title: Premelanosome-negative inflammatory angiomyolipoma of liver with expression of cathepsin K and TFE3.
abstract: We report the first case of inflammatory variant of hepatic angiomyolipoma (AML) with expression of transcription factor E3 (TFE3) protein but negativity for HMB45 and melan A in a 62-year-old female. Imaging studies revealed a tumor in the left lobe of liver, sized 5.8 cm in maximum diameter. Microscopically, the lesion was composed of large polygonal or epithelioid cells with copious eosinophilic granular cytoplasm. There was a very prominent stromal lymphoplasmacytic infiltrate. Immunohistochemically, the tumor cells showed very strong and diffuse positivity for smooth muscle actin, and cathepsin K, while S-100 protein, keratin, desmin, HMB45 and Melan-A are negative. However, there was multifocal and very convincing nuclear positivity for TFE3, thus confirms the diagnosis.
authors.affiliation: Department of Pathology, The First Affiliated Hospital, Zhengjiang University Medicine School Hangzhou 310003, Zhejiang, P. R. China.
authors.forename: Ke
authors.initials: K
authors.lastname: Sun
authors.affiliation: Department of Pathology, Zhejiang Provincial People's Hospital Hangzhou 310014, Zhejiang, P. R. China.
authors.forename: Ming
authors.initials: M
authors.lastname: Zhao
authors.affiliation: Department of Pathology, The First Affiliated Hospital, Zhengjiang University Medicine School Hangzhou 310003, Zhejiang, P. R. China.
authors.forename: Hongtian
authors.initials: H
authors.lastname: Yao
authors.affiliation: Department of Pathology, The First Affiliated Hospital, Zhengjiang University Medicine School Hangzhou 310003, Zhejiang, P. R. China.
authors.forename: Lijun
authors.initials: L
authors.lastname: Wang
authors.affiliation: Department of Hepatobiliary Surgery, The First Affiliated Hospital, Zhengjiang University Medicine School Hangzhou 310003, Zhejiang, P. R. China.
authors.forename: Jianfeng
authors.initials: J
authors.lastname: Wei
mesh_terms: D018207:Angiomyolipoma / Q000378:metabolism / Q000473:pathology*; D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors / Q000096:biosynthesis*; D014408:Biomarkers, Tumor / Q000032:analysis; D056657:Cathepsin K / Q000096:biosynthesis*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008113:Liver Neoplasms / Q000378:metabolism / Q000473:pathology*; D008875:Middle Aged; D058951:gp100 Melanoma Antigen / Q000096:biosynthesis
chemical_list: D051778:Basic Helix-Loop-Helix Leucine Zipper Transcription Factors; D014408:Biomarkers, Tumor; C548637:PMEL protein, human; C069203:TFE3 protein, human; D058951:gp100 Melanoma Antigen; C534130:CTSK protein, human; D056657:Cathepsin K
keywords: Liver; TFE3; angiomyolipoma; cathepsin K; differential diagnosis; epithelioid tumor; inflammatory