Ang-1 and Ang-2 expression in angiomyolipoma and PEComa family tumors

Swati Shrestha; Carolyn Meyers; Jia Shen; Paulina Giacomelli; Michelle A Scott; Chia Soo; Sarah M Dry; Kang Ting; Aaron W James

doi:10.1016/j.jor.2016.12.001

Ang-1 and Ang-2 expression in angiomyolipoma and PEComa family tumors

J Orthop. 2016 Dec 27;14(1):154-160. doi: 10.1016/j.jor.2016.12.001. eCollection 2017 Mar.

Authors

Swati Shrestha¹, Carolyn Meyers², Jia Shen¹, Paulina Giacomelli¹, Michelle A Scott³, Chia Soo⁴, Sarah M Dry², Kang Ting¹, Aaron W James⁵

Affiliations

¹ School of Dentistry, University of California, Los Angeles, USA.
² Department of Pathology, The Johns Hopkins University, Baltimore, MD, USA.
³ Nationwide Children's Hospital, Columbus, OH, USA.
⁴ Orthopedic Hospital Research Center, University of California, Los Angeles, USA; Department of Surgery, University of California, Los Angeles, Los Angeles, CA, USA.
⁵ Department of Pathology, The Johns Hopkins University, Baltimore, MD, USA; Orthopedic Hospital Research Center, University of California, Los Angeles, USA.

Abstract

Objective: Perivascular epithelioid cell tumors (PEComa) are an uncommon family of soft tissue tumors. Previously, we described that the presence of pericyte antigens among PEComa family tumors differs extensively by histologic appearance.

Methods: Here, we extend our findings using the pericyte antigens Angiopoietin-1 (Ang-1) and Angiopoietin-2 (Ang-2), using immunohistochemical detection in human tumor samples.

Results: While Ang-1 showed no expression across any PEComa family tumor, Ang-2 showed expression that like other pericyte markers was largely determined by cytologic appearance.

Conclusion/implications: Pericytic marker expression in PEComa may represent a true pericytic cell of origin, or alternatively aberrant pericyte marker adoption.

Keywords: Angiomyolipoma; Angiopoietin; Lymphangiomyoma; PEComa; Pericyte; Perivascular epithelioid cell tumor.

Grants and funding

K08 AR068316/AR/NIAMS NIH HHS/United States