皮肌炎合并妇科恶性肿瘤11例临床特征与短期随访结局分析

doi:10.16150/j.1671-2870.2021.01.008

摘要/Abstract

摘要：

目的:分析皮肌炎（dermatomyositis,DM）合并妇科恶性肿瘤患者的临床特征及诊断和治疗,并进行短期随访,为DM患者的早期肿瘤诊断和干预提供参考资料。方法:收集我院自2015年至2020年收治的11例DM合并妇科恶性肿瘤患者的临床资料,分析患者临床特征及短期随访结局。结果:11例DM合并妇科恶性肿瘤患者,涉及3种妇科恶性肿瘤,其中子宫内膜癌4例（4/11）,宫颈癌2例（2/11）,卵巢癌5例（5/11）。根据2020年国际妇产科联盟（International Federation of Gynecology and Obstetrics,FIGO）妇科肿瘤分期标准,Ⅲ~Ⅳ期的妇科恶性肿瘤患者为8例。10例患者先确诊DM,在DM确诊之后,即接受了影像学和肿瘤指标的筛查,从而确诊了妇科恶性肿瘤,间隔时间为1周到1年不等;另1例患者初诊DM后筛查肿瘤指标及影像学检查（盆腔及腹腔CT）,未发现恶性肿瘤,但在DM随访过程中,发现癌抗原12-5（cancer antigen 12-5,CA12-5）升高,在诊断DM后第21个月确诊卵巢癌。8例患者在初诊DM时行血清抗转录中介因子1-γ（transcriptional intermediary factor 1-γ,TIF1-γ）抗体检测,6例为阳性。11例患者中,除1例宫颈癌患者直接接受根治性放疗和化疗外,10例患者均接受了妇科肿瘤手术治疗。本研究的随访时间为2个月~2年,患者总生存率为100%,随访中无妇科恶性肿瘤复发, 但2例DM复发。结论:对于确诊的DM患者,如初筛未发现明显妇科恶性肿瘤征象,仍需重视随访及筛查,注意动态随访血清肿瘤标志物;DM合并妇科恶性肿瘤患者其肿瘤分期往往较晚,多为Ⅲ~Ⅳ期;对于DM合并妇科恶性肿瘤的患者,治疗可仍以妇科恶性肿瘤治疗原则为主。

关键词: 皮肌炎, 妇科恶性肿瘤, 癌抗原12-5

Abstract:

Objective: To analyze the clinical characteristics, diagnosis and treatment of dermatomyositis (DM) accompanied with gynecological malignant tumor to provide evidence for early detection and intervention of the disease. Methods: The clinical data of 11 DM patients with gynecological malignant tumor treated in our hospital from 2015 to 2020 were enrolled, and the clinical characteristics and treatment protocols were analyzed. Results: In eleven patients diagnosed DM accompanied with gynecological malignant tumor, three types of cancer were identified, including endometrial cancer (4/11), cervical cancer (2/11), and ovarian cancer (5/11). Eightout of 11 patients were diagnosed stage Ⅲ-Ⅳ gynecological cancer according to 2020 FIGO(International Federation of Gynecology and Obstetrics) gynecological tumor staging. Ten patients were screened for gynecological cancer by imaging and tumor biomarkers,and diagnosed gynecological cancer after being confirmed as DM 1 week to 21 months. The other one patient was found to have ovarian cancer at 21^st month after diagnosis of DM because of the increased level of CA12-5 (carbohydrate antigen12-5) during the follow-up, however, her CT imaging of the pelvic and abdominal cavity and tumor biomarkers were normal in initial screening. The anti-TIF1-γ antibodies in 8 DM patients were tested, and 6 of them were positive. Except one patient with cervical cancer receiving directly radical radiotherapy and chemotherapy, the other 10 patients received gynecological tumor resection. The overall survival rate was 100% during the follow-up of 2 months to 2 years. There was no tumor recurrence duringthe follow-up, but the DM recurrence appeared in 2patients. Conclusions: For the DM patients lacking signs of malignant tumor in the initial screening, the continuing follow-upand screening for tumor biomarkers should get more attention.The DM patients accompanied gynecological malignant tumor often showed late-stage cancer (Ⅲ-Ⅳ staging) and the treatment should be mainly focused on the gynecological tumor.

Key words: Dermatomyositis, Gynecological malignancy, Cancer antigen12-5

中图分类号:

R737.31

刘畅, 曹华, 冯炜炜, 沈立翡. 皮肌炎合并妇科恶性肿瘤11例临床特征与短期随访结局分析[J]. 诊断学理论与实践, 2021, 20(01): 53-59.

LIU Chang, CAO Hua, FENG Weiwei, SHEN Lifei. Eleven cases of dermatomyositis accompanied with gynecological malignant tumor: clinical characteristics and short-term follow-up[J]. Journal of Diagnostics Concepts & Practice, 2021, 20(01): 53-59.

图/表 5

表1

表2

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表5

术前、围手术期DM的治疗方案

序号	妇科肿瘤	肿瘤分期	术前治疗方案	围手术期治疗方案
1	卵巢癌	卵巢及输卵管高级别浆液性乳头状癌ⅢC期	强的松8片（每日1次）+羟氯喹2片（每日1次）+ 帕夫林2片（每日3次）+艾洛松外用	围手术期未改变激素剂量,术后复查,逐渐减量
2	卵巢癌	卵巢高级别浆液性癌 ⅢC期	甲强龙28 mg（每日1次）+羟氯喹2片（每日1次）+ 沙利度胺50 mg（每日1次）+帕夫林2片（每日1次）	术前得宝松1支肌肉注射,沙利度胺、帕夫林及羟氯喹不停,直到2020年4月DM复发
3	卵巢癌	卵巢及早输卵管高级别浆液性癌ⅡA期	强的松15 mg（每日1次）+阿法骨化醇软胶囊 0.25 μg（每日1次）+羟氯喹每日2次）+ 沙利度胺50 mg（每日1次）	术前得宝松1支肌注,维持激素强的松3片（每日1次）,继续沙利度胺50 mg（每晚1次）, 帕夫林2片（每日2次）及羟氯喹2片（每日 2次）,术后强的松15 mg（每日1次）
4	卵巢癌	卵巢及输卵管高级别浆液性癌ⅢC期	强的松3/4片（每日1次）+帕夫林2片（每日1次）+ 羟氯喹2片（每日1次）	强的松15 mg,术前1天至术后3天,每日强的松 6片或甲强龙24 mg静脉滴注;第2次手术术前肌肉注射得宝松1支,术后第4日恢复强的松
5	卵巢癌	卵巢高级别浆液性癌 ⅢC期	甲泼尼龙24 mg（每日1次）	强的松5片（每日1次）;手术当日10片（每日 1次）,第2天5片（每日1次）
6	宫颈癌	宫颈鳞癌ⅣA期	强的松3片（每日3次）	未手术
7	宫颈癌	宫颈鳞癌ⅠB2	强的松6片（每日1次）	术前甲强龙40 mg静脉滴注,手术当日80 mg静脉滴注,术后40 mg静脉滴注3 d;术后第4天起强的松6片（每日1次）,后减至3片（每日1次）
8	子宫内膜癌	子宫内膜浆液性癌ⅢC2	强的松20 mg（每日2次）+他克莫司1 mg （每日2次）	停用他克莫司,手术当天及术后第2天甲强龙 40 mg（每日1次）,第3天起20 mg,之后强的松5片（每日1次）
9	子宫内膜癌	子宫内膜样腺癌ⅢC2期	强的松6片（每日1次）	手术当日至术后3 d每日甲强龙40 mg静脉滴注,术后第4天恢复强的松6片（每日1次）
10	子宫内膜癌	子宫内膜样腺癌Ⅱ期, 部分透明细胞癌	羟氯喹1片（每日2次）+甲强龙30 mg（每日1次）	术前得宝松1支肌肉注射
11	子宫内膜癌	子宫内膜癌肉瘤ⅢA期	强的松8片（每日1次）+羟氯喹2片（每日1次） +帕夫林2片（每日2次）+沙利度胺50 mg（每日1次）	术前1天、手术当日至术后3 d,甲强龙40 mg; 术后口服强的松30 mg（每日1次）,化疗结束后20 mg（每日1次）

表5

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序号	妇科肿瘤	皮疹范围	肌炎表现	肌肉活检	妇科肿瘤治疗方案
1	卵巢癌	额部、眼眶、背部、双上肢、双手指关节伸侧可见红斑,Gottron征阳性	不详	肌炎	手术
2	卵巢癌	全身暗紫红色红斑,颜面部、颈部较重, Gottron征阳性	不详	DM	手术+TC方案化疗
3	卵巢癌	面部,眼睑、躯干下肢可见红斑, Gottron征阳性	无肌痛肌无力	DM	新辅助TC方案化疗+手术
4	卵巢癌	不详	不详	不详	手术+TC方案化疗
5	卵巢癌	额部、颈部、面、耳前、躯干四肢红斑, Gottron征阳性	无肌痛,上肢近端肌力Ⅲ级, 下肢肌力Ⅳ级	请结合临床	手术+TC方案化疗
6	宫颈癌	面部红斑	不详	不详	根治性放化疗
7	宫颈癌	面部、双侧腕关节、颈部、肩部及股沟处红斑, 技工手,Gottron征及披肩征阳性	无肌痛、肌无力	不详	手术
8	子宫内膜癌	颈部、胸前区红斑	无肌痛、肌无力	不详	手术+TC方案化疗+放疗
9	子宫内膜癌	双上眼睑、前胸V字区红斑	四肢肌力Ⅲ级,轻压痛,肌张力正常,环咽肌受累	肌炎	新辅助TC方案化疗+手术
10	子宫内膜癌	面颈部、前胸、后背、腹部、双大腿外侧及双手背红斑,Gottron征阳性	无肌痛、肌无力	DM	手术
11	子宫内膜癌	面部、躯干四肢红斑,部分呈靶形损害, 伴有皮肤破溃	不详	DM	手术+TC方案化疗

序号	妇科肿瘤	抗TIF1-γ抗体		术前AFP (<7 ng/mL)	术前CEA (<5 ng/mL)	术前CA12-5 (<23 U/mL)	术前CA19-9 (<25 U/mL)	术前HE4^a） (pmol/mL)	术前SCCA (<1.5 ng/mL)
序号	妇科肿瘤	MBL法(U/L)	重组蛋白法(U/L)	术前AFP (<7 ng/mL)	术前CEA (<5 ng/mL)	术前CA12-5 (<23 U/mL)	术前CA19-9 (<25 U/mL)	术前HE4^a） (pmol/mL)	术前SCCA (<1.5 ng/mL)
1	卵巢癌	134.1	-	未测	16.47	163.9	61.8	未测	未测
2	卵巢癌	117.9	-	1.92	1.12	823.4	<0.8	未测	未测
3	卵巢癌	77.5	-	5.02	1.52	106.2	4.8	182.1	未测
4	卵巢癌	101.3	-	2.90	1.16	563.8	14.3	414.3	未测
5	卵巢癌	-	71.10	1.76	2.94	224.1	13.0	75.0	未测
6	宫颈癌	-	未测	未测	未测	未测	未测	未测	2.6
7	宫颈癌	-	未测	2.34	1.18	7.6	10.8	未测	5.0
8	子宫内膜癌	-	未测	2.29	2.45	143.1	<0.8	104.0	未测
9	子宫内膜癌	-	222.13	2.35	1.37	9.0	10.2	33.0	未测
10	子宫内膜癌	28.4	-	1 052.30	2.03	24.4	12.1	103.0	未测
11	子宫内膜癌	72.9	-	2.25	1.42	10.7	4.7	70.5	未测