Malignant diseases of the vulva account for an estimated 3% to 5% of gynecologic neoplasia. The pathologic variants are many (Table 1). Squamous cell cancers account for 85% to 90% of these neoplasms. Melanoma, Bartholin gland cancer, Pagets disease, and the various sarcomas are the other principal neoplasms. The preinvasive forms of the squamous cancer tend to occur in younger women and may be associated with in situ lesions of the cervix, vagina, perineum, and anus.
Detection of the disease simply requires a minimal level of awareness on the part of the patient and examination with a significant level of suspicion on the part of the physician.
Patients with vulvar cancer frequently will be aware of a long-standing pruritus vulvae before they may be aware of a mass or lump. Localized vulvar complaints may include pain, burning, and discomfort of the vulva when voiding. Discharge and bleeding may develop. Although some patients come to the physician because of increasing size of a mass, delay is a more common feature.
The delay in diagnosis often occurs because a physician may write a prescription for symptoms without having performed a physical examination. Careful vulvar inspection and biopsy of any new lesion or suspicious area are mandatory.
To the experienced clinician, the diagnosis of a frankly malignant process will be evident. A high level of suspicion of any new lesion is appropriate. The clinician is well-advised to biopsy any new growth or suspicious change in the vulvar epithelium.
The TNM descriptive material and the International Federation of Gynecology and Obstetrics (FIGO) staging are displayed in Table 2. The purpose of staging, whether clinical or surgical, is to allow a common international vocabulary for comparison of data and to reasonably reflect clinical extent of disease. It will also identify subsets of patients with worrisome prognostic factors.
Therapy is obviously influenced by stage of disease, but other factors in addition to those that are included in the FIGO staging criteria (TNM) must be considered. These considerations include not only the size but also the location of the lesion. Histologic features include grade, depth of invasion, and the presence or absence of lymph-vascular space invasion. Equally important is the evaluation of the patients health status and ability to undergo various procedures.
While the current trend in vulvar cancer therapy reflects greater emphasis on individualization and more conservative treatment, the clinician must remember the events that led to the standardization of radical vulvectomy and groin dissection routinely for vulvar cancer. The move to decrease the radicality, thereby decreasing morbidity and major alteration of body image, may be associated with an increase in problems by failure of local control. Thus, caution when considering conservatism is of utmost importance.
Carcinoma in Situ (CIS) and Vulvar Intraepithelial Neoplasia (VIN)The first decision is whether or not to treat. The progression of the various grades of intraepithelial neoplasia to invasive cancer is generally considered to be a very slow and protracted process, except in immunocompromised patients. Opinion is divided as to whether mild and moderate grades of dysplasia (VIN I and II) require therapy. If symptomatic, treatment is certainly justified. VIN III (severe dysplasia and carcinoma in situ) should be treated, but there are instances in the very debilitated patient where surveillance may be permitted.
The extended resections listed in Table 4 depend on the extent of disease. Gross surgical clearance should be the goal, and with the major thrust toward conservative resection, limited excisions rather than more radical (prophylactic) surgical resection may now be performed. Vulvar laser therapy and topical fluorouracil(Drug information on fluorouracil) (5-FU) are selectively employed. Both are associated with significant pain. The latter is associated with a substantial relapse rate.
Microinvasive Vulvar CancerThe International Society for the Study of Vulvar Disease (ISSVD) and FIGO define stage IA carcinoma of the vulva as a single lesion measuring 2 cm or less in diameter and with a depth of invasion of 1 mm or less. Cases with more than one site of invasion were excluded, but the definition did include cases that have lymph-vascular space involvement.
There seems to be a consensus that wide local excision is adequate if the invasive process is 1 mm or less, a T1 lesion (2 cm or less), and without lymph-vascular space invasion. Some also feel that a highly undifferentiated lesion should not be considered for local resection only. If the local excision reveals features less favorable than those noted on the initial biopsy, a more radical excision should be performed. Groin dissection is not necessary for this lesion but may be considered if significant unfavorable features are found.
Clearly Lateral T1 and T2 LesionsRadical local excision with ipsilateral groin dissection to include the superficial and deep groin nodes is commonly employed for these presentations. The separate incision technique is often employed. The other approach is a radical hemivulvectomy with en bloc ipsilateral groin dissection, which keeps in continuity soft tissue and lymphatics between the primary and the groin dissection.
There is general agreement that the laterality be clearly defined, and a good guideline is that the margin of surgical clearance around the lesion should be 2 cm and the lesion be at least 2 cm from the introitus, the urethra, the posterior fourchette, or clitoris. Some modification is reasonable for more anteriorly or more posteriorly situated lesions. With the lymphatic drainage primarily anterior to the groin, posterior dissection might be omitted with a more anteriorly situated lesion. Clitoral sparing is permissible in selected posterior lesions. The dissection should involve the full thickness of the vulva down to the muscular fascia of the urogenital diaphragm.
Recent evaluation of radical local excision with superficial groin dissection without clearance of the deep groin nodes has reflected a higher than anticipated groin relapse and should be employed only in the most selected circumstances. Until data from clinical series using more conservative approaches have matured more fully, many oncologists still favor radical vulvectomy with bilateral groin dissection, especially for T2 lesions.
T1 and T2 Midline Lesions or Bilateral LesionsThe two most commonly employed surgical techniques are the standard radical vulvectomy with en bloc bilateral groin dissection, and the radical local excision with bilateral groin dissection. As in the previous section, individual surgeon preferences favor either the en bloc one incision or separate incisions with intervening skin bridges. Wound healing and morbidity may be slightly more favorable with the latter approach. Larger, more anterior lesions are probably not suitable for consideration of the separate incision approach.
Lesions With Clinically Positive Groin NodesThe radical vulvectomy with bilateral groin dissection is the basic surgical approach. The options at that point include pelvic node dissection or pelvic node exploration to remove any enlarged nodes clinically or radiographically identified.
Postoperative radiation therapy with or without chemotherapy should be added in all but the most exceptional case. Occasionally, preoperative pelvic and groin radiation therapy will be employed followed by a modified groin dissection and vulvectomy. The groin dissection in this instance can be modified with thicker skin flaps and limited to a more conservative dissection when preceded by radiation for occult disease control.
Lesions With Fixed/Ulcerative Groin NodesThere are two ways to treat the groins in this setting. One is primary radical surgery, and the second is primary radiation therapy. Primary radical vulvectomy with groin dissection to clear groin disease may be employed but is frequently a debulking exercise with residual disease intimately involving the femoral vessels. Titanium clip localization can provide a target for an electron-beam boost when postoperative radiation therapy is employed. If preoperative radiation therapy to the vulva, pelvis, and groin is employed with or without chemotherapy, it is followed by more conservative surgery to the vulva and groin. If complete resection was not possible, the residual field could be boosted with electron-beam therapy. Occasionally, the use of myocutaneous flaps or sartorius transposition is considered to cover the femoral vessels as a technique to reduce the risk of vascular blowout.
Vulvar T3 LesionsMinimal involvement of the distal urethra, the vagina, or the anus may lend itself to an extended viscera-preserving radical vulvectomy with bilateral groin dissection. Postoperative radiation therapy may or may not be added. The differences between early T3 lesions and T4 lesions as defined are quite obvious, but clinical expressions of disease are not always neatly distal urethral vs upper urethral or bladder mucosa. Vaginal extension may be quite significant, and anal involvement might be prominent even in the absence of rectal mucosal invasion. Therefore, the significant impact on relapse and survivorship of urethral, vaginal, and anal involvement must not be overlooked. Preoperative pelvic radiation therapy with or without brachytherapy and with or without chemotherapy followed by conservative surgery has produced good results. Exenterative surgery or modification, such as anovulvectomy, may be necessary in selected instances.
Vulvar T4 LesionsFor massive involvement of bladder and/or vagina and/or rectum, selective exenterative surgery with radical vulvectomy and groin dissection may offer the only hope of clearance. A modification would be the radical anovulvectomy for posterior lesions. A note of caution is the virtually nonexistent survivorship with exenterative surgery in the presence of positive nodes. Preoperative pelvic and groin radiation therapy with or without brachytherapy and with or without chemotherapy may produce sufficient regression to allow debulking surgery tailored to the disease presentation or to a modified exenterative procedure.