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Malignant Fibrous Histiocytoma of the Liver: A Neoplasm of the Undifferentiated High-Grade Pleomorphic Sarcoma Group

ICD-O code 8830/3

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Tumors and Tumor-Like Lesions of the Hepatobiliary Tract

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Abstract

The term malignant fibrous histiocytoma (MFH) previously denoted a malignancy suggested to originate from histiocytes. Later, it was realized that MFH represents a heterogeneous group of sarcomas of variable cell lineages. The current WHO classification includes MFH as subtypes of undifferentiated high-grade polymorphous sarcoma (UPS). The tumors exist in the form of five histologic patterns, i.e., storiform-pleomorphic, myxoid, giant cell, angiomatoid, and inflammatory MFH/UPS. Primary MFH in its original sense is a very rare liver neoplasm that occurs in older individuals. Hepatic MFH can be associated with paraneoplastic syndromes, including hypoglycemia due to secretion of ILF-II, and prominent leukocytosis and leukemoid reactions due to ectopic production of granulocyte colony-stimulating factor. Hepatic MFH/UPS presents as solitary or multiple irregular tumor masses and exhibits the same histologic patterns as its extrahepatic counterparts. MFH/UPS of the liver is an aggressive neoplasm with a high recurrence rate.

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    Arthur Zimmermann

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Zimmermann, A. (2017). Malignant Fibrous Histiocytoma of the Liver: A Neoplasm of the Undifferentiated High-Grade Pleomorphic Sarcoma Group. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_71

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