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PubMed2003

The role of radiation therapy in preventing regional recurrences of invasive squamous cell carcinoma of the vulva.

PURPOSE: To evaluate treatment of the inguinal nodes for patients with squamous cell carcinoma of the vulva. METHODS AND MATERIALS: We reviewed the records of 227 patients who had treatment of the inguinal lymph nodes between 1980 and 1998 for squamous cell carcinoma of the vulva. The inguinal nodes were clinically suspicious in 67 patients and clinically negative in 160. Regional treatment was as follows: lymph node dissection (LND) alone in 119 patients, LND plus radiation therapy (RT) in 57, and RT alone in 51. The extent of LND ranged from node excision to radical inguinal LND; all patients treated with LND alone had at least a superficial inguinal LND. Median follow-up of surviving patients was 98 months. Rates of inguinal node recurrence (INR) at 5 years were calculated using the Kaplan-Meier method. RESULTS: Thirty-two patients had INRs (5-year INR rate, 15.4%). Patients who received RT alone or RT + LND were significantly more likely than those treated with LND alone to have T3-4 tumors, tumors >5 cm, or lymph node involvement. However, 5-year INR rates were similar for the three groups (16%, 13%, and 16%, respectively). For patients who had LND only, the risk of INR was greater if the primary tumor was more than 2 cm (p = 0.056) or poorly differentiated (p = 0.04). For patients who had postoperative RT, INR was significantly greater if the time from LND to RT was greater than 50 days (p = 0.03). Ten patients had severe groin or lower-extremity complications after LND. Two patients died of postoperative cardiopulmonary complications. Six patients who were treated with RT had hip fractures or hip replacements after treatment. CONCLUSION: RT alone or in combination with LND is highly effective in preventing INR in patients with squamous cell carcinoma of the vulva and is associated with a low risk of major late complications.

Adolescent↗
PubMed2001

Loss in 3p and 4p and gain of 3q are concomitant aberrations in squamous cell carcinoma of the vulva.

Neoplasm of the vulva is a rare malignancy accounting for <5% of all female genital-tract cancer. However, in recent years the incidence of vulva intraepithelial neoplasia, known to serve as a precursor to carcinoma, has increased in young women generating considerable interest in its pathogenesis. Genetic changes at the molecular level in precursor or invasive vulvar tumors are not well investigated, and DNA copy number changes have not been reported until now. We used comparative genomic hybridization (CGH) to analyze genetic alterations in 10 primary invasive squamous cell carcinomas of the vulva. Chromosomal aberrations were identified in 8/10 cases. The most frequent chromosomal losses were 4p13-pter (five cases), 3p (four cases), and 5q (two cases), and less frequent losses were detected at 6q, 11q, and 13q (one case each). The most frequent chromosomal gains were 3q (four cases) and 8p (three cases), and less frequent gains were found in 9p, 14, 17, and 20q (one case each). The pattern of chromosomal imbalance in vulvar cancer detected by CGH was revealed to be very similar to that in cervical cancers, despite regional differences in their prevalence. These results suggest that the pathogenic pathways in vulvar and cervical carcinomas may be similar and that the genetic background may be common to these two squamous cell carcinomas.

Adult↗
PubMed1997

The relevance of various vulvar epithelial changes in the early detection of squamous cell carcinoma of the vulva.

The objective of this study is to evaluate the possible relevance of vulvar epithelial changes as a risk factor for squamous cell carcinoma of the vulva. The data of 66 women surgically treated for squamous cell carcinoma of the vulva have been analyzed. More than 6500 slides from the resection specimens were revised with special emphasis on concurrent epithelial changes. Synchronous epithelial changes were seen in 63 patients. Thirty-nine patients had synchronous vulvar intra-epithelial neoplasia grade 1 (VIN I), 10 VIN II and 13 VIN III. Thirty-one patients had synchronous lichen sclerosus and 49 squamous cell hyperplasia. The difference between the percentage of patients with epithelial changes diagnosed preceding their carcinoma (30%) and the percentage of patients that had synchronous epithelial changes after reviewing the specimen (95%) was striking. It was concluded that more careful diagnosis, treatment and follow-up of these conditions might lead to an earlier recognition of squamous cell carcinoma of the vulva and therefore to a better prognosis.

Journal Article↗
PubMed1999

Noninvasive detection of inguinofemoral lymph node metastases in squamous cell cancer of the vulva by L-

In the majority of patients with early stage squamous cell cancer (SCC) of the vulva, an inguinofemoral lymphadenectomy is performed (in retrospect) for diagnostic reasons: exclusion of inguinofemoral lymph node metastases. The morbidity of this procedure, however, is significant. The aim of the present study was to evaluate noninvasive detection of inguinofemoral lymph node metastases by positron emission tomography (PET) using L-[1-11C]-tyrosine (TYR) as tracer. In patients with SCC of the vulva, scheduled for resection of the primary tumor and uni- or bilateral inguinofemoral lymphadenectomy, results of preoperative palpation of the groins and TYR-PET imaging were compared with histopathology. PET imaging was performed using two different methods. In a first group (n = 16), nonattenuation corrected 'whole body' scans were performed, and in a second group (n = 9), attenuation corrected static emission scans. Sensitivity, specificity, accuracy, and positive and negative predictive value for palpation were 62%, 89%, 82%, 67%, and 87% per groin. Sensitivity, specificity, accuracy, and positive and negative predictive value for TYR-PET were calculated for the two methodologies separately and overall. There were no significant differences. Overall values were 53%, 95%, 94%, 33%, and 98% per lymph node and 75%, 62%, 65%, 41% and 88% per groin. Detection of inguinofemoral lymph node metastases by TYR-PET is not superior to palpation. Neither palpation nor TYR-PET is able to adequately predict or exclude presence of inguinofemoral lymph node metastases in patients with SCC of the vulva.

Journal Article↗
PubMed1991

[Melanoma of the vulva. Report of 12 cases].

The present retrospective study includes 12 patients with melanoma of the vulva who underwent vulvectomy between 1977 and 1987. Six patients were less than 55 and 6 other patients more than 69 years old. Three women of the younger age group survived (31, 44 and 116 months, respectively), whereas three of them died of the tumour. In the patient group of over 69 years of age 4 patients died of the tumour, one patient did not appear for follow-up and one further patient died in the meantime. Nine patients suffered from deeply infiltrating tumours (Clark levels IV and V), 2 patients had a Clark level II tumour, and in one case the Clark level was unknown. Depth of infiltration correlated with the prognosis of disease, i.e. only one patient with deep tumour infiltration, but both patients with Clark level II tumours survived. Therefore, the importance of early diagnosis of melanoma of the vulva should be emphasized, and accurate inspection of the vulva should be indispensable in the course of routine gynaecological examination also in younger women.

Adult↗
PubMed2002

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.

Adenocarcinoma↗
PubMed1991

Management of locally advanced squamous cell carcinoma of the vulva.

Carcinoma of the vulva is an uncommon malignancy, accounting for 5% of gynecologic cancers. Patients who have locally advanced disease often cannot be managed locally by a radical vulvar resection. Current approaches to the treatment of locally advanced vulvar cancer include ultraradical surgery, radiotherapy, or a combination of treatment modalities. Ultraradical surgery has been used for patients with clinically resectable vulvar lesions and generally has consisted of a radical vulvar operation combined with a partial or total pelvic exenterative type procedure. The use of primary radiotherapy for carcinoma of the vulva remains controversial but may be the only option available when the patient has unresectable disease. Data have accumulated to confirm that megavoltage radiotherapy can cause marked regression of even locally advanced vulvar carcinoma to the point where a more limited resection can then be undertaken, often with an improved resection margin, with sparing of organ function, and improved quality of life. As with preoperative radiotherapy, combined chemoradiotherapy with or without resection has been used increasingly, with some promising results in several squamous cell carcinomas. There are few data on this type of treatment for carcinoma of the vulva.

Carcinoma, Squamous Cell↗
PubMed1985

Melanosis of the vulva. A clinical simulator of malignant melanoma.

Eight patients with melanosis of the vulva are presented in order to illustrate the characteristic clinical and histopathologic features of the condition. Despite clinical features that may be indistinguishable from those of malignant melanoma, the patchy pigmentation of melanosis that is seen mostly on the vulva, but also on the penis, shows wholly different histologic changes than those of melanoma, namely, a slightly increased number of typical melanocytes arranged as solitary units at the dermal-epidermal junction of a hyperpigmented epidermis. It is important to identify melanosis of the vulva for what it is, because overdiagnosis of it as malignant melanoma may result in needless radical surgery.

Adult↗
PubMed2003

Metastatic tumors of the vulva: a clinicopathologic study of 66 cases.

Metastatic tumors involving the vulva are rare, with only a few series and case reports published in the English literature to date. In this study, we present the clinicopathologic features of 66 cases of metastatic tumors of the vulva seen at the University of Texas M. D. Anderson Cancer Center from 1944 to 2001. The patients' age ranged from 18 to 84 years (mean 54.8 years). The most common presentations were vulvar nodules or a mass (39 cases), pain (7 cases), and ulceration (5 cases). In 46.9% of cases, the primary tumor was of gynecologic origin, whereas in 43.9% of cases the primary tumor was of nongynecologic origin. The remainder had unknown primaries. The site most frequently involved by metastasis was the labium majus (44 cases: 18 on the right, 13 on the left, 6 bilateral, and 7 unspecified side). Thirty percent of the patients received chemotherapy as treatment for the metastasis, 27% received radiotherapy, and the rest received some combination of chemotherapy, radiotherapy, and surgery. Of the 60 patients with available follow-up, 52 died of disease within 1-81 months (median 7.5 months) from diagnosis of the metastasis. Metastatic tumors of the vulva are rare; however, the diagnosis of these tumors is facilitated by the knowledge of a preexistent malignancy and the lack of a mucocutaneous intraepithelial lesion.

Adolescent↗
PubMed1986

Malignant melanoma of the vulva: a clinical-pathologic review of 16 cases.

A study of 16 malignant melanomas of the vulva was undertaken to identify the histologic variants found on the vulva and to evaluate the use of microstaging as a predictor of behavior. Ten of 16 cases were melanoma of mucous membrane (MMM) type of acral lentiginous melanoma. This percentage (63%) is notably higher than in previous reports. Three cases were nodular melanoma (NM). All lesions of MMM and NM type occurred on mucous membranes. Three cases were superficial spreading melanoma and all occurred on skin. Neurotropism was observed in 5 of the 10 MMM. Neurotropic melanoma has been reported only once previously in the vulva. Depth of the melanomas was greater than 1.0 mm in 13 of the 16 cases. This correlated with Clark and Chung levels of 3 or higher in the same 13 cases. A poor outcome with an overall 5 year survival of 20% of cases with invasive disease was found. The poor outcome and large number of thick lesions would suggest a correlation, but the distribution is skewed and number of cases too small for statistical documentation.

Adult↗
PubMed1998

Liposarcoma (atypical lipomatous tumors) of the vulva: a clinicopathologic study of six cases.

Liposarcoma, the most common soft tissue sarcoma, usually arises in the limbs, trunk, or abdomen, but very rarely in the vulva. Six cases of liposarcoma of the vulva are described here. They were characterized by their occurrence in predominantly middle-aged women (median age 52), variable size, and focally infiltrative margin. The preoperative clinical diagnosis for all patients was of a benign lesion, with a diagnosis of lipoma in two. Four of six cases had the usual histologic appearance of a well-differentiated liposarcoma/atypical lipomatous tumor with variation in adipocyte size, adipocytic nuclear atypia, and occasional lipoblasts. Two of the six cases had a very unusual histologic appearance, not previously described, with an admixture of neoplastic bland spindle and round cells along with adipocytes showing variation in size as well as numerous mainly bivacuolated lipoblasts. Liposarcomas of other types were not identified. Follow-up data were available for five of the six patients and all were treated by excision with no additional treatment. Four showed no sign of recurrence at 12, 14, 18, and 84 months. One tumor was incompletely excised, regrew over a 10-year period, was re-excised, and showed no further recurrence 31 months thereafter. Liposarcoma can occur in the vulva, is predominantly of the well-differentiated subtype, can show an unusual histologic appearance with an admixture of spindle cells and numerous lipoblasts, and seems to have a behavior similar to these tumors in other anatomic locations.

Adult↗
PubMed1988

Carcinoma in situ of the vulva: 24 years' experience in southwest Florida.

We retrospectively studied 125 patients treated for carcinoma in situ of the vulva from 1961 through 1984, with follow-up ranging from one to 24 years. Patients' ages ranged from 24 to 90 years, with a mean age of 53 years. Multifocal disease was more common in women under age 40. Pruritus was the most common complaint, but 60% of the patients were asymptomatic. Twenty-five patients (20%) had other associated malignancies: carcinoma in situ of the cervix in ten patients, invasive carcinoma of the cervix in six, multifocal carcinoma in situ involving vulva, vagina, and cervix in five, vaginal carcinoma in situ in two, and endometrial carcinoma in two. Sixty-five patients were treated primarily with wide excision, 45 with total vulvectomy, seven with "subtotal" vulvectomy, three with skinning vulvectomy, and three with vulvectomy and bilateral groin node dissection; two patients refused treatment. Fifteen patients had a recurrence--recurrent carcinoma in situ in ten, and invasive carcinoma of the vulva in five. Whether the patient had had total vulvectomy or a lesser procedure appeared to make no difference in the recurrence rate. Thus a well planned excision of vulvar carcinoma in situ appears to be the treatment of choice. All recurrences were in patients over age 40, so although the lesion is histopathologically identical in the younger patients, its biologic behavior seems to be more benign. Since it is possible, however, that invasive disease will ultimately develop in some of the younger patients, careful follow-up is still required.

Adult↗
PubMed2003

Lymphoepithelioma-like carcinoma of the vulva.

Lymphoepithelioma is well known in the nasopharyngeal region. Although this neoplasm is well described in a variety of extranasal sites, where it is termed lymphoepithelioma-like carcinoma, this is only the second report of this neoplasm originating in the vulva reported in the literature. These lesions are reported to be less aggressive in extranasal sites. A 40-year-old woman presented with a lymphoepithelioma-like carcinoma of the vulva, diagnosed by excisional biopsy, which had been present for 1 month. The lesion persisted with ipsilateral lymph node involvement 4 months after initial resection, when the patient returned for definitive surgery. Lymphoepithelioma-like carcinoma can occur rarely in the vulva, and may behave aggressively.

Journal Article↗
PubMed2005

Mucous membrane pemphigoid of the vulva.

BACKGROUND: Mucous membrane pemphigoid is a rare autoimmune blistering disease primarily affecting mucosal surfaces. Blistering and scarring may occur in the eyes, mouth, esophagus, larynx, and on the vulva. Scarring can result in severe structural changes to the vulva that may mimic the findings of other inflammatory dermatologic disorders of the vulva, including lichen sclerosus and lichen planus. CASE: A 58-year-old woman presented with vulvar erosions, esophagitis, and laryngeal blisters. The clinical picture and the histopathology of a vulvar biopsy were suggestive of erosive lichen planus. Direct immunofluorescence, however, revealed findings diagnostic of mucous membrane pemphigoid. CONCLUSION: This case illustrates the importance of examining extragenital mucosal surfaces of any woman presenting with vulvar lesions. In addition, it demonstrates the importance of vulvar biopsy and the usefulness of direct immunofluorescence to differentiate between conditions with similar clinical and histopathologic changes.

Biopsy, Needle↗
PubMed2005

Inflammatory dermatoses of the vulva.

Inflammatory, non-neoplastic epidermal alterations of the vulva can be correctly diagnosed using classification schemes applied to skin elsewhere on the body. A wide range of inflammatory disorders may occur on the vulva, and they may have a similar clinical presentation to HPV lesions. However, HPV is incurable and often is treated surgically. Accordingly, as inflammatory dermatoses commonly occur on the vulva and are often curable with topical therapy, an awareness of these entities and an ability to distinguish them from HPV are imperative.

Child↗
PubMed2005

The basement membrane zone and dermal extracellular matrix in erosive lichen planus of the vulva: an immunohistochemical study demonstrating altered expression of hemidesmosome components and anchoring fibrils.

Histopathology demonstrates disruption of the basal layer of the epidermis in lichen planus (LP) and altered expression of basement membrane zone (BMZ) components occurs in cutaneous and oral LP. This is the first study in erosive LP of the vulva to investigate the expression of components of the BMZ and extracellular matrix by indirect immunofluorescence. Six biopsies from lesional vulval erosive LP were compared with two biopsies from normal vulva and five biopsies from normal skin. In erosive vulval LP there was widespread disruption of several BMZ components compared to normal skin. The hemidesmosome antigens were disrupted and attenuated, or absent. Expression of lamina lucida proteins and anchoring filaments also showed some alteration. Lamina densa components were altered and in particular there was very marked thickening, streaking and fragmentation of the anchoring fibrils. Some dermal extracellular matrix proteins were increased. This study has demonstrated widespread damage to the BMZ in erosive LP of the vulva, in particular the hemidesmosomes (alpha6beta4 integrin, BP230, BP180) and anchoring fibrils (collagen VII). This suggests an alteration in antigenic expression in the BMZ that may lead to exposure of epitopes and thus make these proteins vulnerable to attack by autoantibodies.

Autoantigens↗
PubMed1979

Granular-cell tumors of the vulva: a report of three cases.

Three black women were found to have granular-cell tumors on the vulva; in one, there were several additionally in other cutaneous sites. Although this benign neoplasm is uncommon on the vulva, it should be considered in the differential diagnosis of nodular lesions on the vulva. Granular-cell tumors in multiplicity occur frequently and are not a sign of metastatic disease. This lesion has predilection for blacks. Pseudocarcinomatous hyperplasia occurs typically in overlying squamous epithelium and may be misinterpreted as squamous-cell carcinoma.

Adult↗
PubMed1995

Squamous cell carcinoma in situ arising within lichen planus of the vulva.

BACKGROUND: Lichen planus is a common dermatologic disorder involving the glabrous skin, hair-bearing skin and scalp, nails, and mucous membranes of the oral cavity, penis, and vulva. There have been multiple case reports of squamous cell carcinoma (SCC) developing within lesions of hypertrophic cutaneous lichen planus and erosive oral lichen planus. Recently, there have been several case reports of SCC arising within lesions of the genital mucosa: one case of penile lichen planus, and two cases of vulvar lichen planus. OBJECTIVE: To report a case of SCC in situ developing within erosive vulvar lichen planus. To heighten the awareness, and facilitate the recognition of vulvar lichen planus. RESULTS: We present SCC in situ developing within a lesion of erosive vulvar lichen planus. The patient was treated with simple total vulvectomy by gynecologic surgery. Her vulva remain disease-free 2 years postoperatively. CONCLUSIONS: Vulvar lesions of lichen planus should be recognized and followed closely. In long-standing erosive lesions of the vulva, periodic sampling biopsies should be examined to rule out malignant degeneration.

Aged↗