Clinical Characteristics Of Primary Malignant Melanoma Of The Lung

Objective:Primary malignant melanoma of the lung(PMML)is an extremely rare and highly malignant disease,and there is no uniform standard for diagnosis and treatment.The purpose of this study was to systematically evaluate the clinical characteristics and treatment decisions of patients with the disease and provide evidence to improve detection rates and prolong patient survival.Methods:A computer-based search was conducted to collect PMML related literature published between January 1,2000 and December 31,2000 in various databases at home and 2021.According to inclusion and exclusion criteria,2 investigators independently screened the literature,provided relevant information,assessed bias,and cross-checked the clinical characteristics of 134 patients for statistical analysis,determine sex,age,smoking history,location of disease,treatment,the overall median survival at 6 months,1 year,3 years and 5 years,the median disease-free progression survival and the postoperative disease-free survival were calculated.Document Management,data collection,and statistical analysis using note express,Excel 2010,and SPSS 26.0.The measures were presented as x±s or median(quartiles P25,P75),the counts were presented as percentages,the survival curves were plotted with the Kaplan-meier method,and the survival rates were compared with the Log-rank test,cox proportional hazards regression models were established for multivariate analysis of survival,and HR and 95%CI were calculated for all data with a test level ofα=0.05,P<0.05 as the difference was statistically significant.Results:Finally,125 articles were included,including 134 patients with PMML.Among them,50 were in Chinese literature and 75 in foreign literature.Among the 134 patients with PMML,55.22%were male and 44.78%were female.The ratio of male to female was 1.23:1.Conclusion:PMML is a common clinical manifestation of PMML.The highest age of onset was 90 years,the median age of onset was 58.5 years,and the average age was 58.22±15.34 years.Among 134 patients with PMML,25(18.66%)had a history of smoking and 26(19.40%)did not.Of the 134 patients with PMML,51(38.06%)had left lung,78(58.21%)had right lung,and 4(2.99%)had both lungs.There were 42 cases(31.34%)in upper lobe and 63 cases(47.01%)in lower lobe.Only 117 of 134 patients with PMML presented immunohistochemical results,of whom 31(26.50%)were Ki67 positive,98(83.76%)were S-100 positive,105(89.74%)were HMB-45 positive,and 47(40.17%)were Melanoma positive,1CA was negative in 14 cases(11.97%),CK was negative in 41 cases(35.04%),CK5/6 was negative in 12 cases(10.26%),CKAE1/AE3 was negative in 16 cases(13.68%),and P63 was negative in 16 cases(13.68%).Of the 134 patients with PMML,only 21 had genetic testing and 10 were positive,including 1 positive for MITF and INI1 genes and 1 positive for Brafv600e and PD-L1 genes,one case was positive for Brafv600e gene and EGFR gene,one case was positive for BCL-2 gene,one case was positive for NRAS(D54N),three cases were positive for Brafv600e gene,one case was positive for C-KIT gene and one case was positive for PD-L1 gene,the others were not detected or negative for gene mutation.47 cases(37.9%)were treated by operation alone,34 cases(27.42%)by Operation Plus comprehensive treatment,28 cases(22.58%)by comprehensive treatment,15 cases(12.10%)by no special treatment after diagnosis.Only 92 of 134 PMML patients achieved overall survival,with an overall median OS of 8.0 months(95%CI 3.097-12.903 months);The six-month,one-year,three-year and five-year survival rates were 58.5%,45.2%,31.3%and 22.8%,respectively.60 patients achieved disease-free progression survival,with a median PFS of 23.0 months(95%CI 4.930-41.070 months)and disease-free survival rates of 65.0%,52.6%,and 40.1%at 6 months,1 year,and 3 years,respectively.The median DFS was 28.0 months.The disease-free survival rates of six months,one year and three years were 67.0%,56.5%and 38.5%,respectively.The choice of treatment had a significant effect on prognosis(Log-rank x2=19.820,P=0.000).The results of the univariate analysis showed that,the risk factors of PMML were cough,expectoration,chest pain,pleural effusion,P63 negative,P63 gene mutation positive,choice of treatment,recurrence and metastasis in lung.Multivariate analysis showed that expectoration,chest pain,recurrence and metastasis were independent prognostic risk factors for PMML.Conclusion:(1)the incidence of PMML showed a fluctuating trend in recent 21 years.The most frequent site of PMML was in the lower lobe of right lung.(2)the most common clinical symptoms were cough,expectoration and chest pain.BRAF,NF1 and RAS mutations were the most common.(3)surgery improved survival,with a median postoperative DFS of 28.0 months in 47 patients,a median PFS of 23.0 months in 60 patients,and an overall median OS of 8.0 months in 92 patients.The mode of treatment and the metastasis of the lesion affect the prognosis.The results of this study can provide a basis for the diagnosis and treatment of this disease.