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Pseudotumors and Related Lesions of the Hepatobiliary Tract

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Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Abstract

Pseudotumors and inflammatory pseudotumors form a heterogeneous group of lesions characterized by the presence of an inflammatory infiltrate and proliferations of fibroblasts and myofibroblasts. Pseudotumors are mass-forming lesions, also in the hepatobiliary tract, that may mimic diverse malignancies. Specifically, pseudotumors can grow in the walls of extrahepatic bile ducts, with formation of stenosing and obstructive lesions clinically similar to cholangiocarcinoma. The term inflammatory myofibroblastic tumor (IMT) has been proposed as synonym for pseudotumor, with a generally benign behavior. However, a subset of these lesions show an aggressive growth pattern and even malignant behavior, suggesting a neoplastic character of some pseudotumors. Furthermore, about half of recently analyzed cases of IMT carry rearrangements of the anaplastic lymphoma kinase (ALK) locus on chromosome 2p23, causing aberrant ALK expression. Based on these findings, the classification of so-called pseudotumors, mainly the identification of reactive versus neoplastic forms, requires revision. A variant lesion that is sometimes classified together with IMT is Epstein-Barr virus-positive inflammatory pseudotumor (inflammatory pseudotumor-like follicular dendritic cell tumor).

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    Arthur Zimmermann

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Zimmermann, A. (2017). Pseudotumors and Related Lesions of the Hepatobiliary Tract. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_119

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