Immunohistochemical Expression of Renin & GATA3 Help Distinguish Juxtaglomerular Cell Tumors from Renal Glomus Tumors

Authors

Sounak Gupta, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, 55905, USAFollow
Andrew L. Folpe, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, 55905, USAFollow
Jorge Torres-Mora, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, 55905, USAFollow
Victor E. Reuter, Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, 10065, USAFollow
Jonathan E. Zuckerman, Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at University of California, Los Angeles, CA, 90095, USAFollow
Nadja Falk, Department of Pathology, University of New Mexico Health Sciences Center, Albuquerque, NM, 87131, USAFollow
Melissa L. Stanton, Department of Laboratory Medicine and Pathology, Mayo Clinic, Phoenix, AZ, 85054, USAFollow
Selvaraj Muthusamy, Department of Pathology and Urology, VCU School of Medicine, Richmond, VA, 23298, USAFollow
Steven C. Smith, Department of Pathology and Urology, VCU School of Medicine, Richmond, VA, 23298, USAFollow
Vidit Sharma, Department of Urology, Mayo Clinic, Rochester, MN, 55905, USAFollow
Sanjeev Sethi, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, 55905, USAFollow
Loren Herrera-Hernandez, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, 55905, USAFollow
Rafael E Jimenez, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, 55905, USAFollow
John C. Cheville, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, 55905, USAFollow

Document Type

Article

Publication Date

10-1-2022

Abstract

Juxtaglomerular cell tumors and glomus tumors both arise from perivascular mesenchymal cells. Juxtaglomerular cells are specialized renin-secreting myoendocrine cells in the afferent arterioles adjacent to glomeruli, and juxtaglomerular tumors derived from these cells are therefore unique to the kidney. In contrast, glomus tumors have been described at numerous anatomic sites and may show significant morphologic and immunophenotypic overlap with juxtaglomerular tumors when occurring in the kidney. Although ultrastructural studies and immunohistochemistry for renin may distinguish these entities, these diagnostic modalities are often unavailable in routine clinical practice. Herein, we studied the clinicopathologic features of a large series of juxtaglomerular tumors (n = 15) and glomus tumors of the kidney (n = 9) to identify features helpful in their separation, including immunohistochemistry for smooth muscle actin (SMA), CD34, collagen IV, CD117, GATA3, synaptophysin, and renin. Markers such as SMA (juxtaglomerular tumors: 12/13, 92%; glomus tumors: 9/9, 100%), CD34 (juxtaglomerular tumors: 14/14, 100%; glomus tumors: 7/9, 78%), and collagen IV (juxtaglomerular tumors: 5/6, 83%; glomus tumors: 3/3, 100%) were not helpful in separating these entities. In contrast to prior reports, all juxtaglomerular tumors were CD117 negative (0/12, 0%), as were glomus tumors (0/5, 0%). Our results show that juxtaglomerular tumors have a younger age at presentation (median age: 27 years), female predilection, and frequently exhibit diffuse positivity for renin (10/10, 100%) and GATA3 (7/9, 78%), in contrast to glomus tumors (median age: 51 years; renin: 0/6, 0%; GATA3: 0/6, 0%). These findings may be helpful in distinguishing these tumors when they exhibit significant morphologic overlap.

Recommended Citation

Gupta S, Folpe AL, Torres-Mora J, Reuter VE, Zuckerman JE, Falk N, Stanton ML, Muthusamy S, Smith SC, Sharma V, Sethi S, Herrera-Hernandez L, Jimenez RE, Cheville JC. Immunohistochemical expression of renin and GATA3 help distinguish juxtaglomerular cell tumors from renal glomus tumors. Hum Pathol. 2022 Oct;128:110-123. doi: 10.1016/j.humpath.2022.07.016. Epub 2022 Aug 1. PMID: 35926808.