SQUAMOUS CELL ARCINOMA OF VULVA :
Treatment
A small primary lesion on the vulva (ie, <2 cm) with superficial invasion (ie, <1 mm from the epithelial stromal junction of the adjacent, most superficial dermal papillae) has essentially no risk of lymph node metastasis. Consequently, these lesions can be treated with wide local excision, ensuring that adequate surgical margins are present (not only on the skin but also deep margins).
In larger lesions (ie, stage IB or greater or with stromal invasion >1 mm), the incidence of ipsilateral inguinal lymph node involvement increases as the depth of invasion, as well as the gross size, increases. Consequently, inguinal lymphadenectomy is part of the primary surgical procedure. This can be performed through a small separate inguinal incision, removing the lymph nodes above the cribriform fascia and in the opening of the fascia at the fossa ovalis. If the results are negative on frozen section of these lymph nodes, then a modified partial vulvectomy is the only treatment necessary. If the results on frozen section of the ipsilateral lymph nodes are positive, then most physicians suggest removing the lymph nodes on the contralateral inguinal area as well.
The lesion itself can be treated conservatively, with a partial vulvectomy. Performing complete vulvectomy is an outdated treatment unless the cancer is present bilaterally. If clitoral involvement is present, lymphatic drainage can be direct to the pelvic lymph nodes. Studies have demonstrated that, even with clitoral involvement, the deep lymph nodes are not involved unless the inguinal nodes have evidence of metastasis also. Pelvic lymphadenectomy is largely discontinued, even in cases of lymph node involvement. A large, prospective, randomized study conducted by the Gynecologic Oncology Group (GOG) noted that patient survival rates are better if the pelvic and inguinal area is treated with radiation postsurgically, as compared to patients treated with pelvic lymphadenectomies, even when the pelvic nodes are not involved. Incidence of lymph node metastasis seems to be increased if vascular lymphatic space is involved.
Prognosis
Contemporary data suggest that the overall 5-year survival rate of patients with stage I epidermoid invasive cancer is 85-90%. The survival rate decreases with increasing stage; however, an approximate 5-year survival rate of 40% can be obtained, even in patients with lymph node metastasis.
In a review of the National Cancer Data Base, patients with positive inguinal lymph nodes were found to have 5-year survival rates of 64% with 2-cm lesions and 43% with lesions greater than 2 cm. In patients with primary lesions of any size, the survival rate was identical whether 1 lymph node was positive or 2-3 lymph nodes were positive (55% vs 59%). The survival rate in patients with 4 or more positive nodes was only 33% at 5 years. The 5-year survival rate of patients with 1 positive node, without radiation, was 68% and was 56% with radiation. If 2 or more lymph nodes were positive, the survival rate was 46% without radiation therapy and 48% with radiation.
The 2003 Annual Report noted a 5-year survival rate of 69% in patients with negative lymph nodes who were treated with surgery only, compared to 67% for patients treated with surgery and radiation. If lymph nodes were positive, the survival rate was 35% in patients treated with surgery alone, compared to 49% in patients treated with surgery plus radiation. These studies raise the question of whether or not postoperative radiation therapy is as advantageous as the GOG study noted.
Follow-up
More than 80% of recurrences appear within the first 2 years after therapy, and they may be either local or distant. Because many reoccurring lesions appear locally and near the site of the primary lesion, initial close follow-up is necessary. Visual examination provides the best follow-up.
Local recurrences are more common in patients with large primary tumors than in patients with metastatic disease in the lymph nodes, and local occurrences can appear when the margins are clear on the original operative specimen. Local recurrences can be managed successfully in many instances by repeat local excision and/or interstitial radiation.
Recurrent lesions in the lymph node area, as well as in distant sites, are difficult to treat, and survival rates are poor. If recurrences appear in the inguinal area, excision with or without radiation therapy may be beneficial. Distant metastasis are treated most effectively with chemotherapy, with cisplatin as the drug of choice, and 30% response rates have been achieved in reports.
MELANOMA :
Treatment
In the past, this lesion was treated with radical vulvectomy and bilateral inguinal lymphadenectomy. In recent years, more conservative treatment, such as has been practiced for this lesion elsewhere on the body, has become more common. A radical local excision with 2-cm margins appears to be adequate for most well-circumscribed lesions. Whether inguinal lymphadenectomy should be performed for this cancer is undecided at present. Obviously, if lymph nodes are involved, this finding is not only diagnostic but also prognostic. If lymph nodes are negative, the patient may be reassured. Lymph node involvement is directly related to the depth of invasion. If the disease is intraepithelial, the cure rate is close to 100% and is reported to be as high as 99% with invasion of 1.5 mm or less. The survival rate drops to 65-70% if the lesion invades 1.5-4 mm. Medical management for metastatic disease continues to be experimental. If the melanoma recurs locally in the vulvar area, reexcision may be adequate therapy, with long-term survival.
PAGET DISEASE :
Clinical and histological features
Vulvar lesions usually are hyperemic, and they may be demarcated sharply and thickened with foci of excoriation and induration. The vulvar skin may be thick, leading to the impression of leukoplakia with the cake icing effect. This classic finding almost is pathognomonic for Paget disease. The lesion is usually superficial and is considered an intraepithelial lesion, although an underlying adenocarcinoma may be associated with Paget disease. Older literature suggests that underlying adenocarcinoma occurs in about a quarter of cases, but more recent data find less association of Paget disease with an underlying adenocarcinoma.
Obtain adequate biopsies to make an accurate diagnosis. Histologically, it commonly presents with large cells of clear cytoplasm in a heavy lymphocytic infiltration in the dermis; it can be confused with melanotic melanoma. If any thickened indurated area is present, then obtain adequate deep biopsy in order to rule out adenocarcinoma.
Treatment
If only intraepithelial Paget disease is present, wide local excision is adequate treatment. Histological evaluation of the epithelium extends, in many instances, far beyond the visual limits of the lesion, and, therefore, wide adequate margins are necessary to remove the lesion. If an underlying adenocarcinoma is present, then treat the lesion as invasive squamous cell carcinoma is treated. If tumor cells are present at the margin of the excision, then recurrence can be quite high. Some investigators obtain frozen section of the margins and, if positive, continue to obtain wider margins. Unfortunately, even with negative margins, recurrences are possible, and new lesions can be treated in the same manner as the primary disease (ie, wide local excision). These may occur years after diagnosis of the primary lesion.
Currently, intraepithelial Paget disease and Paget disease with an underlying adenocarcinoma are thought to be 2 separate entities. The cases of patients who were diagnosed with Paget disease and who refused primary treatment have been followed for over a decade without development of an underlying adenocarcinoma. As with other vulvar lesions, frequent visual examinations are necessary in order to determine disease-free status.