發(fā)布時(shí)間：2018-06-10 12:33

  本文選題：外陰非上皮性惡性腫瘤 + 外陰肉瘤�。� 參考：《山東大學(xué)》2017年碩士論文

【摘要】：目的:外陰非上皮性惡性腫瘤發(fā)生率低,目前尚無(wú)大組病例的分析研究。本文回顧性分析我院近10年間外陰非上皮性惡性腫瘤患者的臨床診治情況,對(duì)其發(fā)生率、臨床特點(diǎn)、診斷及治療、復(fù)發(fā)及預(yù)后情況進(jìn)行研究,旨在為臨床決策提供參考。方法:收集2007年1月至2017年1月在山東大學(xué)齊魯醫(yī)院住院患者資料,外陰惡性腫瘤共159例,其中發(fā)生于外陰的非上皮性惡性腫瘤共18例,資料齊全者17例。17例包括10例外陰惡性黑色素瘤、6例外陰肉瘤和1例外陰卵黃囊瘤。回顧性分析17例患者的臨床特點(diǎn)及診治情況,對(duì)患者術(shù)后復(fù)發(fā)及預(yù)后情況進(jìn)行隨訪,隨訪時(shí)間截止到2017年3月。結(jié)果:1、發(fā)生率:17例外陰非上皮性惡性腫瘤占同期外陰惡性腫瘤的10.7%(17/159)。外陰惡性黑色素瘤10例(58.8%),外陰肉瘤6例(35.3%),外陰卵黃囊瘤1例(5.9%),分別占同期外陰惡性腫瘤的6.3%、3.8%和0.6%。2、臨床特點(diǎn):17例外陰非上皮性惡性腫瘤患者發(fā)病年齡為10～82歲,平均發(fā)病年齡48.4歲,以50～64歲比例最大,占總?cè)藬?shù)的41.2%。外陰惡性黑色素瘤和外陰肉瘤的平均發(fā)病年齡及中位發(fā)病年齡分別是56.6歲、57歲和36.7歲、36歲。臨床表現(xiàn)為外陰腫物者16例(94.1%),其中伴疼痛、瘙癢者各占47.1%、23.5%,無(wú)癥狀者占29.4%。其發(fā)生部位主要在小陰唇(41.2%)、大陰唇(35.3%),其中10例黑色素瘤患者最常見(jiàn)的部位在小陰唇(70.0%),6例外陰肉瘤患者主要發(fā)生在大陰唇(50%),1例外陰卵黃囊瘤患者發(fā)生于右側(cè)大陰唇。3、術(shù)前活檢:9例行術(shù)前活檢術(shù)(52.9%),其中8例于外院行活檢術(shù)。術(shù)前活檢病理與術(shù)后病理符合者7例,占77.8%。2例術(shù)前活檢病理與術(shù)后病理不符合者均為外陰肉瘤,分別是上皮樣肉瘤和滑膜肉瘤。7例術(shù)前活檢病理與術(shù)后病理符合者均為外陰惡性黑色素瘤,其中6例活檢方式為部分組織切取活檢,1例為完整組織切除活檢。4、術(shù)前影像學(xué)檢查:7例(41.2%)患者于術(shù)前行影像學(xué)檢查,其中5例為外陰肉瘤,2例為外陰惡性黑色素瘤。5例外陰肉瘤患者中1例行外陰體表超聲,2例行盆腔MRI,2例行盆腔CT。2例行盆腔MRI者檢查前已確診為外陰肉瘤,其中1例是外陰低度惡性黏液纖維肉瘤,另1例是外陰胚胎狀橫紋肌肉瘤。MRI和CT檢查均無(wú)特征性的表現(xiàn)。5、免疫組化:17例中16例患者進(jìn)行了免疫組化分析,另外1例外陰惡性黑色素瘤患者通過(guò)鏡下病理檢查能大致確診且患者拒絕免疫組化分析。9例外陰惡性黑色素瘤患者進(jìn)行免疫組化分析,其中最常用的免疫組化標(biāo)志物有:HMB-45、MelanA、S-100,陽(yáng)性表達(dá)率均為100%。外陰肉瘤免疫組化標(biāo)志物主要是中間絲蛋白、上皮性腫瘤標(biāo)志物、肌分化標(biāo)志物等,新型免疫組化標(biāo)志物myogenin和myoD1在外陰胚胎狀橫紋肌肉瘤中陽(yáng)性表達(dá)。外陰卵黃囊瘤主要有 AFP、glypican-3 和 SALL4。6、手術(shù)治療:17例患者中有4例在外院行初治手術(shù)。13例初治手術(shù)在我院進(jìn)行,其中8例惡性黑色素瘤,5例外陰肉瘤。初治手術(shù)方式包括單純外陰腫瘤切除術(shù)(52.9%),廣泛外陰切除術(shù)(17.60%)和外陰局部廣泛切除術(shù)(17.60%)。9例患者同時(shí)進(jìn)行腹股溝淋巴結(jié)清掃術(shù)(52.9%)。7、術(shù)后輔助治療:17例中1例外陰肉瘤患者失訪,余16例患者中術(shù)后進(jìn)行輔助化療者8例,其中5例為外陰惡性黑色素瘤,2例為外陰肉瘤,1例為外陰卵黃囊瘤。外陰惡性黑色素瘤化療方案有達(dá)卡巴嗪?jiǎn)嗡幓�、替莫唑�?順鉑、奈達(dá)鉑+達(dá)卡巴嗪、順鉑+多西他賽。1例外陰滑膜肉瘤患者術(shù)后進(jìn)行異環(huán)磷酰胺+吡柔比星(IE)聯(lián)合化療,另1例外陰胚胎狀橫紋肌肉瘤患者術(shù)后進(jìn)行長(zhǎng)春新堿+放線菌素-D+環(huán)磷酰胺(VAC)聯(lián)合化療方案。1例外陰卵黃囊瘤患者術(shù)后進(jìn)行博來(lái)霉素+依托泊苷+順鉑(BEP)聯(lián)合化療方案。10例外陰惡性黑色素瘤患者中進(jìn)行干擾素治療者有6例。8、預(yù)后:隨訪截止到2017年3月,局部復(fù)發(fā)者4例,遠(yuǎn)處轉(zhuǎn)移者2例,其中1例肺部轉(zhuǎn)移,1例全身廣泛轉(zhuǎn)移。除1例患者失訪外,其余16例患者中有5例死亡,生存時(shí)間8～96月。3例惡性黑色素瘤患者分別因病變廣泛致多器官功能衰竭、肺轉(zhuǎn)移、化療后骨髓衰竭全身感染而死亡。2例外陰肉瘤患者具體死亡原因不詳。結(jié)論:1、外陰非上皮性惡性腫瘤發(fā)生率低,占外陰惡性腫瘤的10.7%。2、免疫組化在外陰非上皮性惡性腫瘤亞型的診斷中起關(guān)鍵性作用,新型免疫組化標(biāo)志物的出現(xiàn)為診斷提供了新的依據(jù)。如術(shù)前確定腫瘤亞型則有可能避免不必要的外陰廣泛切除術(shù),術(shù)后則可避免不必要的化療。3、外陰低度惡性軟組織肉瘤推薦局部擴(kuò)大切除術(shù)并保證安全的手術(shù)切緣(2cm),外陰高度惡性軟組織肉瘤以廣泛外陰切除術(shù)為主。外陰惡性黑色素瘤的手術(shù)治療以局部擴(kuò)大切除術(shù)為主。
[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.
【學(xué)位授予單位】：山東大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2017
【分類(lèi)號(hào)】：R737.35

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