Hematological indicators of chronic systemic inflammation are significant biomarkers for gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs). We performed a systematic review and meta-analysis to assess the impact of certain factors on the overall survival (OS), progression-free survival (PFS), and disease-free survival (DFS) of patients with GEP-NENs. These factors include the neutrophil/lymphocyte ratio (NLR), platelet/lymphocyte ratio (PLR), lymphocyte/monocyte ratio (LMR), and C-reactive protein (CRP) levels. After searching the Medline, Embase, and Cochrane Library databases from January 1, 2000 to October 20, 2022 and the American Society of Clinical Oncology conference proceedings from January 1, 2017, hazard ratios (HRs) and 95% confidence intervals (CIs) were extracted. Subgroup analyses were conducted to identify the origins of heterogeneity and examine the impact of factor grouping. The effects of the cut-off values and sample size were assessed by meta-regression. The results revealed that higher NLRs, PLRs, and CRP levels were associated with shorter OS (HR = 2.09, 95% CI = 1.55-2.8; HR = 1.79, 95% CI = 1.40-2.28; and HR = 2.88, 95% CI = 2.09-3.95, respectively; all p < 0.001). Higher NLRs and lower LMRs were associated with shorter DFS (HR = 3.34, 95% CI = 2.11-5.29 and HR = 2.71, 95% CI = 2.27-3.24, respectively; both p < 0.001). Higher PLRs and CRP levels were correlated with shorter PFS (HR = 3.48, 95% CI = 1.34-9.03, p = 0.01 and HR = 3.14, 95% CI = 1.63-6.08, p = 0.001). As demonstrated in the research, hematological indicators of systemic inflammation are promising biomarkers for GEP-NEN assessment.
Ectopic adrenocorticotropic hormone syndrome (EAS) is a rare condition caused by pancreatic neuroendocrine tumors (p-NETs). The severe hypercortisolemia that characterizes EAS is associated with a poor prognosis and survival. Mitotane is the only adrenolytic drug approved by the Food and Drug Administration and is often used to treat adrenocortical carcinoma. Combination therapy with mitotane and other adrenal steroidogenesis inhibitors is common for patients with Cushing’s syndrome (CS). Here, we describe three patients who developed EAS secondary to the liver metastasis of p-NETs. All three rapidly developed hypercortisolemia but no typical features of CS. They underwent anti-tumor and mitotane therapy, which rapidly reduced their blood cortisol concentrations and ameliorated their symptoms. Their hypercortisolemia was controlled long term using a low dose of mitotane. The principal adverse effects were a slight loss of appetite and occasional dizziness, and there were no severe adverse effects. Importantly, even when the tumor progressed, the patients’ circulating cortisol concentrations remained within the normal range. In summary, the present case series suggests that mitotane could be used to treat hypercortisolemia in patients with EAS caused by advanced p-NETs, in the absence of significant adverse effects.
Neuroendocrine neoplasms (NENs) are a group of rare tumors with strong heterogeneity. Among these tumors, well‐differentiated tumors can have a relatively good survival prognosis, while some tumors can have strong malignant potential and lead to increased mortality. Traditional Chinese medicine (TCM), which has a long history of thousands of years, has been widely used to treat tumors due to its unique advantages, such as economy, efficacy and few side effects. In recent years, the role of TCM in the treatment of NENs has gradually emerged. It can not only help prevent tumor recurrence and reduce the side effects of radiotherapy and chemotherapy but also relieve symptoms and improve quality of life. Various clinical studies have indicated that TCM has achieved good curative effects at different stages of treatment. We summarized the last 10 years of research progress on the clinical application of TCM in the treatment of NENs from four points of entry, aiming to provide a reference for the treatment of NENs.
The genetic characteristics of rectal neuroendocrine tumors (R-NETs) were poorly understood. Depicting the genetic characteristics may provide a biological basis for prognosis prediction and novel treatment development. Tissues of 18 R-NET patients were analyzed using whole-exome sequencing. The median tumor mutation burden (TMB) and microsatellite instability (MSI) were 1.15 Muts/MB (range, 0.03-23.28) and 0.36 (range, 0.00-10.97), respectively. Genes involved in P53 signaling, PI3K-AKT signaling, DNA damage repair, WNT signaling, etc. were frequently altered. Higher TMB (P = 0.078), higher CNV (P = 0.110), somatic mutation of CCDC168 (P = 0.049), HMCN1 (P = 0.040), MYO10 (P = 0.007), and amplification of ZC3H13 (P < 0.001) were associated with shorter OS. Potentially targetable gene alterations (PTGAs) were seen in 72% of the patients. FGFR1 amplification (22%) was the most common PTGA followed by BARD1 and BRCA2 mutation (each 17%). As for gene variations associated with the efficacy of immune checkpoint blockade (ICB), FAT1 alteration (39%) and PTEN depletion (28%) were commonly observed. In conclusion, frequently altered oncogenic pathways might contribute to the development and progression of R-NETs. Gene alterations significantly associated with prognosis might be potential novel targets. Targeted therapy might be a promising strategy as targetable alterations were prevalent in R-NETs. FAT1 alteration and PTEN depletion might be the main genetic alterations influencing the response to ICB besides overall low TMB and MSI in R-NETs.
Background Type 1 gastric neuroendocrine tumors (NETs) are relatively rare to the extent that some physicians have little experience in diagnosing and treating them. The purpose of this study was to increase the understanding of the disease by analyzing and summarizing the management and prognoses of patients with type 1 gastric NETs at our center. Methods The data of 229 patients (59.4% female) with type 1 gastric NETs who were treated at our center during 2011–2022 were retrospectively analyzed. Results The average patient age was 50.5 ± 10.8 years. Multiple tumors affected 72.5% of the patients; 66.4% of the tumors were < 1 cm, 69.4% were NET G1, and 2.2% were stage III-IV. A total of 76.9% of the patients had received endoscopic management, 60.7% had received traditional Chinese medicine treatment, 10.5% received somatostatin analogues treatment, and 6.6% underwent surgical resection. Seventy patients (41.2%) experienced the first recurrence after a median follow-up of 31 months (range: 2-122 months), and the median recurrence-free time was 43 months. The 1-, 2-, and 3-year cumulative recurrence-free survival rates were 71.8%, 56.8%, and 50.3%, respectively. During a median follow-up of 39 months (range: 2-132 months), one patient had bilateral pulmonary metastasis, and no disease-related deaths were observed. Conclusion Type 1 gastric NETs have a high recurrence rate and a long disease course, underscoring the importance of long-term and comprehensive management.
目的:探讨芪贞抑瘤方对胰腺神经内分泌肿瘤(pNETs)根治性切除术后患者3年无复发生存的影响.方法:回顾性分析随访资料完整的181例Ⅰ~Ⅳ期pNETs术后患者临床资料,以是否接受累积≥6个月的芪贞抑瘤方治疗分为中药组(79例)和对照组(102例).采用Kaplan-Meier法绘制生存曲线,Log-rank法检验2组患者的无复发生存期(RFS)和第1、2、3年的累积无复发生存率的差异,采用多因素Cox风险比例模型确定RFS的独立影响因素.结果:中药组1、2、3年累积无复发率依次为93.4%、82.2%、71.5%,3年中位RFS未达到;对照组依次为 89.2%、78.2%、67.1%,3 年中位 RFS 为 31.0 个月(95%CI:24.416,37.584),2 组差异有统计学意义(P<0.05).中药治疗是影响RFS的独立保护因素(P<0.05),较对照组能降低复发转移风险(HR=0.488,95%CI:0.298,0.799).在病理分级为G1、G2,或临床分期为Ⅱ期的患者中,中药治疗有显著的优势(P<0.05).结论:芪贞抑瘤方在降低pNETs根治术后患者复发转移风险、延长无复发生存时间方面可能有一定作用.
李佩文认为认知功能不单包括记忆力,更涵盖精神意识思维活动等多方面内容,属于中医狭义"神"的范畴.认知功能以五脏精气为物质基础,尤其与五脏神中的心神、脾意、肾志密切相关,且内根于脏神,依赖阳气的化精作用外现为脑神.李佩文临证从"脏神-脑神"理论探讨乳腺癌化疗相关认识障碍的发病机制,同时强调阳气化精为神的关键作用,兼顾痰浊、瘀血、郁热等病理产物.其采用养心安神、健脾益肾为主,温阳行气、化痰祛瘀、清解郁热为辅的治疗方法治疗本病,临床疗效满意.附验案1则.
Introduction: Treatment response to the standard therapy is low for metastatic pancreatic neuroendocrine tumors (PanNETs) mainly due to the tumor heterogeneity. We investigated the heterogeneity between primary PanNETs and metastases to improve the precise treatment. Methods: The genomic and transcriptomic data of PanNETs were retrieved from the Genomics, Evidence, Neoplasia, Information, Exchange (GENIE), and Gene Expression Omnibus (GEO) database, respectively. Potential prognostic effects of gene mutations enriched in metastases were investigated. Gene set enrichment analysis was performed to investigate the functional difference. Oncology Knowledge Base was interrogated for identifying the targetable gene alterations. Results: Twenty-one genes had significantly higher mutation rates in metastases which included TP53 (10.3% vs. 16.9%, p = 0.035) and KRAS (3.7% vs. 9.1%, p = 0.016). Signaling pathways related to cell proliferation and metabolism were enriched in metastases, whereas epithelial-mesenchymal transition (EMT) and TGF-β signaling were enriched in primaries. Gene mutations were highly enriched in metastases that had significant unfavorable prognostic effects included mutation of TP53 (p < 0.001), KRAS (p = 0.001), ATM (p = 0.032), KMT2D (p = 0.001), RB1 (p < 0.001), and FAT1 (p < 0.001). Targetable alterations enriched in metastases included mutation of TSC2 (15.5%), ARID1A (9.7%), KRAS (9.1%), PTEN (8.7%), ATM (6.4%), amplification of EGFR (6.0%), MET (5.5%), CDK4 (5.5%), MDM2 (5.0%), and deletion of SMARCB1 (5.0%). Conclusion: Metastases exhibited a certain extent of genomic and transcriptomic diversity from primary PanNETs. TP53 and KRAS mutation in primary samples might associate with metastasis and contribute to a poorer prognosis. A high fraction of novel targetable alterations enriched in metastases deserves to be validated in advanced PanNETs.
Objective: To dig deeper into the traditional Chinese medicine treatment rules of chemotherapy-induced diarrhea with traditional Chinese medicine (TCM) inheritance computing platform system. Methods: Taking "traditional Chinese medicine", "chemotherapy" and "diarrhea" as the theme words, comprehensive search of the database of CNKI, Wanfang and VIP from its inception to November 2020 was conduct-ed. The formulas of external treatment of traditional Chinese medicine for chemotherapy-induced diarrhea were included and the association rule, clustering and factor analysis were carried out. Results: A total of 145 papers, 57 prescriptions meeting the inclusion criteria were collected, among which high-frequency drugs including Baizhu (Atractylodis macrocephalae rhizoma), Fuling (Poria), Dangshen (Codonopsisradix), Huanglian (Coptidis rhizoma), Zhigancao (Glycyrrhizae radix et rhizoma praeparata cum melle) were the most commonly used. The confidence level was set as 0.7 and the support level was set as 10, 12 core compatibility groups were obtained, and 6 categories were cluster analyzed. Conclusion: The principle of external treatment of chemotherapy-induced diarrhea is mainly "restore deficiency and benefiting qi", "benefiting water infiltration and dampness", "clearing heat" and "inducing astringency". Prescription combination and new prescription combination based on traditional Chinese medicine (TCM) inheritance computing platform system can be used as reference for clinicians and applied in primary hospitals.
万冬桂教授辨析乳腺癌术后上肢淋巴水肿,认为疾病病位在脉络,属本虚标实、因虚致实之证,水饮、瘀血是疾病的关键病理因素,脏腑亏虚是疾病的关键病理基础,水瘀互结,脉络瘀阻是疾病的关键病机,疾病前驱期主要病机为气血亏虚,水瘀初生;症状明显期主要病机为脏腑虚损,水瘀渐盛;转归期主要病机为水瘀胶结,留恋难去.临证应以去菀陈莝为总治则,"去"已成之瘀血、水饮的同时补脏腑亏虚以根"去"酿生"瘀血、水饮"之因.前驱期主要治法为健脾补肾,补气养血;症状明显期主要治法为通补合用,活血利水;转归期主要治法为活血化瘀,攻逐水饮,临床疗效突出.本文对其学术思想及临床经验详细阐述,以期为乳腺癌相关上肢淋巴水肿的治疗拓宽思路.
Background Extra-pulmonary neuroendocrine carcinomas (EP-NECs) are rare, accounting for ~1/100,000 of NECs, aggressive neoplasms and poor prognosis. Sometimes, a non-neuroendocrine component is also accompanying these EP-NECs. Curative surgery is suggested for early stage patients while system chemotherapy and locoregional radiotherapy are considered for advanced inoperable disease. Nonetheless, there was lack of standard second-line treatment strategy. Herein, we report a case of NEC involving a large cell neuroendocrine carcinoma (LCNEC) and adenocarcinoma of the gallbladder treated with a surufatinib-containing regimen in the second-line treatment setting and establish the efficacy of this regimen in the treatment of EP-NECs. Case Description A 58-year-old male presented with symptoms such as distension in the upper right abdomen and a palpable mass. The abdominal magnetic resonance imaging (MRI) scan showed a giant soft tissue mass in the left lobe of the liver, and liver biopsy suggested LCNEC with a non-neuroendocrine (NNE) component. Based on the available literature, a first-line therapy of oxaliplatin + gemcitabine + camrelizumab + apatinib was started initially; however, there was rapid tumor progression. Thus, a second line of treatment was started, where apatinib was replaced with surufatinib, which was given along with oxaliplatin and camrelizumab and continued for seven complete cycles. The patient was re-examined with MRI, which showed a significant decrease in tumor size. And a partial response was achieved. Main adverse events included hand and foot numbness, hypertension, proteinuria, hematuria, and hyperthyroidism. The patient underwent surgery after the second line of treatment and the post-operative pathology report revealed the presence of LCNEC and adenocarcinoma of the gallbladder. Two months later, re-examination result showed no tumor recurrence. Conclusions As yet, the criteria strategy for unresectable EP-NECs to improve survival outcomes is scarce. EP-NECs are badly in need of effective second-line therapy to carry out survival benefits after resistance to first-line regimen. The case report demonstrated that a surufatinib-containing regimen including oxaliplatin and camrelizumab could be an effective treatment strategy for the second-line treatment of EP-NECs. Furthermore, this strategy is well tolerated and treatment-related toxicity are manageable. More clinical trials are warranted to further confirm the efficacy.
Gastroenteropancreatic neuroendocrine neoplasm (GEP-NEN) is a rare, heterogeneous tumor that can be divided into neuroendocrine tumor (NET) and neuroendocrine carcinoma (NEC) due to its degree of differentiation. With the gradual deepening of the exploration of its pathogenesis and treatment methods, the importance of preclinical models has become increasingly prominent. Here we reviewed the published preclinical models such as cell lines, organoid models, patient-derived xenograft models and genetically engineered mouse models. We also discussed the current practical applications of these models and their strengths and weaknesses, to provide threads and directions for future research.
目的:探索香砂六君子汤加减贴剂防治消化道肿瘤患者中、高性致吐化疗方案后消化道反应、免疫机能及生存质量的改善情况.方法:纳入2018年1月至2021年5月中日友好医院中西医结合肿瘤内科确诊消化道恶性肿瘤并行中、高性致吐化疗方案的住院患者140例,参照药物临床试验质量管理规范(GCP)按1∶1随机分为观察组(70例)和对照组(70例),入组者化疗前20 min均给予常规止吐处理1次(托烷司琼5 mg+地塞米松5 mg或甲泼尼龙40 mg),观察组患者在此基础上化疗后第2天加用香砂六君贴疗法1周(选穴为中脘、内关、足三里,每日6h,每日更换敷贴),观察组患者同时贴敷安慰贴剂,观察化疗后两组患者治疗前及治疗后1周消化道反应分级情况、淋巴细胞亚群浓度及患者生存质量评分等数据,并进行统计分析.结果:治疗后,与本组治疗前比较,两组患者化疗相关消化道反应分级均明显下降(P<0.05);与对照组治疗后相比较,观察组患者消化道反应分级下降更明显(P<0.05).观察组总有效率为81.43%(57/70),明显高于对照组的62.86%(44/70),差异有统计学意义(x2=9.73,P<0.05).对观察组进行分层分析,与本组治疗前比较,不同种类肿瘤中食管癌和结直肠癌患者治疗后消化道反应分级明显下降(P<0.05);不同性别患者治疗后消化道反应分级明显均下降(P<0.05).免疫功能结果显示,与本组治疗前比较,对照组患者CD3+,CD4+,CD8+,NK细胞亚群浓度差异均无统计学意义,观察组患者CD3+,CD4+,NK细胞亚群明显升高,且优于对照组(P<0.05).与对照组比较,香砂六君贴观察组的生存质量Karnofsky评分明显升高,生存质量改善(P<0.05).结论:香砂六君贴可降低消化道肿瘤患者中、高性致吐化疗方案后消化道反应分级、改善患者免疫机能及提高生存质量;中医外治穴位贴敷疗法值得临床推广应用.
Objective:To analyze the value of folate receptor-positive circulating tumor cells (FR +-CTC) in the diagnosis and efficacy evaluation of patients with small cell lung cancer (SCLC). Methods:The data of 59 patients with SCLC and 14 patients with benign pulmonary diseases treated in China-Japan Friendship Hospital from May 2017 to October 2019 were retrospectively analyzed. Folate receptor targeted detection was used to detect the level of FR +-CTC in the blood of SCLC patients. The levels of serum progastrin-releasing peptide (Pro-GRP), neuron-specific enolase (NSE), cytokeratin 19 fragment 21-1 (Cyfra21-1) , and carcinoembryonic antigen (CEA) were detected by using chemiluminescence. The median ( P25, P75) was used as all the detection indexes. Mann-Whitney U test was used for pairwise comparison, Spearman correlation test was used to analyze the correlation between two variables, and receiver operator characteristic (ROC) curve was used to evaluate the diagnostic efficacy. Results:The level of FR +-CTC in 59 patients with SCLC was 11.00 FU/3 ml (7.10 FU/3 ml, 14.50 FU/3 ml), and the positive rate of FR +-CTC in patients with SCLC was 66.10% (30/59); the level of FR +-CTC in 14 patients with benign pulmonary diseases was 6.75 FU/3 ml (5.03 FU/3 ml, 7.85 FU/3 ml), and the positive rate of FR +-CTC in 14 patients with benign pulmonary diseases was 14.29% (2/14). The level of FR +-CTC in patients with SCLC was higher than that in patients with benign pulmonary diseases, and the difference was statistically different ( U = 33.50, P < 0.01). The expression level of FR +-CTC was not related to age, gender and smoking history in SCLC patients (all P>0.05). The expression level of FR +-CTC in patients with extensive-stage was higher than that in patients with limited-stage, and the difference was statistically significant ( P < 0.05). Tumor markers Pro-GRP, NSE, Cyfra21-1 and CEA were compared with FR +-CTC, and the ROC curve was drawn; the results showed that FR +-CTC had better sensitivity (71.2%) and specificity (92.90%) in the diagnosis of SCLC. For SCLC patients who received chemotherapy, the decrease range of FR +-CTC in patients with partial remission and stable disease was greater than that in patients with the progression of disease, and the differences were statistically significant (all P < 0.05). Conclusion:FR +-CTC can assist the diagnosis and disease staging of SCLC. For patients receiving chemotherapy, continuous detection of circulating tumor cells can help to evaluate the efficacy of chemotherapy and provide a reference for the choice of clinical treatment.
乳腺癌是一种常见的女性恶性肿瘤,在男性中较为少见.男性乳腺化生性癌(metaplastic breast cancer,MpBC)更是一种极其罕见的恶性肿瘤,在临床上鲜有报道.目前对于其临床特征、影像学特征、治疗方式及预后均尚未清楚.本文将1例接受乳腺改良根治术的男性MpBC患者的临床特征、影像学特征及治疗情况结合文献复习作如下报道.
Background Diarrhea is a common adverse reaction in patients with cancer receiving chemotherapy, for which there is currently no effective method of treatment. Shengjiang Xiexin decoction (SXD), a classic traditional Chinese medicine (TCM) formula, has shown efficacy in alleviating irinotecan-induced diarrhea in preliminary clinical studies. The current study is designed to assess the efficacy and safety of SXD for prophylaxis against irinotecan-induced diarrhea. Additionally, we employ a new approach to analyze and evaluate the data based on the patients’ uridine diphosphate glucuronosyltransferase 1A1 (UGT1A1) genotype, which predicts the risk of diarrhea. Methods and design A prospective, double-blind, randomized, placebo-controlled trial will be conducted in patients with small cell lung cancer (SCLC) from five hospitals in China. For this study, 100 irinotecan-naïve patients will be randomly allocated to either the SXD or placebo arms in a 1:1 ratio. Stratified randomization will be used to divide subjects by UGT1A1 genotype into groups with differing risk of diarrhea. The trial will consist of two cycles of chemotherapy with 14 days of oral administration of SXD or placebo administered beginning between 3 days before and up to 11 days after initiation of each chemotherapy cycle. The primary study outcome is the incidence of diarrhea. Secondary outcomes include the degree of diarrhea, the degree of neutropenia, the rate of alterations in chemotherapy regimens, the amount of antidiarrheal drug taken, the rate of hospitalization, and evaluation of chemotherapy efficacy. Discussion This study is the first to use the UGT1A1 genotype to stratify patients into groups based on their risk of diarrhea, and to provide a complete assessment of chemotherapy-related diarrhea (CRD), including records of diarrhea duration, grading the severity of diarrhea, and evaluating concomitant symptoms. Study results will provide high-level clinical evidence on the use of SXD as prophylaxis for CRD. Trial registration Chinese Clinical Trial Register: ChiCTR1800018490. Registered on 20 September 2018. Retrospectively registered. http://www.chictr.org.cn/edit.aspx?pid=25250&htm=4c
患者男性,59岁.2017年4月因右侧阴部出现肥厚性红色皮疹于我院皮肤科就诊,以湿疹治疗,疗效欠佳且皮损面积逐渐扩大,期间复查CEA呈进行性升高,未予重视.2019年4月触及右侧腹股沟肿大淋巴结,行PET-CT:腹膜后、右侧髂血管旁、右侧腹股沟多发淋巴结肿大,考虑转移性病变可能,来源待定.行右腹股沟淋巴结穿刺活检:淋巴组织中可见恶性肿瘤浸润.2019年6月右侧腹股沟区原肿大淋巴结附近再次出现2个质硬小结节.行胸、腹、盆、泌尿系CT、胃肠镜均未发现原发病灶.
e21590 Background: Lung cancer remains the leading cause of cancer-related death in China. DCVAC/LuCa is an active autologous cellular immunotherapy consisting of autologous DCs loaded with NSCLC antigens from NSCLC cell lines. SQFZ is a kind of TCM injection commonly uesed for NSCLC, made from Ginseng and Astragalus membranaceus. Intent of this study is to evaluate the efficacy and safety of DCVAC/LuCa plus chemotherapy and TCM in patients with advanced NSCLC and wild type driving gene(EGFR/ALK). Methods: This study consist of screening/leukapheresis/treatment/follow-up period. On the treatment period, chemotherapy started after leukapheresis. Four cycles are recommended with limitation to maximum 6 cycles. Up to 12 subcutaneous doses of DCVAC/LuCa treatment were administrated on the 15th day of chemotherapy cycle every 3 weeks for 4 doses, and continued alone after chemotherapy finishes/stops every 6 weeks for the remaining 8 doses. TCM therapies were administered on the -3 to 3 days of DCVAC/LuCa. After completion of the total 12 doses DCVAC/LuCa or discontinuation of study treatment, patients will be followed up until experienced OS event or till 1 year of survival time. The primary efficacy endpoint included ORR, PFS and survival. Results: 23 pts were included, 19 males and 4 females, median age 63(48-80). Pathological type:16 adenocarcinoma, 5 squamous cell carcinoma and 1 other. 7 Ⅲb and 16 Ⅳ stage. ORR was 17.39%(4/23), DCR was 60.87%(14/23). Median PFS was 10.9 months, median follow-up in surviving patients was 22.3 months, 1-year survival is currently 92.86%, 18-months survival is currently 79.59%. 1 case was diagnosed as acute myelogenous leukemia myelodysplastic syndrome after 9 doses of DCVAC/LuCa. There were no other adverse events related to DCVAC/LuCa treatment TCM therapies in this study. Conclusions: DCVAC/LuCa, combined with standard chemotherapy and TCM had exhibited the potential to improve the survival in patients with advanced NSCLC, and decrease the occurrence of adverse events. But the sample size of this study is small, a further clinical investigation is needed.
患者男性,56岁,于2018年4月出现全身多发红色皮疹,高出皮面,压之不褪色,伴持续性右侧头痛.随后出现对称性四肢近端肌无力,伴肌痛、吞咽困难.外院辅助检查示:CK 4942U/L,肌电图示“肌源性损伤”,予以激素治疗2个月,皮疹颜色消退,四肢肌力和吞咽困难略有改善.2018年7月就诊于我院风湿免疫科.完善相关检验检查示:CK 65IU/L.肿瘤标记物:CA 7249U/ml;CEA 5.09ng/ml.类风湿抗体谱、ANA谱、ANCA、肌炎抗体谱均(-).肌1电图:肌源性损害.肌肉MRI、肌肉活检:符合皮肌炎(der-matomyositis,DM)表现.影像学检查颅脑CT、颅脑MRA、颅脑MRI均未见异常.诊断为“DM”,予以激素、免疫抑制剂治疗4个月,皮疹颜色消退,无新发皮疹,四肢肌力较前好转,无吞咽困难.