Poster Session 1.D - Pathological and Oncological Sciences
Dr. Szalai, Fatime
KVGNAE
Department of Pathology and Experimental Cancer Research
+36 30 859 8032
fatie.salay@gmail.com
GPNMB Immunohistochemistry in the Diagnosis of Lymphangioleiomyomatosis
Fatime Szalai1, Judit Pápay1, Katalin Dezső1, Levente Kuthi1,2, Anna Sebestyén1, Andras Khoor3, Ildikó Krencz1
1: Department of Pathology and Experimental Cancer Research, Semmelweis University
2: Department of Surgical and Molecular Pathology, Tumor Pathology Center, National Institute of Oncology
3: Department of Laboratory Medicine & Pathology, Mayo Clinic, Jacksonville, Florida, USA
Poszter
Poster Session 1.D - Pathological and Oncological Sciences
Hungarian
Pathological and Oncological Sciences
Introduction
Lymphangioleiomyomatosis (LAM), a rare pulmonary neoplasm classified as a perivascular epithelioid cell tumor (PEComa), is associated with the cystic destruction of the lung parenchyma, which may lead to respiratory failure. The pathological diagnosis of LAM is aided by positivity for smooth muscle and melanocyte markers; however, distinguishing it from other histological entities remains challenging in some cases, particularly in small biopsies. Glycoprotein non-metastatic melanoma protein B (GPNMB) has recently been identified as a sensitive and specific immunohistochemical marker for PEComas, but only a few cases of LAM have been studied to date.
Aims
The aim of our study was to determine the diagnostic value of GPNMB immunohistochemistry as an ancillary marker in a larger cohort of LAM samples.
Methods
Immunohistochemical analysis of GPNMB expression was performed on human LAM samples (N=15), histological entities potentially considered during differential diagnosis (N=30), and normal lung tissue (N=2). We used the H-score method to quantify staining intensity; an H-score greater than 100 was considered high.
Results
All LAM samples showed high cytoplasmic GPNMB expression, and cell membrane positivity was also observed in some areas. In contrast, histological mimics demonstrated no or low GPNMB expression. Considering the H-score threshold of >100, GPNMB immunohistochemistry demonstrated 100% sensitivity and specificity in identifying LAM in our cohort. Normal epithelial and mesenchymal elements of the lung parenchyma did not stain for GPNMB; however, weak to moderate expression was observed in alveolar macrophages in both normal and diseased lung tissues.
Conclusion
Based on our study, GPNMB immunohistochemistry is a highly sensitive and specific marker that may aid in the pathological diagnosis of LAM. Beyond its diagnostic value, GPNMB positivity in the membrane of LAM cells may predict the efficacy of GPNMB-targeted antibody-drug conjugates (e.g., glembatumumab vedotin) in the treatment of the disease. In addition, recent studies suggest that GPNMB may also serve as a potential serum marker and may indicate the extent of response to mTOR-inhibitor therapy.
Funding
Hungarian Pulmonology Foundation, EFOP‐3.6.3‐VEKOP‐16‐2017‐00009
Semmelweis University
Ildikó Krencz
I do not give consent to the publication of my abstract on the website of the congress.
in doctoral studies after complex exam (PhD)
Szabad
elfogadva
poszter
nem rendelkezett róla
8995
17:06
17:09