Mathematical Biosciences and Engineering

AIMS

http://www.aimspress.com/journal/MBE

MBE, 19(7): 7055-7075.

Published: 12 May 2022

Research article

Identification and validation of a tumor mutation burden-related signature combined with immune microenvironment infiltration in adrenocortical carcinoma

Yong Luo, Qingbiao Chen* and Jingbo Lin*

Department of Urology, the Second People’s Hospital of Foshan, Affiliated Foshan Hospital of Southern Medical University, Foshan 528000, China

* Correspondence: Email: elvislam0738@outlook.com, QingbiaoChen@outlook.com; Tel: +8615625093895; Fax: +86075788032009.

Abstract: Tumor mutation burden (TMB), an emerging molecular determinant, is accompanied by microsatellite instability and immune infiltrates in various malignancies. However, whether TMB is related to the prognosis or immune responsiveness of adrenocortical carcinoma (ACC) remains to be elucidated. This paper aims to investigate the impact of TMB on the prognosis and immune microenvironment infiltration in ACC. The somatic mutation data, gene expression profile, and corresponding clinicopathological information were retrieved from TCGA. The mutation landscape was summarized and visualized with the waterfall diagram. The ACC patients were divided into low and high TMB groups based on the median TMB value and differentially expressed genes (DEGs) between the two groups were identified. Diverse functional analyses were conducted to determine the functionality of the DEGs. The immune cell infiltration signatures were evaluated based on multiple algorithms. Eventually, a TMB Prognostic Signature (TMBPS) was established and its predictive accuracy for ACC was evaluated. Single nucleotide polymorphism and C > T were found to be more common than other missense mutations. In addition, lower TMB levels indicated improved survival outcomes and were correlated with younger age and earlier clinical stage. Functional analysis suggested that DEGs were primarily related to the cell cycle, DNA replication, and cancer progression. Additionally, significant differences in infiltration levels of activated CD4+ T cells, naive B cells, and activated NK cells were observed in two TMB groups. We also found that patients with higher TMBPS showed worse survival outcomes, which was validated in the Gene Expression Omnibus database. Our study systematically analyzed the mutation and identified a

TMBPS combined with immune microenvironment infiltration in ACC. It is expected that this paper can promote the development of ACC treatment strategies.

Keywords: tumor mutation burden; immune microenvironment infiltration; adrenocortical carcinoma; prognosis

1. Introduction

Adrenocortical carcinoma (ACC) is a rare endocrine malignancy. It afflicts one in every million people each year, and the median overall survival is merely 3-4 years [1]. For individuals with local or locally progressive illness, radical resection is presently the sole curative option [2]. However, the cumulative recurrence rate is still high even after surgery [3]. The most widely used TNM (tumor, lymph node, and metastasis) classification was not satisfactory owing to the lack of predominant genomic and molecular characteristics [4,5]. Therefore, identifying pivotal genomic determinants to enhance the predictive accuracy is important for ACC treatment and survival analysis.

In recent years, multiple acknowledged biomarkers for immune responsiveness, including microsatellite instability (MSI), tumor-infiltrating lymphocytes (TILs), and tumor mutation burden (TMB), especially the immune microenvironment infiltration and TMB, have shown great potential in the prediction of advanced or aggressive cancers [6-8]. TILs constitute the most crucial part of immunity since they can mediate the response of the immune system to chemotherapy, and they have revolutionized the treatments for many malignancies [7,9]. In addition, TILs have been confirmed to have a considerable impact on the development of tumors and clinical outcomes in various cancers, including lung cancer, urothelial carcinoma, and colorectal cancer [10-12]. It has been found that high mast cell infiltration indicates a better survival rate in ACC patients [13]. TMB represents the total number of somatic missense mutations in one megabase of genomic regions and has been determined as an emerging biomarker accompanied by immune infiltrates in various malignancies [14-16]. Notably, previous studies revealed that the TMB level could predict immunotherapy effect and survival outcomes across most cancer types [17-19]. High TMB in kidney renal clear cell carcinoma patients indicated an awfully poor survival outcome and inhibited immune cell infiltration [20]. Yan et al. constructed a prognostic signature by combining TMB and immune cell infiltrates to predict survival outcomes in cutaneous melanoma [21].

Increasing evidence revealed that polygenic mutation was related to the carcinogenesis and aggressive progression in ACC, indicating the predictive potential of TMB [1,22]. Mutations were transcribed and translated into novel antigens, which could be recognized and targeted by the tumor-immune system [23]. More mutations contribute to more antigens, making tumors more immunogenic and responsive to the immune system [17]. Nevertheless, merely about 20% of cancer patients could benefit from the immune strategy, which may be due to the involvement of TILs and the status of the tumor immune microenvironment (TIME) [24]. What is worse is that no biosignatures for evaluating the status of immune microenvironment infiltration in ACC have been found based on TMB level. Accordingly, it is of critical necessity to investigate the underlying molecular mechanism of immune infiltrates and the role of TMB based on an effective model containing multiple biomarkers for ACC.

In this research, we intended to explore the prognostic role of TMB with the combination of the

characteristics of immune infiltrates in an attempt to provide a distinctive perspective for the further development of ACC treatment strategies.

2. Materials and methods

2.1. Data acquisition and analysis

First of all, the somatic mutation data of 92 ACC patients were extracted from the Cancer Genome Atlas (TCGA, https://portal.gdc.cancer.gov/). After that, the “Masked Somatic Mutation” data was selected and processed by VarScan software. The Mutation Annotation Format of somatic mutation data was prepared and implemented by the “maftools” R package, which provided a wide range of analysis modules to execute a feature-rich customizable visualization [25]. Then, the gene expression data of 92 ACC samples in HTSeq-FPKM format were obtained. Moreover, we retrieved the corresponding clinical data of all samples. In order to facilitate downstream analysis, all Ensembl gene IDs were converted to gene symbols using an annotation GTF file obtained from GENCODE. Meanwhile, we downloaded transcriptome expression profiles and clinical information of GSE76019, GSE33371, and GSE10927 from the Gene Expression Omnibus (GEO) for validation. The probe matrix of the GSE76019 cohort, including 34 patients, was converted to a gene matrix using the GPL13158 platform. Additionally, the GSE33371 and GSE10927 datasets, containing 23 and 24 samples, respectively, were generated using the GPL570 platform.

2.2. TMB value calculation and prognostic evaluation

TMB refers to the total number of somatic missense mutations in a megabase of the genomic region, comprising base substitutions, insertions, and deletions. The Perl scripts were developed using the JAVA platform to specifically calculate the mutation frequency of all samples. The average length of the human exons is 38 megabase (Mb). Accordingly, the TMB estimate is equal to the total number of variants/38 for each sample. The calculation of TMB for 92 ACC patients was shown in Table S1. The ACC samples were divided into the low and high TMB groups. Then, the Kaplan-Meier analysis was conducted to compare the survival differences between two groups using the “survival” R package. We further assessed the relationship between TMB levels and clinical variables via Wilcoxon rank-sum test.

2.3. Differentially expressed gene (DEG) and functional enrichment analyses

The “Limma” package was selected for differential gene expression analysis without normalization between the two TMB groups with the screening criteria of | Fold change (FC) | > 1 [26]. The heatmap of all DEGs was analyzed and visualized utilizing the “Pheatmap” R package. Then, we used “org.Hs.eg.db” R package to convert all gene symbols into Entrez IDs for each DEG and implemented the Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway enrichment analysis using “enrichplot”, “ggplot2”, and “clusterProfiler” packages [27-29]. Additionally, gene set enrichment analysis (GSEA) was conducted by JAVA software. The “c2.cp.kegg.v7.2 symbols.gmt gene sets” obtained from the MSigDB database were chosen as the reference gene set [30]. False Discovery Rate (FDR) < 0.05 was considered as a threshold.

Homoplastically, the immune-related genes retrieved from the Immport were conducted by the “VennDiagram” package to select immune-related DEGs between two groups [31].

2.4. Survival analysis

A total of 48 immune-related DEGs were screened out and the prognostic values in the two TMB groups were further evaluated. Ultimately, six core genes associated with survival outcomes were identified by the Kaplan-Meier analysis. Additionally, the Cox regression analysis was applied to verify the prognostic potential of these core genes.

The CIBERSORT method was utilized to estimate the immune infractions, which was a newly developed tool that can convert gene expression data of each patient into immune infractions [32]. Detailed differential distributions of immune cells were analyzed and shown by the heatmap. Additionally, multiple algorithms containing EPIC, XCELL, MCPCOUNTER, QUANTISEQ, CIBERSORT-ABS, CIBERSORT, and TIMER were commanded to determine the differential abundances of immune infiltrates of core immune-related genes in ACC.

Figure 1. The landscape of mutation profiles in ACC.

Altered in 69 (75%) of 92 samples.

1776

0

17

0

TP53

CTNNB1

MUC16

MUC4

TTN

CNTNAP5

NF1

PKHD1

ASXL3

DST

HMCN1

PCDH15

PRKAR1A

ANK2

CCDC168

CMYA5

FBN2

MEN1

SVEP1

ADGRG4

AHNAK2 ATRX

DNAH5

EYS

FAT3

FAT4

GNAS

HUWE1

KMT2B

LAMA1

LRP1

OBSCN

TLN2

WDFY4

Frame_Shift_Del

Frame_Shift_Ins

Missense_Mutation

Splice_Site

In_Frame_Del

· Multi_Hit

Nonsense_Mutation

2.6. Establishment of TMB prognostic signature (TMBPS)

We established a novel TMBPS containing six core immune-related genes and assessed its predictive accuracy for all ACC patients. The formula of TMBPS was as follows: TMBPS = E (Bi x Expi) (I = 6). Moreover, the ROC curve was utilized to evaluate the predictive value of multiple clinical parameters in ACC.

2.7. Statistical analysis

The Wilcoxon rank-sum test was mainly used for comparisons between two groups based on the non-parametric hypothesis test. Kruskal-Wallis test was applied to analyze two or more categories. All statistical analyses were implemented using the R software (Version 4.1.1), and a P value less than 0.05 was considered to indicate statistically significant differences.

3. Results

3.1. The landscape of mutation profiles in ACC

The somatic mutation profiles in ACC were analyzed for a comprehensive landscape of mutation profiles. The percentages (≥5%) of the top 34 mutated genes and mutation types marked in different colors were shown in the waterfall plot (Figure 1). On the whole, missense mutation, comprising single nucleotide polymorphisms (SNP) and C > T, was the predominant mutation type (Figure 2A-C). The median number of variants in each sample was 21.5 (Figure 2D), and the variant classifications were represented by different colors (Figure 2E). The simultaneous and exclusive correlation of mutated genes was depicted in Figure 2F. In order to reveal the mutation difference in TMB levels, the landscapes in two TMB groups were compared, as illustrated in Figure 3. Moreover, the gene expression data obtained from TCGA, consisting of 92 ACC patients (32 males and 60 females), along with their clinicopathological features, were summarized in Table 1. The average age of these patients was 47.16 ± 16.30.

3.2. The correlation of TMB with clinical prognosis

The distributed patterns of clinical features of ACC in two TMB groups were depicted by the heatmap. TMB levels were closely associated with survival status and tumor stage (Figure 4A). Kaplan-Meier survival analysis suggested that ACC patients in the high TMB group tended to have a significantly worse survival outcome (Figure 4B,C). This finding is contrary to the result of previous studies [21,33]. Similarly, we assessed the correlation between clinical features and TMB values and found that a higher TMB level was correlated to older age and advanced tumor stage and AJCC-T stage (Figure 4D-F). Nevertheless, no significant correlation was observed between TMB level and gender, AJCC-N stage or AJCC-M stage (Figure 4G-I).

Figure 2. Summary of mutation profiles in ACC. (A) Variant classification; (B) Variant types; (C) SNV classification; (D) Variants in each sample; (E) Summary of variant classification; (F) The simultaneous and exclusive correlation of mutated genes.

A

Variant Classification

B Variant Type

C SNV Class

Missense_Mutation

T>G

250

Nonsense_Mutation

SNP

Frame_Shift_Del

T>A

672

Splice_Site

INS

T>C

989

Frame_Shift_Ins

In_Frame_Del

C>T

3758

Nonstop_Mutation

C>G

1123

In_Frame_Ins

DEL

Translation_Start_Site

C>A

3220

0

1000

2000

3000

4000

5000

6000

0

1000

2000

3000

4000

5000

6000

7000

0.00

0.25

0.50

0.75

1.00

D

Variants per sample

E

Variant Classification summary

1776

Median: 21.5

72

1184

48

592

24

0

0

CTNNB1 [14]

MUC16 [13]

MUC4 [13]

CNTNAP5 [ 8]

F

TP53 [17]

TTN [11]

NF1 [ 8]

PKHD1 [ 8]

ASXL3 [ 7]

DST [ 7]

HMCN1 [7]

PCDH15 [ 7]

PRKAR1A [ 7]

ANK2 [ 6]

CCDC168 [ 6]

CMYA5 [ 6]

FBN2 [ 6]

MEN1 [ 6]

SVEP1 [ 6]

FAT4 [ 5]

FAT4 [ 5]

SVEP1 [ 6]

MEN1 [6]

FBN2 [ 6]

CMYA5 [ 6]

CCDC168 [ 6]

ANK2 [ 6]

* P < 0.01

PRKAR1A [ 7]

P < 0.05

PCDH15 [ 7]

HMCN1 [7]

DST [ 7]

ASXL3 [ 7]

PKHD1 [ 8]

NF1 [ 8]

CNTNAP5 [ 8]

TTN [11]

-log10(P-value)

>3 (Co-occurance)

MUC4 [13]

2

1

MUC16 [13]

CTNNB1 [14]

-

TP53 [17]

2

> 3 (Mutually exclusive)

Figure 3. A comprehensive landscape comparison of mutation profiles between the low (A,C,E) and high (B,D,F) TMB groups.

A

B

Variant Classification

Variant Type

SNV Class

Variant Classification

Variant Type

SNV Class

Missense_Mutation

T>G

31

Missense_Mutation

Frame_Shift_Del

SNP

Nonsense_Mutation

SNP

T>G

219

Nonsense_Mutation

T>A

58

Frame_Shift_Del

T>A

614

Splice_Site

T>C

174

Splice_Site

T>C

815

Frame_Shift_Ins

INS

Frame_Shift_Ins

INS

C>T

188

in_Frame_Del

C>T

3300

In_Frame_Del

C>G

119

Nonstop_Mutation

In_Frame_Ins

C>G

1004

DEL

Translation_Start_Site

DEL

Nonstop_Mutation

C>A

132

In_Frame_Ins

C>A

3055

0-

100-

200

300

400

500

500

0-

100

200

300

400

500

600

0.00

0.25

0.50

0.75-

1.00

0

1000

2000

3000

4000

5000

6000

0

1000

2000

3000

4000

5000

6000

0.00

0.25-

0.50

0.75-

1.00

Variants per sample

Variant Classification summary

Variants per sample Median: 48

Variant Classification summary

22

Median: 14

21

1776-

142

14.

14

1184-

94

7.

7

592

47

0

0

m

AF

NJ

0.

0

D

C

CTNNB1 [7]

MEN1 [4]

MUC4 [4]

PRKARIA [4]

TP53 [4]

MUC 16 [3]

SVEP1 [3]

ASXL3 [2]

CDKL5 [2]

CNTNAP5 [2]

DNAJC13 [2]

FLG [2]

MXRA5 [2]

NF1 [2]

NLRP3 [2]

OPTN [2]

PKHD1 [2]

SLC4A11 [2]

SMC3 [2]

D

TP53 [13]

MUC 16 [10]

SI [2]

TTN [10]

MUC4 [ 9]

CTNNB1 [ 7]

CCDC168 [ 6]

CNTNAP5 [ 6]

DST [ 6]

HMCN1 [6]

NF1 [ 6]

PCDH15 [ 6]

PKHD1 [ 6]

ADGRG4 [ 5]

ANK2 [ 5]

CMYA5 [ 5]

EYS [ 5]

FAT3 [ 5]

FAT4 [ 5]

FBN2 [ 5]

OBSCN [5]

SMC3 [2]

OBSCN [ 5]

SLC4A11 [2]

FBN2 [ 5]

SI [2]

FAT4 [ 5]

. *

PKHD1 [2]

FAT3 [ 5]

OPTN [2]

EYS [ 5]

NLRP3 [2]

CMYA5 [ 5]

NF1 [2]

*P <0.01

ANK2 [ 5]

* P <0.01

MXRA5 [2]

P < 0.05

ADGRG4 [ 5]

. P < 0.05

FLG [2]

PKHD1 [ 6]

DNAJC13 [2]

PCDH15 [ 6]

CNTNAP5 [2]

NF1 [6]

CDKL5 [2]

HMCN1 [ 6]

ASXL3 [2]

DST [ 6]

SVEP1 [3]

CNTNAP5 [ 6]

MUC16 [3]

TP53 [4]

-log10(P-value)

>3 (Co-occurance)

CCDC168 [ 6]

-log10(P-value)

>3 (Co-occurance)

2

CTNNB1 [ 7]

PRKARTA [4]

2

1

MUC4 [ 9]

1

MUC4 [4]

0

TTN [10]

0

MEN1 [4]

1

MUC16 [10]

1

CTNNB1 [7]

2

TP53 [13]

2

> 3 (Mutually exclusive)

> 3 (Mutually exclusive)

E

Altered in 34 (70.83%) of 48 samples.

F

Altered in 41 (93.18%) of 44 samples.

22.

1776

0

7

0.

CTNNB1

15%

0

0

13

TP53

MEN1

MUC16

30%

MUC4

PRKARLG

8%

TTN

2375

MUCA

8%

CINNAY

R

MUC16

CCDC168

16%

6%

SVEPT A9X13

6% 4%

CNTNAPS

14%

DST

6

14% 14%%

COKE5

4%

HM

EN1

C

CNTNAP5

4%

14%

DNAJC1

4%

NF

PCOH15

14% 14%

LG

16

MXRAS

4%

ADGRG

NF1

4% 4%

AHNAK2 ANK2

11%

NLRP3

11%

ANKE

PKHD1

S

4

CMYA5

SLC4A1

2

11%

2

Q

SPATA31E1

FATA

1

2

HUWE1

ABCCY

ABER

KM128

ACACB

OBSCN

11%

LN2

COMOS

11%

GANZª

SLC25437

ZNRF3

2%

SPTAT

9%

2%

WDFY4

9%

. Missense_Mutation

In_Frame_Del

· Missense_Mutation

. Frame_Shift_Del

Frame_Shift_Del

Nonsense_Mutation

Splice_Site

- Frame_Shift_Ins

= Multi_H4

In_Frame_Del

Nonsense_Mutation = Multi_Hit

Splice_Site

” Frame_Shift_Ins

Table 1. Clinicopathological information of 92 ACC patients.
variblesNumber (%)
Status
Alive58 (63.04)
Dead34 (36.96)
Age (year)47.16± 16.30
Gender
Female60 (65.22)
Male32 (34.78)
AJCC-T
19 (9.78)
249 (53.26)
311 (11.96)
421 (22.83)
Unknown2 (2.17)
AJCC-N
080 (86.96)
110 (10.87)
Unknown2 (2.17)
AJCC-M
072 (78.26)
118 (19.57)
Unknown2 (2.17)
Stage
9 (9.78)
II44 (47.83)
III19 (20.65)
IV18 (19.57)
Unknown2 (2.17)
Figure 4. The correlation of TMB with clinical prognosis. (A) The distributed patterns of clinical features between two TMB groups. (B,C) Low TMB indicated a favorable prognosis. (D-F) Higher TMB levels were correlated to older age and advanced tumor stage and AJCC-T stage. (G-I) No significant correlation was observed between TMB and gender, AJCC-N stage, or AJCC-M stage.

A

TMB

Survival ***

Age

Gender

Stage*

TMB

Survival ***

Age

Gender

Stage*

high

Alive

50

Female

Stage I

low

Dead

>50

Male

Stage II

Stage III

Stage IV

unknow

B

TMB

+ High +

Low

Overall Survival (OS)

TMB Progression Free Survival (PFS) )

TMB

+ High * Low

1.00-

1.00-

0.75

0.75

0.50

0.50

0.25

p<0.001

0.25

p<0.001

0.00

0.00

0

1

2

3

4

5

6

7

8

9

10

11

12

0

1

2

3

4

5

6

7

8

9

10

11

12

TMB

High LOW

44

48

40

46

26

39

18 32

Time(years)

9

27

7

22

4

15

2

11

0

8

9

0

4

0 2

Time(years)

0

2

High- Low

44

48

21

42

12

26

4

4

34

6

20

18

2

12

1

8

0

0

1

2

3

4

5

6

7

8

9

10

11

12

6

0 5

0 3

0

2

0

2

Time(years)

0

1

2

3

4

5

Time(years)

6

7

8

9

10

11

12

D

Age

E

Stage

F

T

4

10

10

*

*

3

8

8

TMB

TMB

6

TMB

6

2

4

4

1

2

2

0

0

0

50

>50

I

II

III

IV

T1

T2

T3

T4

G

Gender

H

N

M

3

3

10

..

8

TMB

2

TMB

2

TMB

6

1

1

4

2

0

0

0

Female

Male

NO

N1

MO

M1

3.3. Identification of DEGs correlated to TMB

The heatmap showed that the levels of DEGs were generally lower in the low TMB group (Figure 5A). A total of 859 DEGs were determined by differential analysis for the following investigation (Table S2). To elucidate the potential biological functionality and pathways of DEGs, GO and KEGG enrichment analyses were performed. Nuclear division, DNA helicase activity, and microtubule binding were enriched in the GO category (Figure 5C; Table S3). Additionally, KEGG pathway enrichment analysis and the GSEA revealed that cell cycle, DNA replication, p53 signaling pathway, and pathways in cancer were enriched (Figure 5D,E; Tables S4 and S5). Owing to the fact that TMB was associated with the immune microenvironment, 48 immune-related genes were determined for the next analysis (Figure 5B; Table 2).

Figure 5. Identification of DEGs correlated to TMB. (A) Heatmap of DEGs; (B) Identification of TMB-related immune genes; (C,D) GO and KEGG enrichment analysis of DEGs; (E) GSEA results of DEGs.

A

Type

10 Type

B

KLK1

high

PTGDS

48

L2ORB

low

ZGLP1

ADMIR

5

SCGN

AP000851.2

POU4F1

HOXD13

Immune

FEZF1

0

FOXA2

PLAC1

|logFC|>1

MMP

DLX

SHOX2

-5

GAPLINC

WNT10B

RGS20

MUC1

MMP13

LINC00460

1763

811

AC025575.2

A

SONT

13057

AL 139231.1

UGT1A7

TMEM72

UGT2A3

DIO3

TNR

ADHS

IGLVB-61

KRT19

RAGALT2

CD244

LTF

SMCO3

PYDC1

KCNS1

C

organelle fission

D

nuclear division

chromosome segregation

Cell cycle

mtobc nuclear division

nuclear chromosome segregation

sister chromatid segregation

.

Neuroactive ligand-receptor interaction

mitotic sister chromatid segregation

spindle organization

regulation of chromosome segregation

Oocyte meiosis

mitotic spindle organization

Count

qvalue

chromosomal region

20

Drug metabolism - cytochrome P450

spindle

0.005

condensed chromosome

40

chromosome, centromeric region

60

Progesterone-mediated oocyte maturation

0 010

condensed chromosome, centromeric region

kinetochore

8

qvalue

condensed chromosome kinetochore

mitotic spindle

Drug metabolism = other enzymes

54-04

Count

condensed nuclear chromosome

condensed nuclear chromosome, centromeric region

20-04

55

p53 signaling pathway

15

catalytic activity, acting on DNA

3e-04

20

microtubule binding

DNA helicase activity

Metabolism of xenobiotics by cytochrome P450

microtubule motor activity

3’-5’ DNA

single-stranded DNA-dependent ATP-dependent DNA helicase activity

6

Fanconi anemia pathway

single-stranded DNA-dependent ATPase activity

ATP-dependent DNA helicase activity

ATP-dependent helicase activity

..

DNA replication

purine NTP-dependent helicase activity

.

E

0.025 0.050 0.075 0.100

0.04

0.06

0.08

0.10

GeneRatio

GeneRatio

Enrichment plot: KEGQ_CELL_CYCLE

Enrichment plot: KEGG_DNA_REPLICATION

Enrichment plot: KEGG_PATHWAYS_IN_CANCER

Enrichment plot: KEQQ_P53_SIGNALING_PATHWAY

0.5

T

7

M

A

Enrichment score (ES)

6

4

$ .

UJ

3

2

>

12

11

2

3

.

1

LA

0

#

..

#

..

1.

..

·

1

5

5

.

·

-

-

.

-

#

-

-

-

#

-

-

-

Rank in Ordered Dwamet

Rank in Ordered Ditate

Ranking maitre women

Table 2. The top 20 differential immune genes between two TMB groups.
Gene IDLowHighlogFCpValueFDR
BMP15.11313.0861.3560.0000.001
C3197.18653.931-1.8700.0000.001
SLC40A183.37525.418-1.7140.0000.001
BIRC52.3309.7822.0700.0000.001
ARTN0.2740.6271.1940.0000.002
LTBP12.8467.7191.4390.0000.002
NR4A32.3417.0891.5990.0000.003
HGF2.1300.604-1.8190.0000.005
PDIA20.1820.4181.1970.0000.005
QRFP0.2040.5371.3930.0000.005
CCL230.3620.165-1.1360.0000.006
TMSB10659.3041438.0881.1250.0000.007
XCL20.5040.188-1.4240.0010.008
IL20RB0.2592.6113.3330.0010.008
CYSLTR10.2820.132-1.0990.0010.008
BMP8B0.1650.3641.1390.0010.009
PTGER32.8080.487-2.5270.0010.010
LTF0.7550.084-3.1620.0010.010
PTGFR3.0121.084-1.4740.0010.011
CTF14.7901.875-1.3530.0010.011
Figure 6. The detailed workflow of screening out six core TMB-related immune genes.

TCGA-ACC (92 samples)

1

Retrieve somatic mutation data

Obtain gene expression data

!

!

Screen out differentially expressed genes between two TMB groups (n=859)

Download immune related genes from Immport

TMB-related immune genes(n=48)

Survival Kaplan-Meier and Cox regression analysis

6 core TMB-related immune genes (TMSB15A, MMP9, BIRC5, LTBP1, CCL14, PTGFR)

3.4. Identification of core genes and their relation to the immune microenvironment

Survival analysis and Cox regression analysis were performed to identify core genes that were significantly correlated with survival outcomes. Six core genes were identified and the detailed workflow of screening was shown in Figure 6. We found ACC patients with higher expression levels of TMSB15A, MMP9, BIRC5, and LTBP1 had worse survival outcomes (Figure 7A-D), while patients with high expression levels of CCL14 and PTGFR had better prognosis (Figure 7E,F). The results of Cox regression analysis also indicated that the six core prognostic genes were significantly related to the survival outcomes in ACC patients (Figure 7G,H). To further assess the underlying relation of these core genes with immune infiltrates in ACC, multiple software was applied. It was found that the expression of the six core genes was robustly associated with the abundance of immune cell subtypes (Figure 8).

Figure 7. Kaplan-Meier analysis and Cox regression analysis of six core prognostic genes. (A-D) Higher expression levels of TMSB15A, MMP9, BIRC5, and LTBP1 indicated worse survival outcomes; (E,F) Higher expression levels of CCL14 and PTGFR suggested a better prognosis. (G,H) The forest maps of the hazard ratio and P value of the six core prognostic genes.

A

TMSB15A High Low

B

MMP9 High Low

C

BIRCS -High Low

Survival probability

1.00

Survival probability

1.00

Survival probability

1.00

0.75

0.75

0.75

0.50

0.50

0.50

0.25

p=0.001

0.25

p=0.003

0.25

p<0.001

0.00

0.00

0.00

0

C

4

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1

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TMSB15A

Time(years)

Time(years)

Time(years)

MMP9

BIRC5

17

0

A

a

O

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2

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1

Low

1 8*

No

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LTBP1

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Low

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CCL14 High Low

F

PTGFR_ High Low

Survival probability

1.004

Survival probability

1.004

Survival probability

1.004

0.75

0.75

0.75

0.50

0.50

0.50

0.25

p<0.001

0.25

p=0.004

0.25

p<0.001

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0.00

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Time(years)

Time(years)

Time(years)

LTBP1

CCL14

PTGFR

High

5

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4

L

IN

High

Low

3

37

10

4

a

3

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High Low

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27

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Time(years)

12

Time(years)

Time(years)

G

pvalue

Hazard ratio

H

pvalue

Hazard ratio

TMSB15A 0.001

1.532(1.286-1.824)

TMSB15A 0.001

1.409(1.163-1.705)

MMP9

0.001

1.033(1.018-1.048)

MMP9

0.032

1.027(1.002-1.052)

BIRC5

0.001

1.180(1.123-1.240)

BIRC5

0.021

1.079(1.012-1.150)

CCL14

0.023

0.394(0.177-0.880)

CCL14

0.130

0.450(0.160-1.266)

LTBP1

0.001

1.106(1.060-1.155)

LTBP1

0.002

1.115(1.041-1.195)

PTGFR

0.045

0.815(0.668-0.996)

PTGFR

0.096

0.868(0.735-1.025)

0.0

0.5

1.0

1.5

0.0

0.5

1.0

1.5

Hazard ratio

Hazard ratio

Figure 8. The relation between expression levels of the six core prognostic genes and immune cell subtypes. (A-F) Tumor-infiltrating immune cell analysis of TMSB15A, MMP9, BIRC5, LTBP1, CCL14, and PTGFR.

A

TMSB15A

B

MMP9

TMS815A

TMSBISA

MMPS

MMPP

group

group

-

0

Neutrophil_TIMER

20

Methods

0 Methods

ABS

CRERSORT-ARS

XCELL

MCPCOUNTER

Concor associatod foroblast_XGELL

Macrophago MI_XCELL

T cell CD4+ Th1_XCELL

Menwayto_XCELL

T cel CD4+ Th2_XCELL

· cul naive_XCELL

Youll CD44 THQ_XCELL

Cancer associated fibroblast_EPIC

T call CD4+ EPIC

Methods

Methods

C

BIRC5

D

LTBP1

BIRCS

BIRCS 25

LTBP1

LTBPS

group

group

20

5

5

group

group

Methods

nign

Methods

MAPCOUNTER

QUANTSEO

MCPCOUNTER

XCELL

-

Methods

Methoes

E

CCL14

F

PTGFR

CCL14

PTOFR

PIGPR

group

12

Macrophage_TIMER

group

B cell naive_CIBERSORT

0

group

hugh

CRERSORT


CIBERSORT-ABS

COUNTER

EPIC

XCELL

EPIC

B cel_QUANTISEQ

Macrophage M2_QUANTISEQ

Myeloid dendrite cell_QUANTISEQ

T cell CDB ._ MOPCOUNTER

Myeloid dendrite cell MOPCOUNTER

Toel CD4+ eflector memory_XCELL

Marrighaga M2_XCSLL

Monocyte_XCELL

Toel NK XCELL

T cel CD4+ Th1_XCELL

T cell CD4+ TRZ_XCELL

B cel_EPIC

Methods

Methods

3.5. Relationship between TMB and immune cells in ACC

We evaluated the proportions of 22 immune cells in all ACC samples based on the CIBERSORT method with P < 0.05 (Table S6), which were visualized in the heatmap and box plot (Figure 9A,B). We could find memory resting CD4+ T cells, CD8+ T cells, and M2 macrophage are the predominant immune cell types, while activated dendritic cells and M0 macrophage showed high abundance in the high TMB group. The violin diagram showed that infiltration levels of naive B cells and activated NK cells were relatively higher in the low TMB group, while activated memory CD4+ T cells showed a higher expression level in the high TMB group (Figure 9C).

Figure 9. Relationship between TMB and immune cells. (A,B) The proportions of 22 diverse immune cell types in all ACC samples were visualized in the heatmap and box plot. (C) The correlation of TMB levels with infiltration levels of diverse immune cell subtypes.

A

type

type

T cells CD8

0.6

high TMB

Monocytes

Mast cells resting

0.5

low TMB

T cells CD4 naive

0.4

B cells naive

NK cells activated

0.3

Macrophages M1

NK cells resting

0.2

Eosinophils

0.1

Plasma cells

T cells follicular helper

0

Mast cells activated

Dendritic cells resting

T cells CD4 memory activated

T cells regulatory (Tregs)

Neutrophils

B cells memory

T cells gamma delta

Macrophages MO

Dendritic cells activated

T cells CD4 memory resting

Macrophages M2

DrSV-LO-VDOLL

B

C

100%

. B cells naive

. B cells memory

0.6

Low TMB

Relative Percent

. Plasma cells

80%

. T cells CDB

. T cells CD4 naive

0.5

High TMB

resting

. T cells CD4 memory activated

60%

follicular helper

50.4.

+ armory (Tregs)

8

. NK cells resting

SU

0.3

40%

. NK cells activated

LL

-

. Monocytes

-

Macrophages MO

. M1

0.2

20%

· Macrophages M2

. Dendritic cells resting

. Dendritic cells activated

0.1

calls resting

-

0%

Mast cells activated

0.0

C

TCGA-OR-ASLA

TCGA-OR-ASKZ

TCGA-OR-ASKT

TCGA-OR-A5J8

TCGA-PK-ASHA

TCGA-P6-A50G

TCGA-OR-A5JO

TCGA-OR-A5.28

TCGA-OR-ASJI

TCGA-PA-ASYG

TCGA-OR-A5LX

Eosinophils

. Neutrophils

8 con nove

B oets memory

Plaseria Oil

Youts COS

T cells CDA nad

T cells CD4 memory resting

Tollx CD4 memory activated

T ods folicular helyet

T collo enguastory (Theqs)

T cats gamma ouila

NK cels sesilog

SK outs actives

Monocytes

Macrophages Mo

Macrophages ves

Macrophages M2

Derdrac cols ressing

Dendroc colls activate

Mast ants toneg

Mest cats actesed

3.6. Construction and assessment of TMBPS for ACC patients

In view of the relationship between variants of TMB-related immune genes with poor prognosis and higher immune infiltration, we established a TMBPS comprising the above six core genes and assessed its predictive efficiency for ACC. Based on the Cox regression model, TMBPS was calculated as follows: TMBPS = (0.343290 x TMSB15A + 0.026519 x MMP9 + 0.075941 x BIRC5 + 0.109634 × LTBP1 - 0.798708 × CCL14 - 0.142566 x PTGFR) (Table S7). The relations among survival status, prognostic scores, and core gene expression based on the TMBPS were shown in Figure 10A. ROC curve was introduced and the robust predictive accuracy of TMBPS was illustrated, with AUC = 0.897, higher than that of any other clinical parameters (Figure 10B). In addition, survival analysis revealed that ACC patients with high TMBPS suffered from an unfavorable prognosis (Figure 10C). Similarly, the Cox analysis of the above clinical factors was employed to verify that TMBPS could be an independent and robust prognostic predictor for ACC (Figure 10D,E). In order to validate our results, GSE76019, GSE33371, and GSE10927 derived from the GEO database were applied to assess the effectiveness of ACC prediction by TMBPS (Figure 11).

Figure 10. Construction and assessment of TMBPS for ACC patients. (A) The relations among survival status, prognostic scores, and core gene expression; (B) ROC curve of the TMBPS. (C) Survival analysis curve of the TMBPS. (D,E) The Cox regression analysis of the TMBPS.

A

B

C

0

High TMBPS

0

TMBPS High - Low

TMBPS

. Low TMBPS

1.00

4 6

Survival time (years)

True positive rate

0,8

Survival probability

0.75

N

0.6

0.50

0

0

20

40

60

80

0.4

Patients (increasing TMBPS)

TMBPS (AUC=0.897)

0.2

Age (AUČ=0,607)

0.25

p<0.001

Gender (AUC=0.512)

0 2 4 6 8 10 12

Stage (AUC=0.611)

Dead

T (AUC-0.600)

0.00

0.0

N (AUC=0.438)

Alive

M (AUC=0.503)

7

TMBPS

0

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9

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Time(years)

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High

39

35

22

14

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LOW

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1

1 7

1

1

1 1

1

40

40

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30

25

20

14

10

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3

1

False positive rate

6

1

3

1

A

K

A

7

W

6

12

Time(years)

10 11

D

E

0

20

Patients (increasing TMBPS)

40

60

80

pvalue Hazard ratio

pvalue Hazard ratio

type

type

age

0.328 1.012(0.988-1.038)

age

0.418 1.013(0.982-1.045)

PTGFR

TMSB15A

gender 0.972 0.986(0.451-2.154)

gender 0.790 1.126(0.470-2.701)

CCL14

MMP9

stage

<0.001 2.914(1.860-4.565)

stage

<0.0012.694(1.652-4.394)

BIRC5

LTBP1

TMBPS <0.001 1.034(1.019-1.049)

TMBPS 0.002 1.024(1.009-1.040)

0

1

2

3

4

0

1

2

3

NA

4

Hazard ratio

Hazard ratio

4. Discussion

Tumorigenesis is the result of the accumulation of genetic alterations in the DNA interacting with immune infiltrates [34]. It has been found that TIME paves a novel way for tumor progression and immune response. TILs have been determined as an emerging indicator that affects the prognosis and therapeutic response in various human cancers [35-37]. Although patients with locally progressive ACC generally have a high recurrence rate after radical resection, PD-L1 inhibitors could reactivate dormant TILs and a high PD-L1 expression level indicated a longer postoperative survival, which represented a promising strategy for ACC [38]. Nevertheless, the therapeutic strategies of ACC are still limited and effective biomarkers for immune responses are lacking.

The effective biomarkers can help to identify patients whose immune system would respond so as to avoid waste of money and severe toxicities for non-responders. TMB has proven to be a novel biomarker to predict immune responses in various malignancies, such as prostate adenocarcinoma, urothelial carcinoma of the bladder, and lung cancer [15,39-40]. Luo et al. found that a higher TMB level indicated worse BCR-free survival and TMB was associated with the immune infiltrates in prostate cancer [15]. Jiang et al. showed that a combination of TMB, immune infiltrates, and PD-L1 expression is feasible for the prediction of early-stage lung squamous cell carcinoma [41]. Nevertheless, there were few discussions about the role of TMB and its underlying connection to immune infiltrates in ACC.

In this research, we comprehensively analyzed and visualized the landscape of mutation profiles of ACC patients. It was found that 75% of ACC patients showed various mutation forms, with missense mutations comprised of SNP and C > T mutations accounting for the most. The two most common mutated genes were TP53 and CTNNB1. It was shown that TP53 was a tumor suppressor

protein responding to different cellular stresses through regulating the expression of target genes, inducing cell cycle capture, apoptosis, senescence, and metabolism alterations [42,43]. CTNNB1 was a protein regulating cell growth and adhesion between cells [44,45]. Analysis of the association between TMB level and survival outcomes indicated that ACC patients with high TMB suffered from a worse prognosis. The results demonstrated that higher TMB levels were closely associated with older age and advanced tumor stage and AJCC-T stage. Accordingly, TMB is an effective predictor that could provide valuable information for immunotherapy in various kinds of cancers, including ACC [46,47].

Figure 11. Dataset GSE76019, GSE33371, and GSE10927 were used to validate the effectiveness of ACC prediction by TMBPS. (A-C) The relation among survival status, prognostic scores, and core gene expression. (D-F) Kaplan-Meier survival analyses. (G-I) ROC analysis curves for three cohorts.

A

GSE76019

GSE33371

GSE10927

TMBPS

0 2 4 6 8 10

B

0 2 4 6 8 10

C

0 2 4 6 8 10

· High TMBPS

· High TMBPS

High TMBPS

. Low TMBPS

TMBPS

. Low TMBPS

TMBPS

. Low TMBPS

0

5

10

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35

5

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20

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Patients (increasing TMBPS)

01234567

Survival time (years)

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Survival time (years)

Patients (increasing TMBPS)

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Dead

Dead

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Patients (increasing TMBPS)

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Patients (increasing TMBPS)

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15

20

type

35 type

=

type

2.4 type

type

24 type

1.

Pensa

3

PTOFR

22

2

MMpp

22

-

LTBPT

2

25

COL14

1.8

TBP1

16

18 18

PTGFR

2

MMP9

MS815A

MMPS

LTBPT

L

BIRCS

I

TMS815A

TMS815A

PTOFR

BIRCS

BIRCS

COL54

-

D

TMBPS

High

Low

E

TMBPS High Low

F

TMBPS

High

Low

Survival probability

1.00

Survival probability

1.00

Survival probability

1.00

0.75

0.75

0.75

0.50

0.50

0.50

0.25

p=0.008

0.25

p

0

001

0.25

P<0,001

0.00

0.00

0.00

TMBPS

0

1

2

3

4

5

6

7

8

TMBPS

0

1

2

3

4

5

E

1

8

9

10

11

12

13

14

15

TMBPS

0

#

2

3

4

5

5

1

8

9

10

11

12

Time(years)

Time(years)

Time(years)

ligh

SE

12

8

a

5

8

2

0

High

2

3

0

0

LOW

11

1

0

0

0

0

0

0

0

0

0

0

0

0

0

HAigh

LOW

12 12

4

1

0

5

0

0

0

0

0

12

0

0 1

0 1

0

LOW

15

11

1

1

0

12

8

7

7

5

4

3

2

2

2

2

1

1

1

11

9

5

3

2

2

2

1

1

0

1

2

3

4

5

6

7

À

0

1

2

3

4

5

6

7

8

0

10

11

12

13

14

15

0

1

2

3

4

5

6

7

8

9

10

11

12

Time(years)

Time(years)

Time(years)

G

ROC curve(AUC=0.735)

I

ROC curve(AUC=0.976)

ROC curve(AUC=0.948)

0

1.0

0.

True positive rate

0.8

True positive rate

0.8

True positive rate

0,8

0.6

0.6

06

0.4

0.4

0,4

0.2

0.2

0,2

0.0

0.0

0,0

0.0

0.2

0.4

0.6

0.8

1.0

0.0

0,2

0.4

0.6

0.8

0.6

0.8

False positive rate

1.0

0.0

0.4

1.0

False positive rate

0.2

False positive rate

To further elucidate the potential biological functionality and mechanisms of 859 DEGs between two TMB groups, we conducted differential analysis to single out TMB-related DEGs. GO

enrichment analysis showed that DEGs were primarily involved in cell mitosis. KEGG pathway enrichment analysis and GSEA revealed that DEGs were mainly correlated with cancer progression and immune cell response to tumors, such as DNA replication, cell mitosis, and cell cycle. DNA replication and cell mitosis were the fundamental biological processes in which dysregulation could cause genome instability [48]. The accumulative errors of DNA replication and cell mitosis could cause tumorigenesis, including ACC [49]. These functions were also correlated with the occurrence of cancer, the influence of the tumor microenvironment, and the differentiation and activation of immune cells. Given that the physiological process of cell mitosis demands the homeostasis of the cell cycle, its dysregulation would bring about the disorder of cell growth and the occurrence of cancer [50].

Survival analysis was performed and six core prognostic TMB-related immune genes in ACC were identified. CCL14 was a chemokine inducing the activation of immune cells and a potential prognostic biomarker and tumor suppressor via regulating the cell cycle and promoting apoptosis in hepatocellular carcinoma [51]. BIRC5, as an immune-related gene, was greatly related to multiple immune cell infiltrates in diverse cancers and could inhibit apoptosis and facilitate cell proliferation [52]. We found BIRC5 was greatly related to abundant immune cell subtypes. On the whole, expression levels of these core immune genes were correlated to the abundance of immune infiltrates, including neutrophil cells, macrophage, cancer-associated fibroblast, B cells, and T cells.

We also analyzed the correlation between TMB level and immune infiltrates to reflect the status of the TIME in ACC. In this study, infiltration levels of naive B cells and activated NK cells in the low TMB group were higher, while activated memory CD4+ T cells showed a higher infiltration level in the high TMB group. The possible reason is that the increased amount of neoantigens caused by genomic mutation promoted the immune activation and recognition of memory CD4+ T cells. It has been shown that CD4+ TILs were extraordinarily associated with antigen processing, and the infiltration of memory T cells was involved in the prognosis of multiple malignancies [53-54]. Activated memory CD4+ T cells, which were stimulated by the proliferation of inactive ones, were able to release inflammatory cytokines, thereby promoting tumor growth and accelerating tumor metastasis [55]. These findings verified that immune cells played an extremely important role in antitumor immunity in ACC patients. The identification of effective immunological biomarkers can help to avoid immunotherapy resistance and improve the therapeutic effect, and would become a novel promising therapeutic strategy for ACC patients.

Finally, a new risk score signature containing six core genes was established and its predictive value for ACC was assessed with AUC. It was found that ACC patients with high TMBPS had a poor prognosis than those with low TMBPS. Our results were validated in the other three independent ACC patient cohorts retrieved from the GEO database. Therefore, we confirmed the superiority and effectiveness of our risk signature for the diagnosis and treatment of ACC, and it was expected to be applied in clinical practice in the future.

5. Conclusions

In summary, we revealed a systematic landscape of TMB and identified a TMBPS combined with immune microenvironment infiltration in ACC. This paper will provide a reference for the development of ACC treatment strategies.

Acknowledgments

We are grateful to Guangdong Medical Science and Technology Research Fund Project (B2022332, B2020127) granted to Qingbiao Chen and Foshan Science and Technology Innovation Project (2020001005794) granted to Jingbo Lin.

Conflict of interest

The authors declare no competing interest.

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