Multifocal Hepatic Angiosarcoma with Atypical Presentation: Case Report and Literature Review
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CASE REPORT
Multifocal Hepatic Angiosarcoma with Atypical Presentation: Case Report and Literature Review Stefano Marletta 1 & Enrico Cavallo 1 & Serena Ammendola 1 & Lavinia Stefanizzi 1 & Maria Gaia Mastrosimini 1 & Mirko D’Onofrio 2 & Matteo Brunelli 1 & Anna Caliò 1 & Sara Pecori 3 & Andrea Dalbeni 4 & Andrea Ruzzenente 5 & Paola Capelli 3
# The Author(s) 2020
Abbreviations HAS Hepatic angiosarcoma VCM Vinyl chloride monomer PVC Polyvinyl chloride
Introduction Hepatic angiosarcoma (HAS) is a malignant mesenchymal neoplasm composed of highly atypical, pleomorphic endothelial cells, which diffusely grow along liver sinusoids and other preformed vascular channels, especially at the periphery of the tumor, replacing normal endothelial cells. More solid areas, resembling sarcomas, can be found [1]. Far less common than hepatocarcinoma, representing less than the 2% of all the primary malignant hepatic tumors, HAS is still the most frequent primary malignant mesenchymal neoplasm of the liver. Although most of HAS do not carry a specific etiology, several chemical carcinogens, including vinyl chloride monomer (VCM), Thorotrast (thorium dioxide), and arsenic, have been listed as being responsible for occupational cases. VCM is a
colorless, sweet-smelling gas primary used in the production of polyvinyl chloride (PVC), whose resins are almost ubiquitously present in building materials, construction, and home furnishings. VCM is now classified as an International Agency for Research on Cancer (IARC) Group 1 carcinogen, due to the correlation between HAS and its occupational exposure [2]: initially described in three workers at a VCM polymerization plant in Kentucky in 1974 [3], several other HAS occupational cases have been reported worldwide in the following decades, all linked to workers’ exposure to VCM [4, 5]. Because HAS is an extremely rare tumor, both radiological and histological diagnosis could be really challenging and their integration with clinical data and history has a key role in reaching the correct diagnosis. Herein the case of a primary multifocal HAS is described, with initially misleading unspecific radiological findings. Suggesting the biopsy a malignant vascular lesion of the liver, an autopsy was performed, which confirmed the diagnosis of HAS.
Stefano Marletta and Enrico Cavallo contributed equally to this work.
Case Report
* Stefano Marletta [email protected]
A 73-year-old man with a history of hypertension and congestive cardiac failure was admitted at our hospital for progressive jaundice of unknown etiology. During prior hospitalizations multiple hepatic nodular lesions of uncertain nature had been reported at various imaging exams; furthermore, two ultrasound-guided liver biopsies had already been formerly performed, leading to a diagnosis of nodular regenerative hyperplasia of uncertain interpretation. After admission to our hospital, laboratory tests revealed increased direct bilirubin levels (9.59 mg/dl), while liver enzymes were within the recognized normal ranges. More
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