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To examine the association between surgical margin status and recurrence pattern in invasive vulvar Paget's disease.This is a preplanned secondary analysis of a previously organized nationwide retrospective study in Japan (JGOG-1075S). Women with stage I-IV invasive vulvar Paget's disease who received surgical treatment from 2001-2010 were examined (n=139). Multivariable analysis was performed to assess local-recurrence, distant-recurrence, and all-cause mortality based on surgical margin status.The median age was 70 years. The majority had stage I disease (61.2%), and the median tumor size was 5.0cm. Nodal metastasis was observed in 15.1%. Simple vulvectomy (46.0%) was the most common surgery type followed by radical vulvectomy (28.1%). More than half received vulvar reconstructive surgery (59.0%). Positive surgical margin was observed in 35.3%, and close margin1cm was observed in 29.5%. Vulvectomy type was not associated with surgical margin status (P=0.424). The median follow-up was 5.8 years. Positive surgical margin was associated with increased local-recurrence (5-year cumulative rates for positive versus negative margin: 35.8% versus 15.0%, P=0.010) but not distant-recurrence (18.3% versus 16.0%, P=0.567). Positive surgical margin was also associated with increased all-cause mortality (5-year overall survival rates for positive versus negative margin: 72.6% versus 88.2%, P=0.032). In multivariable analysis, positive surgical margin remained an independent factor associated with increased risk of local-recurrence (hazard ratio 2.80, 95% confidence interval 1.18-6.63) and all-cause mortality (hazard ratio 2.87, 95% confidence interval 1.20-6.83).Positive surgical margin appears to be common in invasive vulvar Paget's disease that is associated with increased local-recurrence and all-cause mortality risks. Role of alternative surgical technique or adjuvant therapy merits further investigation to improve local disease control.

High-quality data are lacking to understand outcomes in women who undergo labiaplasty for labial hypertrophy and whether there is an association with body dismorphic disorder (BDD). This was a cross-sectional study of a retrospective cohort of women who underwent labiaplasty for labial hypertrophy at a tertiary care referral center. Women were identified by CPT codes and were included if the procedure was performed by a urogynecologist. The health record was queried for demographic, pre-, intra-, and postoperative data. For the cross-sectional component of the study, participants were contacted to complete a survey. Thirty-six women met study inclusion criteria; 21 participated in the survey, and retrospective data were available for 20. At the time of labiaplasty, the women had a mean age of 30 ± 12 years and mean BMI of 23.9 ± 4.4 kg/m2. The most common preoperative motivation for undergoing labiaplasty, based on the medical record, was pain (85.0% 17/20). Complications were uncommon and minor. No women met criteria for BDD. Participant survey-reported primary motivation for undergoing partial simple vulvectomy was “pinching” in 10 (47.6%), “pain” in 4 (19.0%), and “appearance” in 7 (33.3%). The majority of women reported that labiaplasty achieved their preoperative goal (20/21, 95.2%). Women who underwent labiaplasty were primarily motivated by functional concerns, and outcomes indicate a high satisfaction with genital appearance postoperatively as well as a positive effect on body image and quality of life. We recommend that surgeons work to understand what motivates women to pursue surgical intervention by asking about cosmetic concerns.

Introduction Little is known about VC in Ethiopia. Our aim was to describe demographic and clinical characteristics of VC patients treated at TASH. Methods This is a retrospective study. A structured questionnaire was used to gather data about patients with histologically-confirmed primary VC seen at TASH from 8/30/2012 to 8/30/2017.Data was analyzed using SPSS software. Results There were a total of 118 patients. The median/mean age was 39/43.17years. Patients presented with more than one symptom, including mass (76%) and ulcer (48%), with a mean duration of 2 years. In 80% of patients, VC extended to adjacent organs including vagina (47%), anus (30%), urethra (23%) and inguinal lymph nodes (46%). 66% of women were HIV-positive with a mean infection time of 72 months and all were taking HAART. 64% of HIV-positive patients had locally-advanced disease. Patients were treated with chemo-radiation (32%), surgery (17%), surgery with adjuvant radiation (2%) and radiation alone (14%). Surgery was simple vulvectomy and bilateral inguinal LND (10%), radical vulvectomy and bilateral inguinal LND (8%) and simple vulvectomy(5%). Five of 22 developed complications including wound infection (2), vaginal stenosis (2) and wound breakdown with delayed healing, lymphocyst and altered skin pigmentation (1). Conclusion VC at TASH is a burden of mainly young and HIV-positive patients with late-stage disease despite receiving active HIV health-care and long-standing symptoms. Delivering education about VC to patients, particularly HIV-positive ones, and to HCPs may ameliorate the disease burden in Ethiopia. HPV serotype study and vaccination could also impact the reduction of VC.

Objectives We aimed to analyze the outcomes of patients who underwent vulvectomy with subsequent V—Y fasciocutaneous flap reconstruction. Methods All medical records of all patients who underwent vulvectomies with V—Y fasciocutaneous flap reconstruction from January 2007 to June 2016 were retrospectively reviewed. Patient clinical and surgical data, demographics, and outcomes were abstracted. Results Of the 27 patients, 42 flaps were transferred. A simple vulvectomy was performed in 8 (30%) patients, partial radical vulvectomy in 15 (56%), and radical vulvectomy in 4 (15%). The median area of defect was 30 cm2. Minor wound separations occurred in 9 patients (33%). Infectious complications occurred in 4 patients (15%); this included urinary tract infections in 2 (50%), postoperative fevers in 2 (50%), and sepsis in 1 (25%) patient with a UTI. There were no instances of flap necrosis, wound dehiscence, or wound infections. Black race was more likely to be associated with an infectious complication with 3 (75%) patients, compared to white race with 1 (4%) patient (p, Highlights • A V—Y fasciocutaneous flap for vulvar reconstruction is a feasible alternative to primary closure. • Minor wound separations and infectious complications were most common. • It is a safe procedure with no instances of flap necrosis, wound dehiscence, or wound infections.

The correct wound classification for vulvar procedures (VP) is ambiguous according to current definitions, and infection rates are poorly described. We aimed to analyze rates of surgical site infection (SSI) in women who underwent VP to correctly categorize wound classification.Patients who underwent VP for dysplasia or carcinoma were collected from the National Surgical Quality Improvement Program database (NSQIP). SSI rates of vulvar cases were compared to patients who underwent abdominal hysterectomy via laparotomy, stratified by the National Academy of Sciences wound classification. Descriptive analyses and trend tests of categorical variables were performed.Between 2008 and 2016, 2116 and 31,506 patients underwent a VP or TAH, respectively. Among VP, 1345 (63.6%), 364 (17.2%), and 407 (19.2%) women underwent simple vulvectomy, radical vulvectomy, or radical vulvectomy with lymphadenectomy, respectively. The overall rate of SSI for VP was higher than that observed for TAH (5.6% vs. 3.8%; p 0.0001). While patients undergoing TAH displayed a corresponding increase in the rate of SSI with wound type (type I: 3.4%; type II: 3.8%, type III: 6.8%; type IV 10.6%; p 0.001), no such correlation was observed for simple VP (type I: 3.3%, type II: 3.0%; type III: 3.2%; type IV: 0%; p = 0.40). On the other hand, a non-significant correlation was observed for radical VP (type I: 4.0%, type II: 10.1%; type III: 14.3%; type IV: 20.0%; p = 0.08). The overall rate of SSI in patients undergoing any radical VP was similar to patients undergoing hysterectomy with a type IV wound (10.1% vs 10.6%, p = 0.87).Patients undergoing VP are at high risk of infection. Simple vulvectomy should be classified as a type II and radical vulvectomy as a type III wound. These recommendations are important for proper risk adjustment.

Highlights • Surgical approach is standard of care, topical imiquimod still experimental for mucosal melanoma. • Imiquimod beneficial for local control of disease if surgery not an option for MIS of vulva/ vagina. • In situ and invasive vulvovaginal melanoma have a high risk for recurrence even years later. • Long-term follow-up after topical imiquimod treatment needed as metastasis can arise years later., The primary treatment of both in situ and invasive vulvar melanoma is wide local excision of the primary neoplasm. However, this can be a surgical challenge for size, multifocal presentation with proximity to urethra or anus and tendency for local recurrence. The data on adjuvant therapy for vulvar MIS is very limited. A 69-year-old patient with melanoma of the vulva underwent a simple vulvectomy with positive margins in peri-clitoral area, followed by modified radical vulvectomy and bilateral inguinofemoral sentinel lymph node dissection with negative margins. She was later diagnosed with MIS of the vulva on different locations and had multiple wide local excisions over several years. One lesion was close to the urethra and a complete excision was difficult. Topical imiquimod × 16 weeks (5% cream) was given. The regimen was augmented from 3 to 5 times weekly. Complete resolution was found at 16 weeks and patient was disease free for 4 years. Recently however, a vaginal melanoma was detected. Imiquimod appeared to be beneficial in the treatment of melanoma in situ of the vulva/ vagina when surgical options were not feasible producing local control of disease with the remaining risk for local and distant metastasis. Metastasis can appear years later, therefore long-term follow-up of patients treated with topical imiquimod is needed.

Context: Vulvar cancer is one of the uncommon gynecological malignancies. Multimodality treatment with surgery, radiotherapy, and chemotherapy are required for treatment of the disease. Aims: The aim of the study was to evaluate clinical outcome in patients of carcinoma vulva, treated at our institution. Subjects and Methods: This was a retrospective-cohort study done in 50 patients with squamous cell carcinoma of the vulva, treated at our institution from January 2008 to December 2014. Data were analyzed on the basis of age, stage, type of treatment received, and treatment-related toxicity. Disease-free survival and overall survival were estimated. Statistical Analysis Used: Kaplan–Meier survival analysis and Chi-square test were used for statistical analysis. Results: Majority of the patients (52%) had presented with Stage III disease. Thirty-six of 50 patients underwent surgery: simple vulvectomy – 2, radical vulvectomy – 34, bilateral inguinal lymph node dissection was done in 32 patients, and 1 patient underwent ipsilateral-inguinal lymph node dissection. Among 40 patients who received radiotherapy and eight patients received palliative radiotherapy. Seventeen patients underwent intensity-modulated radiotherapy (IMRT) and 15 patients received conventional radiotherapy. Significantly less Grade 2 or more skin toxicity (P = 0.003) observed in patients who underwent IMRT. Among non-IMRT group, eight patients required treatment break during radiation. At a median follow-up time of 25.5 months, median overall survival was 31 months and median disease-free survival was 25 months. About 42% patients were alive and free of disease at last follow-up. Conclusions: Modified radical vulvectomy with inguinal lymph node dissection followed by radiotherapy is the mainstay of management of locally advanced carcinoma vulva. Using IMRT, we could minimize the treatment related radiation toxicity and treatment breaks.

El carcinoma de células basales de la vulva es una lesión muy rara, y ocupa el cuarto lugar en el total de los tumores malignos de la vulva atendidos en el Instituto Nacional de Enfermedades Neoplásicas de Lima, con una incidencia de 2,7 por ciento. El simple Vulvectomía es la más adecuada por parte de la terapia cuando el carcinoma de células basales está presente por sí mismo, es decir, puro. La vulvectomía radical y disección de los ganglios regionales bilaterales deben practicarse en todos los casos de lesiones basales células escamosas.

Paget's disease can arise in the breast (mammary Paget disease) or in other locations (extramammary Paget disease) such as anogenital skin in both males and females (Paget disease of the vulva [PDV]). Underlying adenocarcinoma can be found in some cases. This study aims to report clinical aspects, surgical procedures, outcomes, and recurrences of patients with PDV. A retrospective chart review was conducted on patients with pathologically confirmed diagnosis of PDV managed at the Department of Obstetrics and Gynecology, University of Bari, and the “Giovanni Paolo II” National Cancer Institute in Bari, between 1998 and 2018. Records of 24 cases of PDV were examined. Median age of the patients at diagnosis was 69.3 (range 38–84), diagnosis of synchronous cancer was made in 2 cases and in 2 other cases of metachronous disease. Three patients had previously been diagnosed with other oncological diseases. All patients underwent surgery including wide local excision (6), simple vulvectomy (8), and extended vulvectomy (10). Lymphadenectomy was performed in 2 cases and reconstructions with advancement flaps in 7 cases. Four patients were found to have invasive disease and 1 had inguinal node involvement. Positive margins were found in 11 patients. Wound dehiscence and urethral stenosis were found in 4 and 1 case each. Eight recurrences (33.33%) were observed, regardless of positive surgical margins. PDV has a low rate of malignancy but a high rate of recurrence. It should be diagnosed early to avoid repeated surgery and to reduce symptoms and morbidity.

Lymphangioma circumscriptum (LC) is a form of lymphangioma characterized by benign dilation of lymphatic channels, which affects the skin and subcutaneous tissues. The most common sites are mucosa of mouth, tongue, groin, axilla, trunk and proximal region of extremities. Vulva is a rare site of LC. These abnormal lymphatic malformations do not communicate to the normal lymphatics. A female patient, aged 17 years, presented with papule-like condyloma of the labia majora. After simple vulvectomy was performed, symptoms improved. Histology revealed lymphangioma circumscriptum of the vulva. LC is a rare benign pathology that should be kept in mind in any vulval condylomatous lesion. Till date, only a few cases of LC of vulva have been reported in the literature. The treatment of primary vulvar lymphangioma circumscriptum is mainly surgery. Depending on the age of the patient and the extent of lesion, surgical treatment might be in the form of the local excision, labiectomy, or vulvectomy.

Primitive neuroectodermal tumors (PNETs) exhibit chromosomal translocations in common with those of Ewing's sarcoma. They usually originate in bone or soft tissue but rarely arise in the vulva. The current case report presents a case of PNET originating in the vulva in a 60-year-old female, who previously underwent enucleation of a vulvar tumor in another hospital. The pathologist suspected a histopathological diagnosis of PNET, and simple vulvectomy and resection of the inguinal lymph nodes were performed. An ~3 cm mass recurred in the right side of the vulva four years following the initial surgery and the tumor was excised. The tumor comprised small, round-to-oval nuclei and stained positively for MIC-2, synaptophysin, neuron-specific enolase and neurofilament antibodies. To date, the patient remains alive and with no evidence of disease four years following multidisciplinary treatment, despite PNETs usually exhibiting a poor prognosis. This is due to the small tumor size and the absence of distant metastasis.

Surgery is the standard in the management of vulvar cancer. Several studies assessed the feasibility of radiochemotherapy as definitive therapy and/or neoadjuvant procedure in order to limit the extent of surgery. Combined radiochemotherapy is associated with considerable toxicity. The authors report on a modified neoadjuvant radiochemotherapy schedule which is isoeffective to the GOG (Gynecologic Oncology Group) protocol, but associated with less therapy-related toxicity. A 36-year-old woman with stage IV vulvar cancer and long-distance rectal infiltration is reported. Laparoscopic pretherapeutic staging confirmed pelvic and paraaortic lymph node metastases. The patient received a neoadjuvant radiochemotherapy. After complete remission, a simple vulvectomy carried out 3 months later showed no evidence of tumor. Up to now, there is no evidence of tumor progression or recurrence. Preoperative conventionally fractionated simultaneous radiochemotherapy seems to be a feasible and safe treatment option for patients with locally advanced vulvar cancer in order to avoid exenterative surgery.

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.

Aim: To evaluate the coexistence of verrucous and squamous carcinoma of the vulva and to assess the clinical course, survival and rate of recurrent disease of these patients. Methods: The records of 17 patients who were diagnosed with verrucous carcinoma of the vulva over a 12-year period were studied retrospectively. Presence of genuine verrucous carcinoma or coexistence of verrucous and squamous carcinoma of the vulva on vulvar biopsies, results of histopathological assessment of final vulva and inguino-femoral node specimens and histological evaluation of recurrent disease specimens were the main outcome measures. Results: Five of the 17 patients (29.5%) initially underwent radical vulvectomy and inguino-femoral lymphadenectomy. Histology of the specimens verified the coexistence of verrucous and squamous carcinomas in four of the five cases. Twelve women (70.5%) underwent simple vulvectomy for genuine verrucous carcinoma; in the final histology, 10 of these women (58.8%) were confirmed as having genuine verrucous carcinomas while two (11.7%) were found to have both verrucous and squamous carcinomas and were further managed by lymphadenectomy. None of our patients died of the disease. Three women (17.5%) presented with local relapse of the tumour, and were managed by wide local excision of the tumour. Conclusions: In the present study, 35% of patients with verrucous carcinomas of the vulva had coexistent squamous carcinoma. Separation of the cases of genuine verrucous carcinoma from coexistent verrucous and squamous tumours is based on the establishment of correct diagnosis by a large and deep vulvar biopsy as well as the meticulous assessment of the specimen by the pathologist. This will result in the decrease of the rate of over- and under-treatment of these patients.

Background Apocrine carcinoma of the vulva is extremely rare; only two cases have been reported worldwide. Here we report a case of apocrine carcinoma of the vulva. Case A 58-year-old woman complaining of a small, asymptomatic genital tumor visited the gynecology clinic of Chiba Social Insurance Hospital. The biopsy specimen suggested that it was an adenocarcinoma derived from the apocrine gland. The diagnosis was confirmed by periodic acid–Schiff staining and immunohistochemical staining for gross cystic disease fluid protein 15. She underwent simple vulvectomy with hemilateral groin dissection. She has been followed as an outpatient for the past 7 years with no signs of recurrence or metastasis. Conclusion Despite the positive outcome of this case, small, asymptomatic genital lesions should be regarded with caution.

Summary Bowenoid papulosis of the vulva is an old term. Nowadays it must be included in grade III intraepithelial vulvar neoplasia (VIN III), since in the vulva it is considered to be an in situ cancer. Its incidence has increased in the last 25 years, especially in young women. The causes are not exactly known, however it is known that HPV infection, especially type 16 is an important triggering factor. Diagnosis is made by clinical suspicion. It manifests with the presence of hyperpigmented papulose lesions that can be asymptomatic or produce pruritis or vulvodinia. Confirmation of the diagnosis will always be made using biopsy and hystological study of the lesions. It may progress to invasive cancer, especially when the lesions are extensive, in young and immunosupre-sed women, and in smokers. These lesions can coexist with an occult invasive cancer; this is more frequent when the lesions are extensive, and also in elderly women. Treatment is with wide excision taking skin margins, or with simple vulvectomy if the lesions are not extensive, if the lesions are extensive radical vulvectomy and skin grafting are necessary.

Objective. To preserve the organs and function of the anus and ureter, radiotherapy was chosen for an advanced vulvar tumor originating from Bartholin's gland. Methods. The patient was a 74-year-old female with stage III (FIGO) vulvar cancer derived from the left Bartholin's gland. She received 63 Gy to the vulvar lesion with external beam irradiation followed by 30 Gy of high-dose-rate interstitial brachytherapy. Results. One year after radiotherapy, simple vulvectomy and reconstructive surgery were performed. The anal and ureteral functions were preserved. Histological examination revealed no evident malignant cells. Conclusion. Owing to the technological development of radiotherapy and improved reconstructive surgery, radiotherapy in treatment of the vulvar area has become effective and safe and could be indicated more as a treatment option for the sake of organ preservation.

Purpose: This report reviews the increasing role of radiation therapy in the management of patients with histologically confirmed vulvar carcinoma, based on a retrospective analysis of 68 patients with primary disease (2 in situ and 66 invasive) and 18 patients with recurrent tumor treated with irradiation alone or combined with surgery. Methods and Materials: Of the patients with primary tumors, 14 were treated with wide local excision plus irradiation, 19 received irradiation alone after biopsy, 24 were treated with radical vulvectomy followed by irradiation to the operative fields and inguinal–femoral/pelvic lymph nodes, and 11 received postoperative irradiation after partial or simple vulvectomy. The 18 patients with recurrent tumors were treated with irradiation alone. Indications and techniques of irradiation are discussed in detail. Results: In patients treated with biopsy/local excision and irradiation, local tumor control was 92% to 100% in Stages T1-3N0, 40% in similar stages with N1-3, and 27% in recurrent tumors. In patients treated with partial/radical vulvectomy and irradiation, primary tumor control was 90% in patients with T1-3 tumors and any nodal stage, 33% in patients with any T stage and N3 lymph nodes, and 66% with recurrent tumors. The actuarial 5-year disease-free survival rates were 87% for T1N0, 62% for T2-3N0, 30% for T1-3N1 disease, and 11% for patients with recurrent tumors; there were no long-term survivors with T4 or N2-3 tumors. Four of 18 patients (22%) treated for postvulvectomy recurrent disease remain disease-free after local tumor excision and irradiation. In patients with T1-2 tumors treated with biopsy/wide tumor excision and irradiation with doses under 50 Gy, local tumor control was 75% (3 of 4), in contrast to 100% (13 of 13) with 50.1 to 65 Gy. In patients with T3-4 tumors treated with local wide excision and irradiation, tumor control was 0% with doses below 50 Gy (3 patients) and 63% (7 of 11) with 50.1 to 65 Gy. In patients with T1-2 tumors treated with partial/radical vulvectomy and irradiation, local tumor control was 83% (14 of 17), regardless of dose level, and in T3-4 tumors, it was 62% (5 of 8) with 50 to 60 Gy and 80% (8 of 10) with doses higher than 60 Gy. The differences are not statistically significant. There was no significant dose response for tumor control in the inguinal–femoral lymph nodes; doses of 50 Gy were adequate for elective treatment of nonpalpable lymph nodes, and 60 to 70 Gy controlled tumor growth in 75% to 80% of patients with N2-3 nodes when administered postoperatively after partial or radical lymph node dissection. Significant treatment morbidity included one rectovaginal fistula, one case of proctitis, one rectal stricture, four bone/skin necroses, four vaginal necroses, and one groin abscess. Conclusions: Irradiation is playing a greater role in the management of patients with carcinoma of the vulva; combined with wide local tumor excision or used alone in T1-2 tumors, it is an alternative treatment to radical vulvectomy, with significantly less morbidity. Postradical vulvectomy irradiation in locally advanced tumors improves tumor control at the primary site and the regional lymphatics in comparison with reports of surgery alone.

A 28-year-old multiparous lady presented to the Gynaecology outpatient department with a 12 × 5 cm warty growth in the vulva. A biopsy of the growth revealed condyloma acuminata of the vulva. Simple vulvectomy was done. A PCR of the specimen detected the presence of human papilloma virus (HPV)-16 which is usually considered as a high-risk HPV type for carcinogenesis.

Purpose: The objective of our study was to demonstrate differences in relapse rates, total survival times, and complication rates between inguinofemoral radiation and its absence in cases of invasive vulvar carcinoma without lymph node involvement (FIG0 Stages Tl, NO-l). Methods and Materials: From 1974 to 1990, 135 patients with invasive vulvar carcinoma in Stage Tl without clinical evidence of inguinal lymph node involvement underwent simple vulvectomy performed by hot-knife resection without lymphadenectomy. Although 65 patients (Group 1) received postoperative inguinofemoral radiation therapy, 70 patients (Group 2) did not, and none received local vulva irradiation. Results: The S-year survival rates were 93.7% in Group 1 and 91.4% in Group 2 (p = NS). Although clitoris involvement was significantly more prevalent in the irradiation group (p = 0.04), inguinal relapse was found less frequently in Group 1 (4.6% or 3 out of 65 patients) than in group 2 (10% or 7 out of 70 patients) (p = 0.32). The complication rates were, 7.7% in Group 1 and 2.9% in Group 2,2.7% for vaginal stenosis (two patients in each group), 1.5% for inguinal pain (one patient in Group 1), 1.5% for rectovaginal fistula (one patient in Group 1)) 1.5% for vulvar infection (one patient in Group 1). Conclusion: No statistically significant differences in the relapse rates and survival times were found. Risk factors were equally distributed in both study groups except for clitoris involvement. The 5.year survival rates in both groups were similar to those reported in the literature for radical vulvectomy and inguinal lymph-node dissection (83-96% ). Morbidity in our study was low. Although our data showed similar results in both groups, we are not recommending at this time to omit groin radiation in general, but it may be justified in low-risk cases. 0 1997 Elsevier Science Inc.

Summary The purpose of this report is to comment on the case of a 16 year-old nulliparous patient who presented to the gynaecolgy clinic in Kawabe General Hospital, Zambia. She had a past history of good health, but had noticed a rapid increase in the size of her external genitalia over a period of 6 to 12 months, which interfered with walking. On examination a large mass was observed in the vulva, almost occluding it, which had an irregular surface with striae in the form of cock´s crests and the characteristic odour of condyloma acuminata. Vaginal examination with a speculum was impossible. Blood tests showed reactive serology and HIV positive status. Following unsuccessful antibiotic and local antiviral treatment, simple vulvectomy was carried out in two stages, completely excising the tumour. There were no visible lesions of similar characteristics found in the vagina. Histological examination confirmed the clinical suspicion of giant condyloma acuminata of the vulva.

Vulvar Paget's disease is very rare malignancy originating in vulvar apocrine-gland-bearing skin cells or as a manifestation of adjacent primary anal, rectal or bladder adenocarcinoma.1 It is also called extramammary Paget's disease and affects both sexes, in the vulvar, perineal or scrotal areas. Vulvar Paget's disease is most commonly seen in postmenopausal Caucasian females and appears clinically as red, eczematous and pruriginous – and sometimes painful – lesions. In most cases it is an intraepidermal adenocarcinoma in which tumor cells involve the epidermis and sometimes the underlying skin appendage, but sometimes there is focal dermal invasion.2 Probably because of its multicentric nature, Paget's disease has a chronic and relapsing course. Surgery has been the treatment of choice, but disease frequently extends far from the visible lesion and surgical margins are frequently positive. The Paget's cells are larger than keratinocytes, have clear chromatin, a prominent nucleolus and gray–blue cytoplasm and they may appear vacuolated with hematoxylin and eosin staining.3 All these characteristics are consistent with their glandular nature. Many patients undergo multiple surgical procedures, including wide local excision, simple vulvectomy or radical vulvectomy. However, the margins of primary surgical specimens are positive in more than half of the patients.3 Consequently, the recurrence rate is about 47%.4 The disappointing results of surgical treatment and the high morbidity and side-effects of extensive vulvar resection have encouraged many authors to try innovative clinical treatment for vulvar Paget's disease.

Background. This report consists of a retrospective analysis of 50 patients with primary invasive and 17 with recurrent histologically confirmed vulvar carcinoma treated with radiation therapy for locoregional disease. Methods. Of the patients with primary tumors, 13 were treated with wide local excision plus radiation therapy; 13 had radical vulvectomy followed by irradiation to the operative fields and inguinal-femoral/pelvic lymph nodes; 8 received similar postoperative radiation therapy after partial or simple vulvectomy; 16 patients had radiation therapy alone after biopsy; and 17 had recurrent tumors treated with radiation therapy alone. Results. In patients treated with biopsy/local excision, local tumor control was 92-100% in T1-3N0 disease, 40% in similar stages with N1-3, and 27% in recurrent tumors

The authors report a case of usual-type (basaloid-type) vulvar intraepithelial neoplasia (VIN) 3 that failed to respond to imiquimod cream. A 51-year-old Japanese woman visited her local gynecologist complaining of vulvar itching. Atypical cells were noted in cytology smears, but nine vulvar biopsy specimens showed benign proliferation of epithelial tissue. The patient was placed under careful observation for 8 months, when the vulvar smears once again showed atypical cells and biopsy specimens revealed VIN3. The patient was then referred to our hospital where she was given a diagnosis of VIN 3, basaloid type of usual type. The biopsy specimens were positive for p16 and the lesions were confirmed to be human papilloma virus (HPV)-related. We recommended simple vulvectomy but the patient requested conservative treatment with imiquimod cream. With her written informed consent, we prescribed imiquimod cream to be self-administered 3 times a week. Colposcopy and pap smear test were performed every 2 weeks. Four weeks after the start of treatment, a fingertip-sized papule was detected at the patient’s vaginal introitus. By 6 weeks, the lesion had enlarged, and biopsy specimens revealed invasive squamous cell carcinoma. At 7 weeks, we performed simple vulvectomy. The surgical specimen showed stage pT1b keratinizing-type squamous cell carcinoma. HPV-16 DNA was detected in the specimen.

We report a 25-year-old unmarried girl who developed multiple papular lesions on both labia majora with the past history of documented HPV-6 viral infection in the vulva. A wide local excision was performed and histopathological report confirmed a case of angiokeratoma. To the best of our knowledge this is the first case of angiokeratoma of vulva following chronic HPV infection.

OBJECTIVE This study compared the rates of survival, recurrence, and the occurrence of complications after surgery for vulvar cancer in selected patients treated by simple vulvectomy or wide local excision (WLE) and ipsilateral superficial inguinal lymphadenectomies (ISIL) and who were in a representative group of previous patients treated by standard radical surgery (control). MATERIALS AND METHODS Superficial inguinal lymphadenectomies were performed in 32 patients with laterally localized squamous cell tumors of 1 to 3 cm in size and without palpable lymph nodes. Eight cases, which showed histological evidence of lymph node metastasis, were submitted to conventional radical treatment and excluded from the study. Of the remaining 24 patients, 12 underwent vulvectomy, 7 hemivulvectomy, and 5 WLE. The results of this group were compared with those of 21 historical controls who previously had tumors of 1 to 3 cm and had been treated by radical vulvectomy with superficial and deep bilateral inguinal lymphadenectomy. RESULTS Dehiscence of the flaps occurred in 66.6% of the control patients and in 8.3% of the ISIL group (chi , p

A case of isolated giant vulval neurofibroma in a 34 year old para 1 lady is reported. She presented with a large vulval swelling involving labia majora, minora and the clitoris preventing her from having sexual intercourse. She had vulval biopsy sent for histopathological examination and it was diagnosed as “chronically inflammed neurofibroma of the vulval”. Because of the large size, simple vulvectomy was performed and she recovered well postoperatively. Literature on vulval neurofibroma regarding the presentation and management was revieved. Key Words: Vulva Neurofibroma, Vulvectomy [ Trop J Obstet Gynaecol, 2004;21:190-192]

Giant condyloma acuminatum (GCA) is a rare sexually transmitted disease caused by Human papilloma virus (HPV) and characterised by invasive growth. Very few cases of GCA of vulva have been reported till now in the English literature [1–3]. We report a case of 34-year-old lady with GCA of vulva who was successfully treated by simple vulvectomy and reconstruction of the defect by ‘‘four-flap technique’’. A 34-year-old, married woman presented with complaints of growth on her vulva for the last 8 months. The patient was 6 months pregnant when she first noted the lesion and did not seek any medical help. Two months after her caesarean delivery, she underwent cryosurgery, but with no improvement. She denied any history of sexual promiscuity. On examination there was a 14 cm 9 10 cm 9 6 cm friable, foul smelling growth on her vulva involving both labia majora, extending anteriorly to mons pubis and posteriorly to perianal region (Fig. 1a). There were a few satellite lesions on labia minora and perianal region. Clitoris, urethra and external anal sphincter were not involved. Colposcopic, proctoscopic and oropharyngeal examinations were normal. The patient and her partner were screened negative for sexually transmitted diseases and high-risk HPV DNA. Incisional biopsy showed squamous cell papilloma with koilocytic changes and it was positive for HPV DNA type 6. A simple vulvectomy, achieving wide margins, was performed. Small lesions in perianal area and labia minora were also excised. Reconstruction of vulva was performed using ‘four flap technique’ (personal communication by Dr. Prakash V, Department of plastic surgery, Safdarjung Hospital, New Delhi). In this technique, four random pattern flaps in the dimension of 1:1 or 1:2 were raised adjacent to the defect and advanced medially to resurface the defect. This resulted in defect being transferred laterally, but this defect was smaller than the original defect and could be closed primarily (Fig. 1b). Final histopathology report confirmed the diagnosis of condyloma acuminatum with mild degree of dysplasia. Functional outcome was good with no sexual complaints. She remained free of recurrence at 12 months of follow-up (Fig. 1c). Many treatment strategies have been documented in the literature for management of GCA, but mainly in the form of case reports. Complete surgical excision with histologically clear margins, with or without adjuvants, is the mainstay of the treatment for GCA, including that of vulva [1, 3]. Other modalities include combined radiotherapy and chemotherapy, topical agents (e.g. podophyllin, 5-FU, imiquimod, bleomycin) and intralesional injections of interferons [4–6]. The skin defects created after excision of GCA in the peri-anal region can be managed with mesh skin grafting, flaps and even healing by secondary intention [1, 7–9]. Flaps have been shown to give better results for reconstruction of vulva [8, 9]. The additional advantage of our ‘four flap technique is that the scars do not extend over the perineum or thighs. In our patient, the postoperative period P. Mittal (&) R. Gupta R. Dewan S. Singhal J. Suri Department of Obstetrics and Gynaecology, VMMC and Safdarjung Hospital, New Delhi 110029, India e-mail: drpratima@hotmail.com

The earliest description of vulvar cancer was published by Morgagni 53 in 1769, but for the subsequent 250 years, there was little discussion of the condition in the medical literature. Although Rothschild 63 of Freiburg presented a dissertation in 1912 describing more than 300 cases, it is Basset 5 who is generally considered to have laid the foundation for the modern approach to the condition. Basset believed that vulvar cancers originated on the clitoris, and that early, bilateral, nodal involvement was typical through two lymphatic pathways, one of which followed the round ligaments to the external iliac nodes, whereas the other was to the inguinofemoral nodes. His surgical technique, based on anatomic studies of cadavers, emphasized the importance of removing the inguinal, femoral, and distal iliac nodes en bloc bilaterally, together with a radical excision of the entire vulva 68 : One should in every case strive to remove in a single mass all the tissues to be extirpated. It will always be preferable to start with the nodes and lymphatic pedicles on each side carrying out removal of the tumor only as the last step. Despite the radicality of the approach, of the 176 cases he reviewed only 27% survived 1 year or more, with most of those who died suffering from groin recurrences. He did, however, hold out the hope that, with earlier diagnosis and the application of his systematic surgical approach, far better outcomes could be achieved in the future. This hypothesis was tested by Taussig, 71 who published his first article in 1917 describing 15 cases of vulvar cancer and who, in 1940, was able to publish his experience with 155 cases treated with a variety of surgical approaches between 1911 and 1940. Among cases in which vulvectomy alone was performed, the 5-year survival was 8%; among cases in which vulvectomy was combined with superficial or incomplete nodal dissection, it was 29%; but in cases in which the complete Basset type approach was used, it was 59% despite the fact that 41% of women so treated had histologically positive lymph nodes. The surgical mortality rate of the procedure was 7%. It is of considerable interest that Taussig modified the Basset procedure to improve wound healing by using separate incisions in some cases for the vulvectomy and each groin dissection. He did not find any recurrences in the skin bridges. One of the most important figures in the development of surgical treatments for vulvar cancer was Way. In his Hunterian Lecture in 1948, 78 he discussed the principles underlying his radical approach to surgery for vulvar cancer. After discussing the lymphatic drainage of the vulva, he reviewed the results of a range of treatments applied in Newcastle up to 1940. Simple vulvectomy with local excision of tumor gave a 24% 5-year survival rate, diathermy coagulation, 10%; excision of the vulva or hemivulvectomy with unilateral groin dissection, 23%; and vulvectomy with bilateral superficial groin dissection, 21%. With radiotherapy alone, the 5-year survival rate was approximately 13%. Way read of Taussig's results at what he described as "a time of despondency concerning the treatment of carcinoma of the vulva." He recognized that clinical assessment of groin nodes was an unreliable indication of their involvement with the disease and concluded that: We require an operation which will remove the vulva very widely and include a removal of all the premalignant lesion; which will remove the nodes up to and including the external iliacs on both sides and which will take away the lymphatic anastomosis situated in the mons veneris. His lymph node dissection aimed to remove all the affected groups of nodes and the next group of nodes beyond them. The extent of the procedure was considerably greater than that described by Basset. Way considered not only that was closure of the vulvar wound impossible but also, if it could be achieved, that the result would be unsatisfactory and a local recurrence certain. In a later publication, Way 80 described how, by binding the patient's legs together at the knees and holding the knees in a flexed position postoperatively, primary closure of the vulva could be achieved in most cases and that the postoperative stay decreased from approximately 90 days to 23 days. Problems with fluid balance from the raw wound and infection were the two major immediate complications of the technique, whereas lymphedema was the commonest longer-term problem. In earlier years, the surgical mortality was approximately 12%, but by 1971 it had decreased to 3%. The application of this surgical approach in 367 cases between 1938 and 1975 led to an overall corrected 5-year survival rate of 58%, and when the nodes were negative, it was 91%. On the basis of the dramatic turnaround in survival achieved by this type of surgery, Way concluded that "the ideal treatment of cancer of the vulva is radical vulvectomy with superficial inguinal and deep pelvic node dissection." Although the widespread adoption of radical vulvectomy with groin and pelvic lymph node dissection resulted in a dramatic improvement in overall survival of women, the radicality of the procedure, especially for older women, and the morbidity, both physical and psychological, were causes for great concern, and in the past 20 years, considerable research has led to major advances in the treatment of the disease.

Vulval biopsies from a 66-year-old lady complaining of pruritus showed intraepithelial Paget's disease. On simple vulvectomy, extramammary Paget's disease was found to be associated with vulval intra-epithelial neoplasia (VIN III) and superficial dermal invasive adenocarcinoma. Bilateral inguinal lymphadenectomy later revealed metastatic spread of the tumour. We believe this is a unique combination and discuss the possible origins in relation to this lady's past medical history of breast carcinoma 20 years and spinal metastases 17 years before.

We report two women in whom vulval Paget's disease occurred in association with local adenocarcinoma and previous breast adenocarcinoma. The first patient presented at the age of 83 years with moist erythematous changes over the perineum and an indurated area near the anus. Biopsy of the indurated area showed Paget's cells throughout the epidermis and, below, adenocarcinoma infiltrating the dermis. Ten years previously, she had undergone a left mastectomy for infiltrating ductal carcinoma of the breast. The second patient was diagnosed as having Paget's disease at the age of 74 years. A vulval biopsy showed Paget's cells in the epidermis but, in addition, there were changes suggestive of adenocarcinoma of the sweat glands. Her symptoms of vulval itching had started at the age of 45 years and had led to a simple vulvectomy at the age of 57 years. Retrospective review of this vulvectomy specimen showed Paget's disease. She had also previously been treated for infiltrating ductal adenocarcinoma of the breast and adenocarcinoma of the rectum. The management of Paget's disease is difficult because of its high recurrence rate and, as illustrated by our two cases, treatment is difficult if the patients are elderly and in poor general health.

We encountered an unusual case of extramammary Paget's disease (EMPD) recurring on the skin of the lower abdomen and entire right leg two and a half years after simple vulvectomy for minimally invasive Paget's disease of the vulva. Histologic examination of the skin metastases demonstrated that the proliferation of Paget cells was confined to the dermis, most of them were located in lymphatic vascular spaces. This case confirms that minimally invasive Paget's disease of the vulva may sometimes be an aggressive disease. We consider that lymphatic metastases already existed in this patient at the time of initial surgery; thus, the recurrence of EMPD on extragenital skin sites may have been prevented if initial treatment would have included radical vulvectomy and bilateral groin lymph node dissection instead of simple vulvectomy without groin lymph node dissection.

Iatrogenic immune suppression following renal transplantation is frequently associated with certain neoplasms, including vulvar carcinoma. We describe a patient with a vulvar carcinoma less than 1 mm depth of invasion and less than 3 mm superficial spread 12 years after renal transplantation. A simple vulvectomy was performed but 4 months later disease recurred in the inguinal nodes. The patient died 20 months later with progressive disease in the retroperitoneum and liver metastases.

Cutaneous lymphangiectasia, also called as acquired lymphangioma, is a benign cutaneous disorder involving the dermal and subcutaneous lymphatic channels. It can rarely occur on the vulva. We describe a 35-year-old woman who came with multiple raised lesions over the vulva and left upper thigh of 1 year duration. She gave history of getting treated for multiple swellings that developed over right side of the neck and inguinal regions on both sides three decades ago. On local genital examination, the patient had a large polypoidal growth involving both sides of the vulva, left upper thigh, and over pubic area. Multiple linear scars were present over the upper thighs and groin bilaterally. The patient underwent simple vulvectomy and left thigh growth excision. Histopathological examination of the vulvectomy specimen confirmed our diagnosis.

EDITORIAL COMMENT: We accepted this paper for publication because detailed analysis of results in 90 cases of invasive carcinoma of the vulva treated by one team of surgeons in a 10-year period provides important information for those who must decide how to treat women with these lesions. The legends to Figure IA and B present an anecdotal case illustrating that even aged women with invasive cancer merit consideration of radical surgery. Summary: Ninety-nine patients with carcinoma of the vulva were referred to the Gynaecologic Oncology Unit, Royal Brisbane Hospital, over 10 years. Ninety of these patients had a squamous cell carcinoma (SCC). They were assessed by the 1969 FIGO clinical staging. Each stage was related to nodal involvement, size, depth, histological grade, lymphvascular space involvement, perineural permeation and multifocal disease site. The operability rate was 85%. Treatment was individualized in line with recent philosophies for more conservative surgery where appropriate. Mortality was 2.6%. Five-year survival of surgically treated patients was 60.3%; node negative patients 100%, and node positive patients 25.2%. After adjustment for stage and size, the only other independent statistically significant feature was perineural penetration. Local recurrence was more likely with increased stage and size, unclear margins and multifocal involvement. It is important to note that medically unfit patients who had vulvectomy alone and who later developed positive nodes had 100% mortality. This group of patients significantly decreases survival rates, confirming the importance of carrying out inguinofemoral lymphadenectomy at the time of initial surgery. Morbidity was decreased by conservative surgery. Lymphoedema remains the most common chronic complication. No significant difference was shown in local recurrence between different types of surgery, wide excision, hemivulvectomy, simple vulvectomy or radical vulvectomy (22%), confirming the safety of the more conservative approach of recent years.

Gynecol Oncol 2000;77:183–89 This is a retrospective review of 76 patients with Paget's disease of the vulva from the MD Anderson Cancer Center. The authors also divided patients into four groups by diagnosis, the majority (46) being intraepithelial Paget's disease. Nine patients had invasive Paget's disease; an additional 13 patients had intraepithelial Paget's disease with another coexisting cancer. Coexisting cancers of interest included endometrial, ovarian, breast, and cervical carcinomas. The mean age of patients at diagnosis was 67.5 years. Surgical treatment included wide local excision, simple vulvectomy, and radical vulvectomy equal in numbers. Wide local excision appeared to be as effective overall in treating the condition as other modalities. Wide local excision was associated with a higher risk of recurrent disease, but, overall, patients with wide local excision tended to survive longer than patients treated more radically. Comment: This paper is an important study and follow-up of this unusual disease of the vulva. It is written well. It highlights that most Paget's disease of the vulva is intra-epithelial. Involved margins are quite common and do not necessarily correlate to disease recurrence. Additionally, it highlights the problem of underlying adenocarcinoma in a number of patients and the possibility of coexisting cancers. Within this study, there were coexisting cancers of the endometrium, ovary, breast, and cervix. Paget's disease also has been described in other studies with bladder carcinoma and colon cancer. Concerning margin status, the authors point out that margins were positive in 53% of patients; Paget's disease recurred in 31% of patients with positive margins and 33% of patients with negative margins. It has been my practice not to be concerning with margin status and to observe patients with positive margins. Wide local excision has been my treatment of choice to rule out underlying adenocarcinoma followed by close follow-up of the patients. I believe the readers of the Journal of Lower Genital Tract Disease would benefit from reading this entire article. (CJD)

Based on 124 patients with squamous cell carcinoma of the vulva treated at the 1. Frauenklinik der Universität München from 1971 to 1980, the influence of pretreatment characteristics on survival was assessed. The patients underwent a simple vulvectomy with local and inguinal irradiation. All histologic specimens were worked up in the same manner, and all available specimens were reverified. Follow-up lasted from at least 2 up to 12 years post-treatment, with no dropouts. Using the Cox model of multivariate analysis, five pretreatment characteristics were found to most strongly influence survival: age, dissociated tumor growth, lymphatic spread, tumor thickness, and ulceration. These pretreatment characteristics were implemented in an algorithm for survival-oriented prognostic forecasting. Survival data as predicted from this algorithm correlated well with observed survival data. The validity of these prognostic factors needs to be examined in further studies using comparable patient populations and study designs.

We have reviewed our treatment of premalignant and malignant disease of the vulva in 78 patients seen at Colorado General Hospital since 1955. Premalignant lesions can be managed conservatively. Wide local excision or local 5-FU cream usually arrests premalignant disease without further surgery. Patients with premalignant disease treated by one of these methods need to be followed closely in order to detect recurrence. Microinvasive lesions can be treated by wide local excision in selected cases; in others simple vulvectomy is the treatment of choice. Forty-three patients with invasive vulval carcinoma were treated by radical vulvectomy and removal of lymph nodes. Extension at the operation to include the deep retroperitoneal nodes aided in the cure of but one of the 43 patients. Thirty-four patients (79 per cent) are alive and free of disease three to 18 years after radical vulvectomy. Patients with metastasis to regional lymph nodes had a 40 per cent survival rate. If the patients had Stage I, II, or III disease, the presence or absence of positive lymph nodes did not affect the prognosis in our series. All patients with Stage IV disease had positive nodes and eight of nine died.

Between 1970 and 1982, 113 patients were treated for invasive vulvar cancer in FIGO stages I-IV; 97 patients were available for follow-up. Forty-one patients (42.3%) underwent radical vulvectomy and lymphadenectomy, 21 underwent simple vulvectomy, and 12 (12.4%) had electric resection of the lesion; 42 patients (43.3%) received postoperative radiotherapy. The 5-year survival rate was 61.8% after surgery and radiotherapy. Five-year survival in stages I, II, and III was 85.3%, 60.7%, and 17.9%, respectively. Overall 5-year survival was 52.6%. Patients with small, highly differentiated squamous cell cancers, without lymph node involvement, did best.

From 1951 to 1976 234 patients with invasive carcinoma of the vulva were treated at the Department of Obstetrics and Gynaecology, Medical School Hannover. 53.3% of these patients underwent simple vulvectomy. Radical vulvectomy and lymphadenectomy was performed in 17.9%. 45.7% received postoperative radiotherapy. -213 patients were followed up 5 years or more. After surgery alone the 5-year-survival rate was 56.9%, after operation and radiotherapy 43.4%, after exclusive radiation 14.2%. The 5-year-survival rate of all patients was 42.7%.

Various studies assess the significance of depth of invasion as an important prognostic factor in squamous cell carcinoma of the vulva. However, methodologic problems exist with regard to the measurement points. While the deepest point of invasion can be measured accurately, the upper reference point is arbitrarily chosen. The goal of the present study was to identify the method of measurement and the threshold value, allowing the clearest prognostic differentiation between groups of patients with vulvar carcinoma. The study involved 124 patients treated between 1971 and 1980, who had received identical treatment (simple vulvectomy followed by local and inguinal irradiation) and identical histopathologic workup (large-scale sections). Beginning with the deepest point of tumor invasion, comparative measurement was carried out with three different points of reference: to the basement of the most superficial dermal papilla (method A), to the surface of the tumor (method B), and to the basement membrane of the deepest rete ridge (method C). The data were used to determine differences in specified end points. The results and conclusions are as follows: (1) The morphometrically determined degree of tumor invasion has prognostic significance. (2) Measurement of tumor thickness (method B) prognostically differentiates patient groups better than measurement of invasion from the most superficially lying epithelial papilla (method A), if classified into tumors up to and over 0.5 cm. (3) Patients with superficial invasive vulvar carcinomas up to 0.5 cm can be further prognostically differentiated into two groups, when measuring depth of invasion from the deepest rete ridge (method C), and classifying into tumors up to and over 0.3 cm.

A comparative clinicopathologic study of 10 cases of vaginal “botryoid sarcomas” and 17 cases of “botryoid sarcomas” of extravaginal origin from the files of the Mayo Clinic and of 71 cases of “sarcoma botryoides” of the vagina from the literature is described. There is no basis for differentiating vaginal botryoid rhabdomyosarcoma from botryoid rhabdomyosarcomas which occur in other sites or for classifying this tumor with the mesodermal mixed tumors of the cervix and uterus. All of these tumors are embryonal rhabdomyosarcomas. Their botryoid configuration results from the forces of growth acting in an epithelium-lined hollow cavity. An embryonal rhabdomyosarcoma (botryoid type) of the vagina begins in the subepithelial layer of the vagina, rapidly expands within the limits of the vagina before infiltrating the vaginal wall and extending beyond the limits of the vagina, and exhibits a predilection for metastasizing to the regional lymph nodes and lungs. Vaginal bleeding or a vaginal tumor heralds the onset of this tumor. Other symptoms appear after extravaginal extension occurs. Early detection and prompt aggressive surgical treatment appear to be the most important factors regarding a favorable outcome. This tumor is best treated by a combined pelvic exenteration, pelvic lymphadenectomy, and simple vulvectomy procedure. Chemotherapy and radiation therapy should be reserved for recurrent disease only.

A simple technique of closing the defect following simple vulvectomy is described. The labia minora as a pedicle graft avoids the problems encountered by conventional methods.

Although stenosis of the vaginal introitus is a relatively uncommon complication of simple or radical vulvectomy, when it does occur it can be most distressing and disabling to the patient. Numerous types of repair have been described with varying indications. In this report, the literature is reviewed and indications for the specific procedures are discussed. The case history of a patient suffering with introital stenosis after simple vulvectomy is presented, and the little known but very practical vulvar Z-plasty procedure is illustrated and described.

Patients with extramammary Paget's disease of the vulva at the University of California--Irvine Medical Center and the Memorial Medical Center of Long Beach Women's Hospital between 1976 and 1986 were treated with nonradical surgery. These 14 patients were analyzed in a retrospective fashion to determine if conservative management was successful in eradicating the disease process while preserving appearance and sexual function. All patients were treated with nonradical surgery: skinning vulvectomy with split-thickness skin graft (86%), hemivulvectomy (7%), and simple vulvectomy (7%). In all cases intraoperative frozen-section analysis of surgical margins was performed to determine the extent of disease and outline the margins of resection. No patients had Paget's disease associated with an underlying adenocarcinoma. Three patients developed recurrent Paget's disease. Two of these patients recurred at the site of a positive margin and thus represent persistent disease. Only one patient (7%) had recurrent disease in the face of negative margins. The median duration of follow-up was 50 months. All patients are presently free of disease. The rationale for this conservative management is discussed.

A retrospective study of 56 patients treated for carcinoma in situ of the vulva over a 24-year period has shown a relative increase in the occurrence of this neoplasm. Only 6.7% of patients in the first 14 years of the study period were less than 35 years of age, as compared with 29.3% in the last decade. In the earlier years of the study, simple vulvectomy was the primary treatment, and 81.3% of the patients were disease-free at 5 years. Wide local excision, used in the later years, resulted in a rate of 46.2% with no evidence of disease. Involvement of surgical margins played a significant role in this increased incidence of recurrence. Only one patient, originally treated by simple vulvectomy, later developed early stromal invasion. A wide local excision technique should be strongly considered as initial management of this multifocal neoplasm. Close follow-up is mandatory. Recurrence of disease may be treated with appropriate wide excision.