Dataset Information

A case of adrenal undifferentiated pleomorphic sarcoma with tertiary lymphoid structures responded to pembrolizumab.

ABSTRACT:

Introduction

Although undifferentiated pleomorphic sarcomas are aggressive, a subset of these tumors are immunogenic and may respond to immunotherapy.

Case presentation

A 69-year-old man developed bilateral adrenal tumors and underwent bilateral adrenalectomy. Pathological examination revealed undifferentiated pleomorphic sarcoma harboring tertiary lymphoid structures and infiltration of CD8⁺ T cells. Genome profiling revealed PD-L1 amplification, microsatellite instability, and a high tumor mutation burden. He developed local recurrence and multiple peritoneal dissemination 2 months after surgery; adriamycin chemotherapy was ineffective for these lesions. Sustained complete remission of all lesions was achieved by administering pembrolizumab.

Conclusion

Immunohistochemical analysis focusing on tertiary lymphoid structures and genome profiling to evaluate microsatellite instability and tumor mutation burden are essential for precision medicine and informed clinical decision-making when treating advanced undifferentiated pleomorphic sarcoma.

SUBMITTER: Kokubun H

PROVIDER: S-EPMC10622227 | biostudies-literature | 2023 Nov

REPOSITORIES: biostudies-literature

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Publications

A case of adrenal undifferentiated pleomorphic sarcoma with tertiary lymphoid structures responded to pembrolizumab.

Kokubun Hidetoshi H Kijima Toshiki T Takada-Owada Atsuko A Mamiya Daisuke D Kurashina Ryo R Okubo Naoya N Uematsu Toshitaka T Takei Kohei K Ishida Kazuyuki K Kamai Takao T

IJU case reports 20230914 6

<h4>Introduction</h4>Although undifferentiated pleomorphic sarcomas are aggressive, a subset of these tumors are immunogenic and may respond to immunotherapy.<h4>Case presentation</h4>A 69-year-old man developed bilateral adrenal tumors and underwent bilateral adrenalectomy. Pathological examination revealed undifferentiated pleomorphic sarcoma harboring tertiary lymphoid structures and infiltration of CD8<sup>+</sup> T cells. Genome profiling revealed PD-L1 amplification, microsatellite instabi ...[more]

PMID: 37928308

Similar Datasets

Project description:IntroductionUndifferentiated pleomorphic sarcoma (UPS) can be associated with a relatively dense immune infiltration. Immune checkpoint inhibitors (anti-PD1, anti-PDL1, and anti-CTLA4) are effective in 20% of UPS patients. We characterize the immune microenvironment of UPS and its association with oncologic outcomes.Material and methodsSurgically resected UPS samples were stained by immunohistochemistry (IHC) for the following: tumor-associated immune cells (CD3, CD8, CD163, CD20), immune checkpoints (stimulatory: OX40, ICOS; inhibitory: PD-L1, LAG3, IDO1, PD1), and the adenosine pathway (CD73, CD39). Sections were reviewed for the presence of lymphoid aggregates (LA). Clinical data were retrospectively obtained for all samples. The Wilcoxon rank-sum and Kruskal-Wallis tests were used to compare distributions. Correlations between biomarkers were measured by Spearman correlation. Univariate and multivariate Cox models were used to identify biomarkers associated with overall survival (OS) and disease-free survival (DFS). Unsupervised clustering was performed, and Kaplan-Meier curves and log-rank tests used for comparison of OS and DFS between immune clusters.ResultsSamples analyzed (n=105) included 46 primary tumors, 34 local recurrences, and 25 metastases. LA were found in 23% (n=10/43), 17% (n=4/24), and 30% (n=7/23) of primary, recurrent, and metastatic samples, respectively. In primary UPS, CD73 expression was significantly higher after preoperative radiation therapy (p=0.009). CD39 expression was significantly correlated with PD1 expression (primary: p=0.002, recurrent: p=0.004, metastatic: p=0.001), PD-L1 expression (primary: p=0.009), and CD3+ cell densities (primary: p=0.016, recurrent: p=0.043, metastatic: p=0.028). In recurrent tumors, there was a strong correlation between CD39 and CD73 (p=0.015), and both were also correlated with CD163+ cell densities (CD39 p=0.013; CD73 p<0.001). In multivariate analyses, higher densities of CD3+ and CD8+ cells (Cox Hazard Ratio [HR]=0.33; p=0.010) were independently associated with OS (CD3+, HR=0.19, p<0.001; CD8+, HR= 0.33, p=0.010) and DFS (CD3+, HR=0.34, p=0.018; CD8+, HR=0.34, p= 0.014). Unsupervised clustering of IHC values revealed three immunologically distinct clusters: immune high, intermediate, and low. In primary tumors, these clusters were significantly associated with OS (log-rank p<0.0001) and DFS (p<0.001).ConclusionWe identified three immunologically distinct clusters of UPS Associated with OS and DFS. Our data support further investigations of combination anti-PD-1/PD-L1 and adenosine pathway inhibitors in UPS.

Dataset Information

A case of adrenal undifferentiated pleomorphic sarcoma with tertiary lymphoid structures responded to pembrolizumab.

Introduction

Case presentation

Conclusion

Publications

A case of adrenal undifferentiated pleomorphic sarcoma with tertiary lymphoid structures responded to pembrolizumab.

Similar Datasets

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