascular and Perivascular Tumors

  • Dorukhan Bahceci,
  • Ryan M. Gill

摘要

Vascular and perivascular tumors of the liver encompass a spectrum of benign and malignant neoplasms, often presenting diagnostic challenges due to overlapping histologic and radiologic features. While cavernous hemangiomas are the most common benign hepatic vascular tumors, rarer entities such as hepatic small vessel neoplasm (HSVN), angiosarcoma, and epithelioid hemangioendothelioma (EHE) require careful histopathologic and molecular analysis. Malignant vascular tumors, particularly angiosarcoma, exhibit aggressive behavior and can mimic benign lesions, necessitating immunohistochemical and molecular profiling for accurate diagnosis. EHE, characterized by WWTR1::CAMTA1 or YAP1::TFE3 fusions, presents as an infiltrative neoplasm with a distinctive myxohyaline stroma. HSVN, a recently recognized entity, demonstrates GNAQ or GNA14 mutations and may be mistaken for angiosarcoma. Perivascular epithelioid cell tumors (PEComas), including hepatic angiomyolipoma, can exhibit epithelioid morphology and mimic hepatocellular neoplasms, requiring melanocytic and smooth muscle markers for confirmation. The role of biopsy remains critical, particularly for lesions with ambiguous imaging characteristics. This chapter discusses the histopathologic, immunophenotypic, and molecular features of hepatic vascular and perivascular neoplasms, emphasizing differential diagnoses and diagnostic pitfalls. Advancements in molecular diagnostics are enhancing classification and risk stratification, aiding in the differentiation of benign from malignant vascular tumors. A comprehensive approach integrating clinical, radiologic, histologic, and molecular findings is essential for optimal diagnosis and management of these neoplasms.