Paget's disease of the vulva

OBJECTIVE: Our goal was to review our experience with Paget's disease of the vulva relative to initial examination, treatment, and oncologic outcome. STUDY DESIGN: Patients who were treated for extramammary Paget's disease of the vulva at the University of South Florida were identified in our vulvar cancer database for the period 1988 through 2000. The charts were reviewed, and the data were collected regarding patient demographics, previous Paget's treatment, symptoms, surgical margin status, associated malignancies, and time to recurrence. RESULTS: Twenty-three women with extramammary Paget's disease of the vulva were treated by the Division of Gynecologic Oncology during the 12-year period. The average patient was postmenopausal and white and had symptoms for 21 months before the diagnosis was made. A pruritic lesion was the most common symptom. Treatment included wide local excision or vulvectomy, depending on the extent of disease. Six of the 23 patients displayed invasive disease and, consequently, underwent radical resection. There were 8 recurrences that were found, on average, 30 months after the surgical procedure. Two of the 8 patients had invasive disease at their primary operation, and 1 patient had underlying invasive disease at the time of recurrence. The average follow-up time was 39 months (median, 13.5 months; range, 1-216 months). CONCLUSION: Delay in diagnosis did not correlate with size or extent of disease. Margin status did not change the natural course of disease. Recurrence is relatively common, and long-term monitoring is recommended, with repeat excision of symptomatic lesions.     

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。     

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.     

Clinically inapparent invasive vulvar carcinoma in an area of persistent Paget's disease: a case report

BACKGROUND: Invasive vulvar carcinoma is reported to occur in 5 to 20% of patients with vulvar Paget's disease. We report a case in which a clinically inapparent invasive lesion was discovered on reexcision of microscopically persistent vulvar Paget's disease. CASE: A 58-year-old woman presented with a diagnosis of vulvar Paget's disease. A wide local excision of the lesion was performed and pathologic analysis revealed microscopic Paget's disease at two of the margins. The patient returned for a follow-up 4 months later and a vulvar biopsy revealing persistent Paget's cells was obtained from the area of the prior microscopically positive surgical margin. A reexcision was performed from the normal-appearing vulva and invasive vulvar carcinoma was noted in this specimen. CONCLUSIONS: This case demonstrates several concerning aspects of this disease, most important of which is that the clinically apparent lesion did not contain the clinically significant invasive lesion. Invasive vulvar carcinoma may occur in association with microscopically persistent vulvar Paget's disease, a condition often encountered after primary treatment with wide local excision.     

FOXP3+ regulatory T-cells are abundant in vulvar Paget's disease and are associated with recurrence

Objective.

To characterize clinical features of vulvar Paget's disease, and examine the quantity of immunosuppressive regulatory T-cells in vulvar Paget's tissue.

Methods.

Vulvar Paget's cases from 1992 to 2007 from two institutions were identified by pathology database search. Regulatory T-cells were identified with FOXP3 immunohistochemistry and quantified at the dermal-epidermal junction using image analysis software. Thirteen non-neoplastic inflammatory cases were stained for comparison.

Results.

Cases included 33 women treated for primary vulvar Paget's, and 7 referred at recurrence. Of the 24 primary cases with greater than 5 months follow-up, recurrence was documented in 12/24(50%). Eight women (20%) recurred multiple times, but no recurrences were invasive. Significantly more patients with positive margins developed recurrent disease (82% vs 23%, p = 0.01). Secondary neoplasms occurred in 10/40(25%). FOXP3+ cells at the dermal-epidermal junction were quantified in 29 primary and 13 recurrent tissue samples. FOXP3+ cells were absent in surrounding normal vulvar skin. FOXP3+ cells averaged 66/HPF in primary vulvar Paget's and 66/HPF in recurrent Paget's, compared to 22/HPF in non-neoplastic inflammatory cases (p = 0.0003, p = 0.001). Primary cases with positive surgical margins had more FOXP3+ cells than those with negative margins (85 vs 49, p = 0.01). Recurrent cases with positive margins had more FOXP3+ cells than negative cases (84 vs 33, p = 0.06). FOXP3 levels in primary specimens were higher in cases which recurred (78 vs 35, p = 0.02).

Conclusions.

Increased regulatory T-cells may be associated with more extensive cases of vulvar Paget's disease that result in positive surgical margins and are associated with recurrence of disease, suggesting immunosuppression as a key factor.     

Prognostic factors in Paget's disease of the vulva: a study of 21 cases.

Twenty-one cases of vulvar Paget's disease were studied to assess possible prognostic indicators, including presence and depth of invasion, status of resection margins, tumor DNA cell content, and immunoreactivity for p53 and estrogen receptor proteins. Immunostaining for cytokeratin 7 (CK7), cytokeratin 20 (CK20), and gross cystic disease fluid protein-15 (GCDFP) were also performed. Patients were 45 to 82 years of age (mean, 66.9 years). Ten of 21 patients (47.6%) had invasive Paget's disease. Dermal invasion was < or = 1 mm in 7 of 10 cases and 2 mm, 3 mm, and 8 mm in the remaining three invasive tumors. Of the seven patients with minimally invasive Paget's disease (< or = 1 mm depth of invasion), five are alive with no evidence of disease, one died of an unrelated illness, and one is alive with biopsy-proven in situ Paget's disease, having refused operative treatment. Of the three patients with more than minimally invasive Paget's disease (> 1 mm), all had nodal metastases; one patient is alive with no evidence of disease, one died of undertermined causes, and one died of metastatic Paget's disease. The remaining 11 patients had Paget's disease confined to the epidermis and its adnexal structures. Seven of these patients were alive at last follow-up with no evidence of disease. Of the remaining four patients, one died of metastatic cervical cancer, one died of metastatic bladder cancer, one died of an unrelated illness, and one patient is alive with biopsy-proven in situ Paget's disease and awaiting operative treatment. Twenty of the 21 cases represented primary vulvar Paget's disease while one represented possible local spread from a cervical adenocarcinoma. The immunoprofiles were GCDFP+/CK7+/CK20- in 14 cases, GCDFP+/CK7+/CK20+ in 4 cases, and GCDFP-/CK7+/CK20- in 2 cases. All tumors were estrogen receptor-negative. Immunostaining for p53 was positive in 16 tumors and negative in four tumors. Seven of 12 (58%) patients with positive margins experienced local recurrence of Paget's disease, while the disease recurred in 1 of 4 patients with negative margins. Recurrence was observed in 3 of 5 patients with diploid tumors and in 4 of 10 patients with aneuploid tumors. Neither of these differences is statistically significant. This study supports the recognition of a category of minimally invasive vulvar Paget's disease that has a low risk of distant metastasis and death caused by disease. Status of surgical resection margins, tumor cell DNA ploidy, estrogen receptor expression, and p53 immunoreactivity are not predictive of local recurrence.     

Testosterone versus clobetasol for maintenance of vulvar lichen sclerosus associated with variable degrees of squamous cell hyperplasia

OBJECTIVE: To evaluate the therapeutic regimens and symptomatic response rates in patients with vulvar lichen sclerosus associated with variable degrees of squamous cell hyperplasia (mixed disease). MATERIAL AND METHODS: Eighty-three women with biopsy-proven vulvar mixed disease were evaluated for this retrospective study. All patients were initially treated with topical fluorinated corticosteroids, and then 2% testosterone propionate in petrolatum or 0.05% clobetasol 17-propionate (44 (53%) versus 39 (47%)). RESULTS: The remission rates were 82 and 93% in the testosterone and clobetasol subgroups at the end of 6 months (p=0.112), respectively. The disease recurred in 8% of the patients. The recurrence rates in the testosterone and clobetasol arms were 13 and 5%, respectively (p=0.163). The histopathological review of the repeat vulvar biopsies of the patients without symptomatic relief revealed 6 (60%) patients with persistent disease, 2 (20%) with lichen sclerosus, 1 (10%) with atypical squamous hyperplasia, and 1 (10%) with VIN1. Two patients with recurrent disease and 2 patients with vulvar intraepithelial neoplasia I-II or atypical squamous hyperplasia were treated with skinning vulvectomy. CONCLUSIONS: Clobetasol resulted in higher remission and lower recurrence rates than those in testosterone therapy, although statistically significant differences were not obtained. In the evaluation of patients without symptomatic relief, the first step should be a vulvar biopsy to exclude the presence of atypical components.     

Clinical study and prognosis of 56 cases of vulvar intraepithelial neoplasia

OBJECTIVE: To study main clinical characteristics of patients with vulvar intraepithelial neoplasia 3 (VIN3). To investigate the long-term outcome and risk factors associated with recurrence or progression to invasive carcinoma. PATIENTS AND METHODS: Retrospective study of 56 patients with VIN3 from January 1st 1995 to December 31st 2003. RESULTS: Lesions were unifocal for 30 patients (53.6%) whereas they were multifocal for 26 patients (46.4%). When the lesion was multifocal, women were younger than in the unifocal group (41.2+/-16.7 vs. 52.5+/-13.5 years, P<0.03). Clinical symptoms, disease characteristics and medical history were not different between the two groups. Clinical HPV infections were more frequent in the multifocal group (65.4 vs. 23.3%, P<0.01). The mean follow-up was 39 months. Nine patients (16.1%) had recurrence of VIN3. Progression to invasive carcinoma occurred in 4 patients (7.1%). Multifocal lesions, occult micro-invasive disease and positive margins were related to recurrence or progression to invasive carcinoma. However, age at diagnosis, HPV infection, lichen sclerosis, immunosuppression and initial treatment did not correlate with evolution. DISCUSSION AND CONCLUSION: Recurrence and progression to invasive carcinoma can occur during VIN3 evolution. Our results confirm previous reports and suggest that all patients need a long-term follow-up regardless of patients' age or clinical characteristics.     

Paget''s disease of the vulva: clinicopathologic study of type 1 cases treated at a single institution

The aim of this study was to evaluate clinicopathologic characteristics of primary cutaneous Paget's disease of the vulva. Between 1986 and 2005, 22 patients with primary cutaneous Paget's disease of the vulva (type 1) were treated at Tohoku University Hospital. Medical records were reviewed for pathologic diagnosis, patient age, associated neoplasms, type(s) of eczema, symptom duration, treatment, surgical procedures, recurrence, and length of follow-up. Patient age ranged from 51 to 85 years (median 71.5 years). Median duration of symptoms was 24 months (range 2-60 months). Type 1a (intraepithelial) Paget's disease accounted for 18 patients, with 3 type 1b (invasive) cases and 1 type 1c (intraepithelial disease with underlying adenocarcinoma) case. Mean length of follow-up was 53.7 months, and median follow-up was 49 months (range 6-199 months). Only two patients had an associated internal malignancy: T-cell leukemia and breast cancer. Mapping biopsy was performed in 14 of the 18 type 1a cases. All patients were free of disease at the surgical margins and are alive without recurrence. The four patients with type 1b or 1c disease had lymph node metastases. Two has died of disease, and two are alive with no recurrence. The rate of secondary malignancy seems to be low in primary cutaneous Paget's disease of the vulva. Mapping biopsy with careful examination of characteristic skin surface may be useful for surgery of type 1a cases. Inguinal lymphadenectomy is recommended in cases with question of invasion or known underlying adenocarcinoma.     

Characteristics of recurrence in patients who underwent lymphatic mapping for vulvar cancer

OBJECTIVE: To evaluate patients with vulvar cancer who experienced a recurrence after undergoing lymphatic mapping and sentinel lymph node (SLN) biopsy. METHODS: We reviewed the records of 52 patients who underwent vulvectomy and lymphatic mapping with blue dye for treatment of vulvar cancer at our institution from 1993 to 1999 and identified patients who experienced recurrent disease. RESULTS: Fourteen (27%) of 52 patients experienced a recurrence. The patients' median age was 60 years (range 35-84 years). Nine patients had squamous lesions, four patients had melanoma, and one patient had Paget's disease with stromal invasion. Four tumors were stage T1, seven were T2, and three were T3. Eight lesions were located at the midline and six were lateral. Thirteen patients underwent superficial inguinal lymphadenectomy while one patient underwent SLN biopsy only. Postoperatively, seven patients underwent no further treatment, six underwent radiation therapy, and one patient underwent chemotherapy. The median follow-up was 46 months and the median disease-free interval was 21 months. Primary recurrence was in the vulva in eight patients (57%), in the groin in three patients (21%), and distant in three patients (21%). Nine of 32 (22%) squamous lesions recurred, four (57%) of seven melanomas recurred, and the sole patient with invasive Paget's recurred. Patient weight was found to be significantly different between patients who experienced a recurrence and those who did not (P = 0.05). At least one SLN was identified in 46 (88%) of the 52 patients. One (17%) of six patients in whom no SLN was identified experienced a recurrence, and 13 (28%) of 46 patients in whom a SLN was identified experienced a recurrence (P = 0.5). In the 41 patients with negative SLNs and negative non-SLNs, the recurrence rate was 24%; in the six patients with positive SLNs and negative non-SLNs, the recurrence rate was 40%; and in the five patients with positive SLNs and positive non-SLNs, the recurrence rate was 40% (P = 0.6). No patients had a negative SLN and positive non-SLN. Of the three patients who experienced a recurrence in the groin, one had a negative SLN and negative non-SLN, one had a positive SLN and positive non-SLN, and one had no SLN identified and a negative non-SLN. CONCLUSIONS:. This heterogeneous group of patients who underwent lymphatic mapping with blue dye had similar patterns of recurrence to reported series of patients who did not undergo lymphatic mapping. Groin relapse following a negative SLN biopsy is of concern and suggests that long-term follow-up data are required before lymphatic mapping and SLN biopsy alone can be considered standard treatment for patients with vulvar cancer.     

Therapeutic value of trichloroacetic acid in the treatment of isolated genital warts on the external female genitalia

OBJECTIVE: To evaluate the value of 85% trichloroacetic acid (TCA) in the treatment of human papillomavirus (HPV)-associated genital warts of the external genitalia and to detect the recurrence rate and side effects of this therapeutic regimen. STUDY DESIGN: All patients with a suspected HPV-related papillary vulvar lesion after initial examination underwent vulvoscopic evaluation with a magnification of 8-20x using acetic acid and toluidine blue. Under local anesthesia, biopsies were taken from acuminate or papillary warts for histopathologic confirmation and from suspicious areas to exclude preinvasive or invasive diseases. Following histopathologic diagnosis, patients were treated with 85% TCA. RESULTS: Overall, 51 patients with isolated vulvar and/or perianal genital warts were included. Of those patients, 11 (21.5%) had acuminate and 40 (78.5%) had papular genital warts. All the women had lesions of the labia minora. The other localizations were as follows: labia majora, 18 (35.3%); lateral vulva, 5 (9.8%); clitoris, 9 (17.6%); fourchette, 16 (31.3%); and perianal area, 7 (13.7%). All lesions were successfully treated by the end of the treatment period (median, 4; range 2-5). None of the patients had recurrence or new lesions during the 6-month follow-up period. In the second 6 months, 9 patients (17.6%) were diagnosed with recurrent lesions. Although all the patients experienced transient burning pain during therapy, none of them discontinued the therapy. Ulceration was observed in 8 patients (15.6%). Of those patients only 3 had permanent scarring (5.8%). CONCLUSION: We recommend the use of TCA in patients with external genital warts, especially for mild to moderate cases. It is associated with a high success rate and low morbidity if sufficient care is taken during application.     

Margin distance and other clinico-pathologic prognostic factors in vulvar carcinoma: a multivariate analysis

OBJECTIVES: To determine the importance of margin status and other prognostic factors associated with the recurrence and survival of patients with squamous cell vulvar carcinoma. METHODS: Data were analyzed using Kaplan-Meier methods and Cox proportional hazards regression. All slides were re-reviewed by two gynecologic pathologists. RESULTS: Ninety patients (median age: 69) were treated for vulvar carcinoma from 1984 to 2002, including 28 FIGO stage I, 20 stage II, 26 stage III and 16 with stage IV disease. Sixty-three (70%) patients underwent complete radical vulvectomies and 27 (30%) had modified radical vulvectomies. Nineteen (20%) patients received adjuvant radiotherapy. Five-year disease-specific survival rates were 100%, 100%, 86% and 29% for stages I-IV, respectively. None of the 30 patients with a pathologic margin distance >8 mm had local recurrence. Of the 53 women with tumor-free pathologic margin of <8 mm, 12 (23%) had a local recurrence. Moreover, women with >2 positive groin nodes had significantly higher recurrence risk compared to those with <2 metastatic groin nodes (p<0.001). On multivariate analysis, positive groin nodes and margin distance were important prognostic factors for recurrence. Moreover, stage, tumor size, margin distance, and depth of invasion were significant independent predictors for disease-specific survival. The median follow-up was 58 months (range: 2-188). CONCLUSIONS: Pathologic margin distance is an important predictor of local vulvar recurrence. Our data suggest that a > or =8-mm pathologic margin clearance leads to a high rate of loco-regional control.     

Vulvar Paget disease. Two cases with cytokeratin 7 and 20 expression

Two cases of vulvar Paget's disease are described in two women aged 75 and 60 years, with onset several years earlier as eczema-like manifestations, and evolving into erosive, slightly infiltrative lesions. In both cases immunohistochemical examination revealed positivity for cytokeratins CK7 and CK20. This finding suggested the diagnosis of primitive vulvar Paget's disease, a relatively benign form, unlike the aggressive and rapidly progressive secondary vulvar Paget's disease.     

Vulvar intraepithelial neoplasia: aspects of the natural history and outcome in 405 women

OBJECTIVE: To determine the clinical characteristics, outcome following surgical treatment, and natural history of cases of untreated vulvar intraepithelial neoplasia (VIN). METHODS: Four hundred five cases of VIN 2-3 seen between 1962 and 2003 were reviewed. RESULTS: The mean age of women with VIN decreased from 50.0 years before 1980 to 39.0 in subsequent years. After treatment of VIN by excision and/or laser vaporization, half the women required at least one further treatment by the 14th year. It is estimated that 50% of women with positive surgical margins had at least one further treatment within 5 years, but only 15% of women with negative surgical margins required further treatment. Invasive vulvar, perianal, or urethral carcinoma occurred in 17 (3.8%) women (mean age 42 years) after treatment. Nine (2%) cases represented treatment failure, with a median treatment-to-invasion interval of 2.4 years. Eight (1.8%) cases represented new "field" carcinomas, with a median initial treatment-to-invasion interval of 13.5 years. Ten untreated cases, aged 32-76 years (mean age 55 years), progressed to invasion in 1.1-7.3 years (mean 3.9 years). Age, previous pelvic radiotherapy, unifocality, immunosuppression, and association with multicentric neoplasia were not risk factors for the development of invasive vulvar carcinoma in this study. Spontaneous regression of VIN occurred in 47 women (mean age 24.6 years), with a median interval to complete regression of 9.5 months. CONCLUSION: Surgically treated VIN has a high rate of recurrence. Untreated VIN in women over 30 years has an appreciable invasive potential.     

Transitional cell carcinoma of the bladder mimicking recurrent paget's disease of the vulva: report of two cases, with one occurring in a myocutaneous flap.

BACKGROUND: Transitional cell carcinoma of the bladder may spread superficially along and beyond the urogenital epithelium, mimicking vulvar Paget's disease. CASES: These two cases illustrate unusual aspects of transitional cell carcinoma of the bladder and vulvar Paget's disease. Both patients had a history of breast cancer and previously had multiple operations for recurrent vulvar Paget's disease; one patient had a radical vulvectomy with transverse rectus abdominal muscle flap reconstruction. Both had a history of recurrent transitional cell carcinoma of the bladder. Both presented with recalcitrant transitional cell carcinoma of the bladder and clinically recurrent vulvar Paget's disease. Pathologic evaluation, however, revealed pagetoid spread of carcinoma in situ (CIS) throughout the urothelium, with an invasive component in the cervix and extension of the CIS into the rectum in one patient. CONCLUSION: If the history of the patient includes transitional cell carcinoma of the bladder and vulvar Paget's disease, histologic evaluation is needed for accurate diagnosis and proper treatment.     

Invasive vulvar Paget's disease.

Seven cases of invasive vulvar Paget's disease are presented. These are compared with 10 cases of intraepithelial vulvar Paget's disease. On the basis of both clinical and histopathologic observations, the concept of intraepithelial vulvar Paget's disease is supported, and this entity is distinguished from the invasive form. Therapy for the latter is discussed.     

Photodynamic diagnosis of vulvar precancerous conditions and invasive cancers using 5-aminolevulinic acid

BACKGROUND: Photodynamic therapy (PDT), in particular Photodynamic diagnosis (PDD) is a modern, non-invasive technique using photosensitizer like 5-aminolevulinic acid (5-ALA) in detection of the vulvar diseases. Photodynamic therapy gives the possibilities to differentiate inflammatory diseases from precancerous lesions and invasive vulvar cancer. OBJECTIVE: To assess accuracy of the PDD in detection, localisation and differentiation of precancerous diseases and invasive cancer of the vulva. MATERIAL AND METHODS: This study included 107 patients with vulvar disorders, 30 patients with VIN I, 31 patients with VIN II, and 46 patients with VIN III and vulvar carcinoma. All women underwent a standard gynaecologic examination. 5-ALA was topically applied to the vulva. After 180-360 minutes the vulvar skin was illuminated by a short--are xenon lamp at 380-440 nm and an output of 200 mW (D-Light; Karl storz). A filter to select the emitted wavelength range 630-670 nm was used in order to achieve differentiation's of the fluorescence--positive and negative areas. After the PDD, biopsies were taken from patients with multifocal VIN lesions. Macroscopic appearance, fluorescence pattern in scale of three degrees (zero, +, ++) and histology of the lesions were compared. RESULTS: The sensitivity, specificity, positive and negative predictive value for the detection of VIN I were 85.7%, 81.2%, 80.0%, 86.6% respectively, for VIN II there were 93.3%, 93.7%, 93.3% and 93.7%, and for VIN III and invasive vulvar cancer there were 96.3%, 94.7%, 96.3% and 94.7% respectively. The sensitivity, specificity, positive and negative predictive value for the detection of all VIN and invasive vulvar cancer common were 92.9%, 90.2%, 91.2%, 92.0 respectively. CONCLUSION: The Photodynamic therapy becomes a valuable, non-invasive diagnostic tool that lowers the amount of false negative diagnosis in cases of VIN and vulvar cancer.     

Vulvar carcinoma in premenopausal Jamaican women

Between the years 1958 and 1973, 119 patients with primary invasive cancer of the vulva were treated at the University Hospital of the West Indies, Kingston, Jamaica. Seventy-seven (64.7%) were premenopausal women. Because the pattern of the disease differed from that in late middle and older aged Jamaican women, the results of the disease are presented. The analysis stresses that vulvar carcinoma before menopause in our community is rare in the absence of various sexually transmitted granulomatous diseases of the vulva.     

Paget's disease of the vulva

Thirty-six patients with Paget's disease of the vulva were reviewed. Median age of the patients at diagnosis was 64 years (range 41-84 years). Five patients (14%) had an associated invasive adenocarcinoma of the vulva at the time of diagnosis. Of 31 patients with superficial noninvasive Paget's disease, 28 were available for follow-up. Treatment of patients with superficial Paget's disease was surgical and based on the extent of disease. Procedures performed included total vulvectomy (25), wide local excision (4), and skinning vulvectomy with skin graft (1). The median follow-up was 108 months (range 6-266 months). Twenty-two of twenty-eight patients remained free of disease. Six patients have required multiple procedures for recurrent superficial Paget's disease. Treatment of Paget's disease of the vulva is surgical. Radical surgery is the preferred treatment of patients with an associated invasive adenocarcinoma; three of five patients with an invasive adenocarcinoma were long-term survivors. Patients with superficial Paget's disease of the vulva should be treated by local excision utilizing frozen-section margin evaluation as a guide to extent of excision. Only one patient with an initial diagnosis of superficial Paget's disease of the vulva developed invasive adenocarcinoma.     

Photodynamic therapy using a methyl ester of 5-aminolevulinic acid in recurrent Paget's disease of the vulva: a pilot study

OBJECTIVE: In the past, treating vulvar Paget's disease through surgery has resulted in a high recurrence rate of the disease. Photodynamic therapy (PDT) using 5-aminolevulinic acid (5 ALA) is an effective treatment for some conditions such as Bowen's disease, subsets of basal cell carcinomas and vulvar carcinoma. Methyl 5-aminolevulinate (MAL) is an ester of 5 ALA that seems to be more effective and produces fewer side effects than 5 ALA. This paper outlines a pilot study designed to test the feasibility of using MAL-PDT in the treatment of recurrent vulvar Paget's disease. METHODS: 5 MAL-PDT was applied for 3 h and than irradiated with red-light (620 nm) using a total light dose of 37 J/cm2 for a period of 10 min. Patients taking part in the study were treated once every 3 weeks, for a total of three treatments. Vulvar biopsies were obtained before and 1 month after the PDT-treatment. RESULTS: Seven patients were enrolled in the study. Four cases had a complete clinical response, and this was pathologically confirmed in two of the cases. The cosmetic outcome was acceptable and the treatment was well tolerated. All the patients developed local edema and mild local pain, controlled with non-steroidal antiinflammatory drugs (NSAIDS). One patient experienced severe pain and a mild local phototoxicity reaction. CONCLUSIONS: MAL-PDT is a feasible treatment and seems to offer a reliable strategy in the control of vulvar Paget's disease and of its symptoms.