17例外阴非上皮性恶性肿瘤患者临床病例分析并文献复习

发布时间：2018-06-10 12:33

本文选题：外阴非上皮性恶性肿瘤 + 外阴肉瘤　；参考：《山东大学》2017年硕士论文

【摘要】：目的:外阴非上皮性恶性肿瘤发生率低,目前尚无大组病例的分析研究。本文回顾性分析我院近10年间外阴非上皮性恶性肿瘤患者的临床诊治情况,对其发生率、临床特点、诊断及治疗、复发及预后情况进行研究,旨在为临床决策提供参考。方法:收集2007年1月至2017年1月在山东大学齐鲁医院住院患者资料,外阴恶性肿瘤共159例,其中发生于外阴的非上皮性恶性肿瘤共18例,资料齐全者17例。17例包括10例外阴恶性黑色素瘤、6例外阴肉瘤和1例外阴卵黄囊瘤。回顾性分析17例患者的临床特点及诊治情况,对患者术后复发及预后情况进行随访,随访时间截止到2017年3月。结果:1、发生率:17例外阴非上皮性恶性肿瘤占同期外阴恶性肿瘤的10.7%(17/159)。外阴恶性黑色素瘤10例(58.8%),外阴肉瘤6例(35.3%),外阴卵黄囊瘤1例(5.9%),分别占同期外阴恶性肿瘤的6.3%、3.8%和0.6%。2、临床特点:17例外阴非上皮性恶性肿瘤患者发病年龄为10～82岁,平均发病年龄48.4岁,以50～64岁比例最大,占总人数的41.2%。外阴恶性黑色素瘤和外阴肉瘤的平均发病年龄及中位发病年龄分别是56.6岁、57岁和36.7岁、36岁。临床表现为外阴肿物者16例(94.1%),其中伴疼痛、瘙痒者各占47.1%、23.5%,无症状者占29.4%。其发生部位主要在小阴唇(41.2%)、大阴唇(35.3%),其中10例黑色素瘤患者最常见的部位在小阴唇(70.0%),6例外阴肉瘤患者主要发生在大阴唇(50%),1例外阴卵黄囊瘤患者发生于右侧大阴唇。3、术前活检:9例行术前活检术(52.9%),其中8例于外院行活检术。术前活检病理与术后病理符合者7例,占77.8%。2例术前活检病理与术后病理不符合者均为外阴肉瘤,分别是上皮样肉瘤和滑膜肉瘤。7例术前活检病理与术后病理符合者均为外阴恶性黑色素瘤,其中6例活检方式为部分组织切取活检,1例为完整组织切除活检。4、术前影像学检查:7例(41.2%)患者于术前行影像学检查,其中5例为外阴肉瘤,2例为外阴恶性黑色素瘤。5例外阴肉瘤患者中1例行外阴体表超声,2例行盆腔MRI,2例行盆腔CT。2例行盆腔MRI者检查前已确诊为外阴肉瘤,其中1例是外阴低度恶性黏液纤维肉瘤,另1例是外阴胚胎状横纹肌肉瘤。MRI和CT检查均无特征性的表现。5、免疫组化:17例中16例患者进行了免疫组化分析,另外1例外阴恶性黑色素瘤患者通过镜下病理检查能大致确诊且患者拒绝免疫组化分析。9例外阴恶性黑色素瘤患者进行免疫组化分析,其中最常用的免疫组化标志物有:HMB-45、MelanA、S-100,阳性表达率均为100%。外阴肉瘤免疫组化标志物主要是中间丝蛋白、上皮性肿瘤标志物、肌分化标志物等,新型免疫组化标志物myogenin和myoD1在外阴胚胎状横纹肌肉瘤中阳性表达。外阴卵黄囊瘤主要有 AFP、glypican-3 和 SALL4。6、手术治疗:17例患者中有4例在外院行初治手术。13例初治手术在我院进行,其中8例恶性黑色素瘤,5例外阴肉瘤。初治手术方式包括单纯外阴肿瘤切除术(52.9%),广泛外阴切除术(17.60%)和外阴局部广泛切除术(17.60%)。9例患者同时进行腹股沟淋巴结清扫术(52.9%)。7、术后辅助治疗:17例中1例外阴肉瘤患者失访,余16例患者中术后进行辅助化疗者8例,其中5例为外阴恶性黑色素瘤,2例为外阴肉瘤,1例为外阴卵黄囊瘤。外阴恶性黑色素瘤化疗方案有达卡巴嗪单药化疗、替莫唑胺+顺铂、奈达铂+达卡巴嗪、顺铂+多西他赛。1例外阴滑膜肉瘤患者术后进行异环磷酰胺+吡柔比星(IE)联合化疗,另1例外阴胚胎状横纹肌肉瘤患者术后进行长春新碱+放线菌素-D+环磷酰胺(VAC)联合化疗方案。1例外阴卵黄囊瘤患者术后进行博来霉素+依托泊苷+顺铂(BEP)联合化疗方案。10例外阴恶性黑色素瘤患者中进行干扰素治疗者有6例。8、预后:随访截止到2017年3月,局部复发者4例,远处转移者2例,其中1例肺部转移,1例全身广泛转移。除1例患者失访外,其余16例患者中有5例死亡,生存时间8～96月。3例恶性黑色素瘤患者分别因病变广泛致多器官功能衰竭、肺转移、化疗后骨髓衰竭全身感染而死亡。2例外阴肉瘤患者具体死亡原因不详。结论:1、外阴非上皮性恶性肿瘤发生率低,占外阴恶性肿瘤的10.7%。2、免疫组化在外阴非上皮性恶性肿瘤亚型的诊断中起关键性作用,新型免疫组化标志物的出现为诊断提供了新的依据。如术前确定肿瘤亚型则有可能避免不必要的外阴广泛切除术,术后则可避免不必要的化疗。3、外阴低度恶性软组织肉瘤推荐局部扩大切除术并保证安全的手术切缘(2cm),外阴高度恶性软组织肉瘤以广泛外阴切除术为主。外阴恶性黑色素瘤的手术治疗以局部扩大切除术为主。
[Abstract]:Objective: the incidence of non epithelial malignant tumor of vulva is low and there is no large group of cases. The clinical diagnosis and treatment of non epithelial malignant tumor of vulva in our hospital during the last 10 years is reviewed, and the incidence, clinical characteristics, diagnosis and treatment, recurrence and precondition are studied in order to provide reference for clinical decision. Methods: 159 cases of malignant tumor of vulva were collected from January 2007 to January 2017 in Qilu Hospital of Shandong University. There were 18 cases of non epithelial malignant tumor of vulva, 17 cases of.17 including 10 cases of malignant melanoma, 6 cases of vulvar tumor and 1 yolk sac tumor. Retrospective analysis of 17 patients. The clinical features and diagnosis and treatment were followed up for the postoperative recurrence and prognosis, and the follow-up time ended in March 2017. Results: 1, the incidence of 17 cases of vulvar malignant neoplasm accounted for 10.7% (17/159), 10 cases of malignant melanoma of the vulva (58.8%), 6 cases of vulvar sarcoma (35.3%), and 1 vulvar yolk sac tumor (5.9%). 6.3%, 3.8% and 0.6%.2 of malignant vulvar tumors in the same period, respectively, and clinical characteristics: 17 cases of negative non epithelial malignant tumors were 10~82 years old, the average age of onset was 48.4 years old, the average age and median age of 41.2%. malignant melanoma and vulvar sarcoma in the total number of malignant melanoma and vulvar sarcoma, which accounted for the total number, respectively. 56.6 years old, 57 years old and 36.7 years old, 36 years old. 16 cases (94.1%) were clinically manifested as vulvar swelling, with pain, itching in 47.1%, 23.5%, and asymptomatic in 29.4%. mainly located in the labia labium (41.2%) and labia labia (35.3%), among which 10 cases of melanoma were the most common in the labia (70%), and vulvar sarcoma 6 in 6 cases. In the labia labium (50%), 1 cases of yolk sac tumor occurred on the right labia labia.3, preoperative biopsy: 9 cases of preoperative biopsy (52.9%), of which 8 cases were performed by biopsy. Preoperative biopsy pathology and postoperative pathology 7 cases, preoperatively biopsy pathology and postoperative pathological non conformation of vulvar sarcoma, respectively, are epithelioid sarcomas. .7 cases of synovial sarcoma were all malignant melanoma of vulvar, of which 6 cases were biopsy in partial tissue, 1 with complete biopsy of.4 and preoperative imaging examination: 7 cases (41.2%) underwent imaging examination before operation, of which 5 were vulvar sarcoma and 2 were malignant melanoma of the vulva. Of the 5 cases of vulvarosarcoma, 1 were treated with superficial sonography, 2 were pelvic MRI, 2 cases had pelvic CT.2 routine pelvic MRI, which had been diagnosed as vulvar sarcoma, 1 were vulvar malignant myxosarcoma, the other 1 were.MRI and CT of vulvar rhabdomyosarcoma, no characteristic.5, immunohistochemistry: 16 cases of 17 cases. In the other 1 cases of malignant melanoma of the vulva, 1 cases of malignant melanoma of the vulva were analyzed by immunohistochemistry. The most commonly used immunohistochemical markers were HMB-45, MelanA, S-100, and the positive rate of the positive expression was 100%. vulva. The immunohistochemical markers of sarcoma are mainly intermediate filament protein, epithelial tumor marker, muscle differentiation marker and so on. The new immunohistochemical marker myogenin and myoD1 are positive in the vulvar rhabdomyosarcoma. The yolk yolk sac tumor is mainly AFP, Glypican-3 and SALL4.6, and 4 of the 17 patients are treated in the external hospital. 8 cases of primary treatment were performed in our hospital, including 8 cases of malignant melanoma and 5 cases of vulvar sarcoma. The primary treatment method included simple vulvectomy resection (52.9%), extensive vulvectomy (17.60%) and extensive partial excision of vulva (17.60%).9 patients with inguinal lymph node dissection (52.9%).7 after operation: 17 cases were treated in 17 cases. 1 patients with vulvar sarcoma were lost and 8 cases were treated with adjuvant chemotherapy in the remaining 16 patients, of which 5 were malignant melanoma of vulva, 2 were vulvar sarcoma and 1 were vulvar yolk sac tumor. The chemotherapy regimen of malignant melanoma of the vulva had Dhaka basazine single drug chemotherapy, temozolamine + cisplatin, nedaplatin + Dhaka BBA, cisplatin + docetaxel and.1 cases of vulva. Synoviosarcoma patients underwent combined chemotherapy with isosophosphamide + pirirubicin (IE), and 1 cases of vulvar rhabdomyosarcoma patients underwent vincristine + actinomycin -D+ cyclophosphamide (VAC) combined chemotherapy regimen.1 excepting yolk sac tumor patients to perform.10 exceptions of bleomycin + etoposide + cisplatin (BEP) combined chemotherapy after operation There were 6 cases of interferon.8 in patients with malignant melanoma of the shade. The prognosis was followed up to March 2017, 4 cases with local recurrence, 2 cases of distant metastasis, 1 cases of pulmonary metastasis and 1 cases of extensive metastasis. 5 cases died in the other 16 patients except 1 cases, and the survival time was 8~96 months.3 cases of malignant melanoma, respectively. The specific cause of death in patients with.2 exceptions to the vulvar sarcoma was unknown because of the extensive cause of multiple organ failure, pulmonary metastasis and systemic infection of bone marrow failure after chemotherapy. Conclusion: 1, the incidence of non epithelial malignant tumor of the vulva is low, which accounts for the 10.7%.2 of vulvar malignant tumor, and the immunohistochemical method plays a key role in the diagnosis of the subtype of the non epithelial malignant tumor of the vulva. The appearance of a new immunohistochemical marker provides a new basis for diagnosis. For example, it is possible to avoid unnecessary extensive excision of the vulva and avoid unnecessary chemotherapy.3, and to avoid unnecessary chemotherapy after operation, and to recommend local enlargement of the vulvar soft tissue sarcoma and ensure the safe surgical margin (2cm) and high vulva. The majority of malignant soft tissue sarcomas are mainly vulvar resection. Local enlargement resection is the main surgical treatment for vulvar malignant melanoma.
【学位授予单位】：山东大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R737.35

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