背景:由于其罕见,妊娠期外阴癌(VC)的发病率未知;在1955年至2014年之间,全球仅报告了36例病例报告。漏报也可能是妊娠VC未知发生率的一个促成因素。这项研究的目的是分析诊断,妊娠和/或母乳喂养期间诊断的外阴癌病例的治疗和结果。
方法:患者1在妊娠18周(WG)诊断为2级VC(pT1a,pN0,0/4前哨淋巴结活检(SLNB)涉及),并通过切除肿瘤(R0)进行治疗。她目前在诊断后4年无复发。患者2在7WG诊断为2级VC(pT1b,pN1a,1/17SLNB,R0),并在孕早期和孕中期用SLNB治疗。她目前在诊断后5年无复发。患者3在30WG诊断为2级VC(pT1b,pN0,0/5SLNB,R0)。随后,她经历了许多通过切除治疗的产后局部复发,目前在诊断后3年无复发。患者4后来被诊断为VL,在母乳喂养期间的14个月,被诊断为3级VC(pT1b,pN1a,1/14SLNB,R0)。该患者目前在诊断后9年无复发。患者5在怀孕期间未被诊断,但被诊断为G3VC(pT2,pN2c,2/17SLNB,R0)产后8个月。患者由于肿瘤受累程度和淋巴结转移,术后接受放化疗。尽管有辅助治疗,患者进展并发生骨转移.对肿瘤组织的分析显示PD-L1(程序性细胞死亡蛋白1)的表达增加,表明患者可能受益于纳武单抗治疗以阻断PD-L1相互作用;不幸的是,患者在诊断后24个月去世,然后才开始免疫治疗治疗。
结论:在VC病例中,手术切除和同步SLNB在妊娠期间被认为是安全的,与非孕妇具有可比性。怀孕期间不应延迟及时的诊断检查和治疗,因为延迟诊断可能导致肿瘤进展并带来致命后果。
BACKGROUND: The incidence of vulvar cancer (VC) in pregnancy is unknown due to its rarity; between 1955 and 2014 only 36 case reports were reported worldwide. Underreporting may also be a contributing factor to the unknown incidence of VC in pregnancy. The aim of this study was to analyze the diagnosis, treatment and outcome of vulvar cancer cases diagnosed during pregnancy and/or breastfeeding.
METHODS: Patient 1 was diagnosed at 18 weeks\' gestation (WG) with Grade 2 VC (pT1a, pN0, 0/4 sentinel lymph nodes biopsy (SLNB) involved) and was treated by having the tumor resected (R0). She is currently recurrence-free at 4 years post-diagnosis. Patient 2 was diagnosed at 7 WG with Grade 2 VC (pT1b, pN1a, 1/17 SLNB, R0) and was treated during the first trimester and during the second trimester with SLNB. She is currently recurrence-free at 5 years post-diagnosis. Patient 3 was diagnosed at 30 WG with Grade 2 VC (pT1b, pN0, 0/5 SLNB, R0). She subsequently experienced a number of local recurrences postpartum that were managed by resection and is currently recurrence-free at 3 years post-diagnosis. Patient 4 was diagnosed a VL later, at 14 months during breastfeeding, that was diagnosed as Grade 3 VC (pT1b, pN1a, 1/14 SLNB, R0). The patient is currently recurrence-free at 9 years post-diagnosis. Patient 5 was not diagnosed during pregnancy, but was diagnosed with G3 VC (pT2, pN2c, 2/17 SLNB, R0) 8 months postpartum. The patient due to the extent of tumor involvement and lymph node metastasis, underwent chemoradiation therapy post-surgery. Despite adjuvant therapy, the patient progressed and developed bone metastases. Analysis of the tumour tissue revealed increased expression of PD-L1 (programmed cell death protein 1) indicating that the patient may have benefited from treatment with nivolumab to block the PD-L1 interaction; unfortunately the patient passed away at 24 months post-diagnosis before immunotherapy treatment could commence.
CONCLUSIONS: Surgical resection and simultaneous SLNB in VC cases are considered safe during pregnancy, with comparable outcomes to non-pregnant women. Prompt diagnostic workup and treatment should never be delayed during pregnancy as delayed diagnosis could lead to tumour progression with fatal consequences.