外阴佩吉特病7例临床分析

外阴佩吉特(Paget)病是一种少见的外阴皮肤恶性肿瘤，多发生在绝经后，手术后仍具有较高的复发率。1993年1月至2002年12月我院共收治外阴佩吉特病7例。现将其临床特点、治疗及预后作一总结，以提高对此病的认识。     

外阴透明细胞汗腺瘤1例

患者4 0岁,住院号2 6 76 74。因发现外阴肿物6年,疼痛7d ,于2 0 0 2年1 1月1 4日入院。患者于6年前无意间发现外阴阴阜有一肿物,豆粒大小,无疼痛,未治疗,肿物渐增大至鸡卵大小。7d前开始感觉肿物疼痛,呈阵发性针刺感,近4d肿物表面破溃,有脓血性分泌物流出,无发热、畏寒等不适。妇科检查:外阴阴阜上方可见一约4cm×4cm×4cm大小的囊实性肿物。肿物光滑、红肿、压痛,表面有一破溃口,有脓血性分泌物溢出。阴道通畅,宫颈光滑,子宫大小正常,双附件区无异常。入院后予抗感染治疗,局麻下行肿瘤切除术。剖开肿物见内有一空腔,腔壁有烂肉样组织,呈乳…     

外阴Paget病的临床特点与治疗分析

目的:探讨外阴Paget病的临床特点及治疗方法。方法:回顾分析8例外阴Paget病的临床资料。结果:8例外阴Paget病中7例(87.5%)为绝经后妇女,平均年龄64.5岁,5例(62.5%)以瘙痒为首发症状,4例有皮肤湿疹样改变,4例局部皮肤增厚、僵硬或有溃疡、红肿。2例合并其他部位的恶性肿瘤。8例在发现外阴病变或出现症状后5~120个月(平均43.37个月)经外阴活检组织学确诊。治疗以手术为主,8例中3例有浸润性病变或合并腺癌,扩大手术范围或术后辅助放疗,1例发展为癌。1例腹股沟淋巴结转移者也进展为癌。4例为表皮内Paget病,其中3例行局部病灶切除术或外阴单纯切除术,1例放射治疗,均未复发。结论:外阴Paget病多见于绝经后患者,症状以瘙痒为主,有就诊延迟现象。治疗以手术为主,表皮内Paget病可行外阴单纯切除术或局部病灶切除术,伴有浸润性病变、合并腺癌或淋巴结转移者常需扩大手术范围。复发常见,患者均需终生随访。     

外阴佩吉特病术后复发1例

患者 6 5岁。外阴瘙痒 16年 ,近期加重伴外阴色素减退 1年余 ,于 1998年 4月 30日入院。既往糖尿病史 16年伴轻度外阴瘙痒。 1997年 2月始瘙痒加重 ,同时发现外阴约 1cm× 1cm大小白斑 ,表面覆盖白色分泌物 ,剧痒难忍。1年间白斑逐渐长大至 3cm× 4cm。 1998年 3月 16日病灶活检 ,病理报告为外阴佩吉特 (Paget’s)病。妇科检查 :左侧大阴唇内侧及小阴唇外侧可见 4cm× 4cm色素减退区 ,表浅糜烂 ,阴道无异常 ,宫颈、子宫萎缩 ,双侧附件无异常。拟诊 :外阴Paget’s病。在连续硬膜外麻醉下行单纯外阴切除 ,切除范围在病灶外 1cm ,包括左小阴唇…     

外阴基底细胞癌7例临床分析

基底细胞癌(basal cell caminoma，BCC)又称基底细胞上皮瘤，好发于身体暴露部位，特别是颜面部，也可发生于外阴部，约占外阴恶性肿瘤的2．0％，13％。我们对资料完整的7例患者进行了分析，对其发生、发展、临床表现、病理类型及预后特点进行了探讨。     

肌皮瓣转移用于外阴癌根治术后外阴重建

１９８９年７月－１９９４年４月对１８例外阴恶性肿瘤患者行外阴广泛切除并腹股沟深、浅淋巴结及盆腔淋巴结清扫术。术后患者者餐阴皮肤及皮下组织有大面积缺损区，因而行外阴整复及重建。根据整复与重建的方法不同而分为３组。     

非上皮来源外阴肿瘤的临床病理分析

目的 探讨非上皮来源的少见外阴肿瘤的临床和病理特点.方法 回顾性分析1986年至2007年,北京协和医院住院治疗的42例非上皮来源外阴肿瘤患者的临床病理资料.结果 42例患者的平均发病年龄44.1岁;临床表现为外阴肿物者有32例,占76%;有伴随症状者21例,占50%.肿瘤直径1～15 cm不等.治疗主要以手术治疗为主,其中行外阴病灶切除术40例,外阴单纯切除术1例,外阴广泛性切除术1例.术中见肿物直径>10 cm有2例,均为侵袭性血管黏液瘤患者.手术切缘干净者41例,1例未切净者为侵袭性血管黏液瘤,第2次手术切净.术后组织病理学诊断中,最常见的类型,良性肿瘤依次为汗腺瘤(7例,17%)和血管瘤(5例,12%);恶性肿瘤依次为平滑肌肉瘤(3例,7%)和侵袭性血管黏液瘤(2例,5%).结论 非上皮来源的外阴肿瘤少见,组织来源多为间叶组织,大部分临床表现缺乏特异性,术后组织病理学诊断是确诊的关键.良性肿瘤治疗上多采取局部病灶切除术,而恶性肿瘤,一般需行扩大切除术或外阴广泛性切除术.     

外阴癌33例的临床资料及预后分析

目的:总结33例外阴癌病例的临床资料及治疗方法,分析与外阴癌预后有关的因素。方法:回顾分析1990年1月至2011年2月我院收治的33例外阴癌的临床资料,应用SPSS软件比较分析不同的临床特点及有关预后因素。结果:33例中鳞癌20例,基底细胞癌2例,恶性黑色素瘤4例,乳房外Paget's病4例,上皮样肉瘤1例,腺样囊性癌1例,疣状癌1例。根据国际妇产科联盟(FIGO)及恶性黑色素瘤分期标准(American jointcommittee on cancer,AJCC),Ⅰ期9例(27.3%),Ⅱ期14例(42.4%),Ⅲ期9例(27.3%),Ⅳ期1例(3.0%)。无和有淋巴结受累患者的中位生存时间分别是43.0和21.5个月(P<0.05),侧位型和中线型患者的中位生存时间分别是40.0和20.5个月(P<0.05)。结论:外阴癌的治疗方式应根据病情个体化。外阴癌的预后与肿瘤部位及是否累及淋巴结等密切相关。     

放射治疗外阴癌39例临床分析

本文总结分析中山医科大学肿瘤医院自1964年1月至1985年12月,综合或单独应用放射治疗外阴癌39例的治疗方法及疗效.其中37例首次治疗,另2例在外院手术后复发再次治疗.在本组资料中,鳞癌37例,乳头状瘤癌变及腺鳞混合癌各1例.治疗方法及疗效:①单独放射治疗11例,包括病灶镭模敷贴、~(60)Co 针组织间插植、高能X 线外照射等,5年生存5例.②外阴病灶切除加外阴高能X 线外照射2例,均生存.③外阴广泛切除加腹股沟区放射16例,生存4例.④根治术后加腹股沟、盆髂区外照射10例,生存3例.本文结合文献讨论了放射治疗在外阴癌治疗中的应用.     

外阴硬化性苔癣合并外阴鳞状细胞癌一例

外阴硬化性苔藓（vulva lichen sclerosis,VLS）是女性慢性难治性皮肤病。主要发生于外阴及肛周皮肤,以外阴皮肤变白、瘙痒为主要特征,若伴有外阴疼痛及硬结明显者应警惕发展为外阴鳞状细胞癌（vulva squamous cell carcinoma,VSCC）。本文介绍了一例有40余年VLS病史的56岁女性,于大阴唇部位取材,组织病理示“外阴硬化性苔藓,轻度”,后因阴蒂部位粘连,且疼痛明显,行外阴粘连分离术,见阴蒂色暗红,呈小菜花状,并分别用活检钳及冷刀两次病理取材后组织病理证实为VSCC,遂行扩大性外阴切除术+双侧腹股沟淋巴结切除术。术后进一步给予放疗+化疗。本例提示了对于VLS病史较长,且局部硬结,疼痛明显者,应警惕癌变,若阴蒂处疼痛且粘连者,应行粘连分解,暴露阴蒂,必要时重复取材,以免漏诊误诊。     

The outcomes of patients with positive margins after excision for intraepithelial Paget's disease of the vulva

OBJECTIVES: Vulvar Paget's disease is a rare neoplasm that usually occurs in postmenopausal women. Treatment with surgical excision can be complicated by extension of microscopic disease in an irregular manner well beyond the visible margins of the lesion. The objective or our study was to analyze the outcomes of patients with primary vulvar intraepithelial Paget's disease who had positive microscopic margins after primary excision. METHODS: We reviewed the records of all patients with Paget's disease of the vulva treated at our institution from 1/80 to 9/02. Patients whose sample showed stromal invasion or an underlying carcinoma were excluded. Data were collected regarding patient demographics, disease location, treatment, surgical margin status, additional treatment, and clinical outcome. RESULTS: The medical records and histopathologic specimens of 28 women with intraepithelial Paget's disease of the vulva were evaluated. Surgical treatment consisted of radical vulvectomy in 3 patients (11%), simple vulvectomy in 18 patients (64%), and wide local excision in the other 7 patients (25%). Of the 20 patients with microscopically positive margins, 14 (70%) developed recurrent disease and the remaining 6 (30%) are disease free. Of the 8 patients with negative margins, 3 (38%) developed disease recurrence and the remaining 5 (63%) are disease free. With a median follow-up of 49 months (range, 3-186 months), there was no correlation between disease recurrence and margin status (P=0.20). Of the 17 patients who recurred, 14 (82%) underwent additional surgical excision and 1 patient was treated with Retin-A. The remaining 2 patients refused further treatment and were lost to follow-up. In those patients who underwent surgery for recurrence, between 1 and 3 re-excisions were performed. Of the 15 evaluable patients who were treated for recurrent disease, 12 (80%) had no evidence of persistent disease and 3 (20%) had persistent disease at a median follow-up of 63.7 months (range, 18.5-186 months). CONCLUSIONS: Microscopically positive margins following surgical excision of vulvar intraepithelial Paget's disease is a frequent finding, and disease recurrence is common regardless of surgical margin status. Long-term monitoring of patients is recommended, and repeat surgical excision is often necessary.     

Extramammary Paget's disease with diffuse involvement of the lower female genito-urinary system

Extramammary Paget's disease of the lower female genito-urinary system is an uncommon neoplasm with a high rate of recurrence. A 52-year-old white female with a history of extramammary Paget's disease, originally excised in 1985 by skinning vulvectomy and who had multiple recurrences, including two in a vaginal graft and with extension to the urethra and the uterine cervix, in 1992 underwent an anterior pelvic exenteration for control of the disease process. Standard surgical management for extramammary Paget's disease without invasion or without an underlying adenocarcinoma is simple or skinning vulvectomy. However, because the disease commonly recurs, diffuse involvement may require more extensive surgery including pelvic exenteration in extraordinary cases.     

Simultaneous vulvar intraepithelial neoplasia and Paget's disease: Report of two cases

Abstract. Orlandi A, Piccione E, Francesconi A, Spagnoli LG. Simultaneous vulvar intraepithelial neoplasia and Paget's disease: Report of two cases.
We describe a very rare association between intraepithelial, extramammary Paget's disease and human papillomavirus- (HPV) negative, keratinized type of VIN III observed in two elderly women. In both cases, morphological and immunohistochemical investigation showed two heterogeneous but intimately admixed neoplastic populations of vulvar epithelium. Atypical keratinocytes stained markedly and diffusely positive for high molecular weight cytokeratins, and moderately for p53 protein and c-erbB-2 immunostainings. Paget cells were diffusely positive for CEA, EMA, and low molecular weight cytokeratins, moderately and focally for c-erbB-2 and (in one case) for S-100. Morphological and immunohistochemical phenotypic differences between Paget cells and atypical keratinocytes suggest a simultaneous and incidental association of two distinct neoplastic disorders more than a mixed carcinoma in situ of vulvar epithelium.     

Pediatric Granular Cell Tumors of the Vulva: A Report of 4 Cases and a Review of the Literature

Background

Granular cell tumors (GCTs) are rare soft tissue lesions that can involve the female genital tract, including the vulva. Although malignant tumors are aggressive and uncommon, benign and atypical lesions still have associated risks, including recurrence and multisite development.

Liposomal doxorubicin for treatment of metastatic chemorefractory vulvar adenocarcinoma

BACKGROUND: Primaryadenocarcinoma of the vulva is a rare entity, and for widely metastatic vulvar adenocarcinoma, no effective treatment has been established. CASE: A 65-year-old woman was diagnosed with regionally advanced vulvar adenocarcinoma, with bulky involvement of bilateral groin lymph nodes, and associated extramammary Paget's disease. Initial therapy consisted of multiagent chemotherapy and vulvar and groin irradiation, followed by radical vulvectomy with groin and pelvic lymph node dissection. She subsequently developed widely metastatic disease including brain, pulmonary, hepatic, osseus, and subcutaneous lesions. Treatment with liposomal doxorubicin (Doxil) resulted in dramatic regression of metastatic lesions and marked improvement in quality-of-life. She remains clinically well, greater than 1 year since initiating Doxil treatment for widely metastatic vulvar adenocarcinoma, and has surpassed 5 years of survival since her initial diagnosis. CONCLUSIONS: We report the first case of Doxil used for the treatment of metastatic chemorefractory vulvar adenocarcinoma. We observed that Doxil was a well-tolerated and effective agent for this gynecologic malignancy, and warrants further investigation.