CLINICAL PRACTICE GUID ELINES

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1 t SOGC CLINICAL PRACTICE GUID ELINES No. 2, May 1993 This has been provided by the Oncology Committee, Socieq of Obstetricians and Gynaecologists of Canada CURRENT CONCEPTS IN MANAGEMENT OF VULVAR CANCER INTRODUCTION I Vulvar carcinoma is uncommon with an incidence of approximately four percent of all gynaecologic malignancies. Most (90%) are squamous cell carcinomas with melanomas, adenocarcinomas, basal cell carcinomas and sarcomas accounting for the remaining ten percent. Several changes have occurred in the presentation of vulvar cancer and the most appropriate way to treat it. In the early 1900s, most patients presented with advanced stage disease and were treated with local excision. Prognosis was poor, with 20 to 25 percent surviving five years. Radical en bloc vulvectomy and inguinal node dissection improved five year survival to 60 to 70 percent (1,2). Major operative morbidity and long term physical and psychological morbidity resulted. In the 1980s, most vulvar cancer presented as Stage I or II disease. Patients are now more likely to seek medical help at the onset of symptoms and physicians recognize the importance of examination and biopsy prior to initiation of treatment. In 1988, the International Federation of Gynaecology and Obstetrics (mco) introduced a new surgical/pathological staging system for vulvar carcinoma (Table I) (3). A surgicavpathological system was instituted because of the inaccuracy of estimating clinical node status and the prognostic signacance histologically proven inguinal node metastasis. A standard treatment for vulvar cancer cannot be applied to all patients. Each patient needs to be carefully evaluated clinically and a treatment plan initiated based on clinical findings and altered, when appropriate, depending on surgicavpathological outcome. Four groups of patients may be identified. Principal Author: Mark Heywood, University of Manitoba J ulnical Practice Guidelines: These Clinical Practice Guidelines do not define a standard of care nor is it intended to dictate an exclusive course of treatment of procedure to be followed. It presents methods and techniques of clinical practice that are acceptable and used by recognized authorities for consideration be obstetricians and gynaecologists, and incorporating into their practice. Variations of practices taking into account the need of individuals, patient resources and limitations unique to the institutions or type of practice may be appropriate. A guideline can and will be modified by local conditions, if so it should be documented in individual departments and/or hospitals. Copies available, $5 each. Les Directives cliniques n ont pas pour objet de définir un étalon à suivre pour la promulgation des soins. Elles n ont pas été élaborées non plus pour dicter une procedure ou un mode de traitement exclusifs à suivre. Elles présentent plut& des méthodes et des techniques de pratique medicale qui sont acceptées et utilisées par les autorités compétentes dans le domaine. Les obstétriciens, obstétriciennes et gynéocologues peuvent s en inspirer et les appliquer dans leur pratique. Les méthodes de pratique proprement dites pourraient varier selon les besoins de la personne, les restrictions et les ressources à I egard des malades au sein d un établissement quelconque et le genre de pratique exercé. il se peut que l on soit obligé de dévier des directives cliniques à cause des conditions du milieu; en I occurrence, il faut qu il y ait documentation à i appui au sein du département ou de l hôpital. Copies disponibles, 5,00$ chacune.

2 1. Superficially Invasive Vulvar Cancer This term is identified by the International Society for the Study of Vulva Disease (ISSVD) as a solitary tumor, two cm or less in diameter, with a depth of invasion into the underlying stroma of one mm or less (4). The deepest point of invasion is measured from the nearest most superficial dermal papillae (Fig. 1). Since the Parker et al. initial report of incidence of lymph node metastases, it has become apparent that only tumors with one mm or less of invasion are not at risk for nodal metastasis (Table II) (5). These lesions may be treated with wide local excision without inguinal femoral lymphadenectomy. Wide local excision means excision of the tumor with two to three cm margins of normal tissue to the lesion and, in a vertical plane to fascia or symphysis pubis. Should final histopathological analysis reveal greater than one mm of invasion with a nonmidline lesion, then an ipsilateral inguinal femoral lymphadenectomy should be performed. It has been recommended that the superficial ipsilateral groin nodes be removed immediately prior to a wide local excision to preclude the possibility of metastatic disease. 2. Stage I Vulvar Cancer In the past, the standard of care for all vulvar cancers was an en bloc radical vulvectomy and inguinal femoral node dissection. This led to an overall five year survival rate of 60 to 70 percent. However, 50 percent of patients are now presenting with early stage disease (Stage I). Additionally, the radical en bloc dissection has significant postoperative morbidity. Morbidity is related to wound breakdown with prolonged hospitalization and chronic leg oedema. Major long term morbidity is related to the psychosexual changes. Anderson and Hacker reported sexual arousal amongst those patients at the eighth percentile and body image amongst the same group at the fourth percentile compared with a control group of women who had not undergone vulvectomy (6). There is no standard therapy of Stage I cancer of the vulva. The appropriate surgical procedure is that which offers the highest probability of cure with minimal physical and psychosexual morbidity. Two components require consideration for treatment: the primary lesion and the inguinal femoral lymph nodes. A more conservative surgical excision rather than the en bloc dissection generates two major concerns. The first is that the nature of the disease is a diffuse one and, therefore, the whole vulva requires treatment. The second is that if an en bloc dissection is not performed, the skin bridge (the skin between the lesion and the nodal resection) will be at high risk as a site for recurrence. Fortunately, available data have not substantiated these concerns. Radical local excisions (removal of the tumor with two to three cm margins of normal tissue, carried down to fascia or symphysis pubis) with inguinal femoral node dissection through separate incisions has provided at least an equal opportunity of cure (Table III). It is important that when there is greater than one mm of invasion that at least ipsilateral inguinal femoral lymphadenectomy be performed as there is a 14 percent (49/340) chance of lymph node involvement (Table I). The prognosis from recurrent disease if the groin is not dissected is dismal with 89 percent of patients dying from the disease (7). If the primary lesion is unilateral, away from the midline, then an ipsilateral groin dissection is sufficient. Only one report has shown positive contralateral nodes with negative ipsilateral nodes in Stage I disease for a cumulative incidence of 0.4 percent (2/488 patients (7,8). The technique of inguinal femoral node dissection through separate incisions, initially described by Byron et al. and subsequently modified by Hacker et al., has provided several benefits (9, 10). Postoperative hospital stay decreased to a mean of 19 days, with chronic leg oedema occurring in 20 percent of patients compared with previously reported incidence of between 32 percent and 65 percent (11, 12). It was stated that the reduced frequency of leg oedema with separate incisions was due to a reduction in major groin incision breakdown (Table IV). Of the 14 percent of patients who have positive inguinal nodes, a decision has to be made regarding the role of adjunctive treatment. Those with less than three positive nodes have a low incidence of groin or pelvic recurrence (2.9% groin, 0% pelvic) (13). Adjunctive groin and/or 3

3 i pelvic radiotherapy is probably not justified. In the series of Hacker et al., none of 50 Stage I patients had three or more nodes positive and in the Gynaecologic Oncology Group Study 9 of 190 (4.7 %) had three or more positive. Those few patients with three or more positive nodes should probably receive ipsilateral groin and pelvic radiotherapy as there is a high incidence of groin and pelvic recurrences (33% groin, 44% pelvic) (13, 14). Systemic recurrences are even more frequent (66%)..-,) -l 3. Clinical Stage II or III In general, those patients in whom groin nodes are not clinically suspicious should be treated with a radical vulvectomy and bilateral inguinal femoral lymphadenectomy through separate incisions. When inguinal nodes are suspicious or positive, then the en bloc dissection should be used as it is in this situation that retrograde lymphatic permeation may occur, leading to skin bridge recurrences. The Gynaecological Oncology Group Study reported that radiation therapy to the pelvis and groin was superior to pelvic lymphadenectomy as adjunct therapy when there were two or more positive nodes (15). Two year survival was 63 percent for the radiation therapy group compared with 37 percent for the pelvic lymphadenectomy group. There was no difference with only one node positive. The groups were not separated into less than three nodes vs three or more nodes positive; therefore, it is not possible to determine if those with two nodes positive would benefit from radiation therapy. Hacker et al. have demonstrated that pelvic nodal metastasis did not occur unless the inguinal nodes are clinically suspicious or there are three or more histologically positive unilateral nodes (1 3). Adjunctive radiation therapy, therefore, should be used when there are three or more positive inguinal nodes. Radiation therapy to the ipsilateral groin and pelvic sidewalls should be recommended in those patients with unilateral nodal metastasis, and whole pelvic and bilateral groin radiation therapy should be recommended in those patients with bilateral nodal involvement. The benefit of radiation therapy for two positive unilateral nodes remains to be determined. 4. Advanced Disease Advanced disease is uncommon. It includes tumors in Stage III or IV where the primary tumor involves anus, rectum, rectovaginal septum and/or upper urethrabladder. A surgical approach might include radical vulvectomy and inguinal node dissection with a pelvic exenteration, either posterior, anterior or complete. There is high operative morbidity and mortality with additional long term, psychological, and physical morbidity. However, in selected groups, a 50 percent five year survival is achievable. As an alternative to such radical surgery, Boronow proposed, in 1973, an approach using both radiation therapy and surgery (16). In 1982, Boronow reported 26 patients with primary tumors treated with combined therapy (17). Sixty- five percent were alive from one to eleven years post treatment. No patient required exenteration. The radiation therapy component was brachytherapy (1 1 patients), external beam and brachytherapy (14 patients) or external beam alone (1 patient). Surgery was radical vulvectomy plus or minus inguinal/pelvic lymphadenectomy. Hacker et al. similarly reported eight patients with advanced vulvar lesions treated with external radiation therapy and individualized surgical resection (18) (which were with no evidence of fixed pelvic or distant spread). Five of the eight (62.5%) were alive without clinical disease between 15 months and 10 years after therapy. All patients developed moist desquamation secondarily to radiation therapy, 50 percent requiring temporary cessation of therapy. Surgery was possible four to six weeks following radiation therapy. In four patients (50%), there was no residual tumor within the surgical specimen, suggesting that vulvar lesions responded to radiation more like squamous cell carcinoma of the skin than that of the cervix or vagina. The radiation field used was to the vulvar lesion alone in those patients with clinically nonsuspicious groin nodes but extended to include groin and pelvic nodes in those patients with suspicious or positive groin nodes,

4 FIGURE 1 DEPTH OF INVASION / \ TUMOR THICKNESS

5 TABLE I STAGING ) Stage I Tumor confined to the vulva and/or perineum, two cm or less in greatest dimension, nodes are not palpable Stage II Tumor confined to the vulva and/or perineum more than two cm in greatest dimension, nodes are not palpable. Stage III Tumor of any size with: 1. Adjacent spread to the lower urethra and/or the vagina, or the anus, and/or 2. Unilateral regional lymph node metastasis Stage IVa Tumor invades any of the following: Upper urethra, bladder mucosa, rectal mucosa, pelvic bone and/or bilateral regional node metastasis Stage IVb Any distant metastasis, including pelvic lymph nodes TABLE II INCIDENCE OF LYMPH NODE METASTASES VS DEPTH OF INVASION IN STAGE I CANCER OF THE VULVA DEPTH OF INVASION NUMBER POSITIVE NODES PERCENT <1 mm 120 O O mm mm mm mm >5 mm Total From Berek JS, Hacker NF. Practical Gynecologic Oncology, Williams and Wilkins, 1989, p. 397.

6 TABLE III LOCAL RECURRENCE VS SURGICAL TREATMENT OF PRIMARY TUMOR IN STAGE I CANCER OF THE VULVA SURGICAL METHOD NUMBER NUMBER REQUIRED PERCENT Radical Local Excision Radical Vulvectomy From Berek JÇ, Hacker HW. Practical Gynecologic Oncology, Williams and Wilkins, 1989, p. 401 TABLE IV CHRONIC LEG OEDEMA IN PATIENTS WITH SEPARATE GROIN INCISION INGUINAL LYMPHADENECTOMIES WOUND BREAKDOWN NUMBER CHRONIC LEG OEDEMA PERCENT Yes No Total From Hacker et al (10) 3

7 i REFERENCES 1. Taussig FJ: Cancer of the vulva: An analysis of 155 cases. Am J Obstet Gynecol 1940, 40: Way S. Carcinoma of the vulva. Am J Obstet Gynecol 1960, 79: Int J Gynecol Obstet 1989, 28: Wilkinson EJ, Kreate BL, Lynch PJ, ISSVD. Report of the ISSVD Tumorology Committee. J Reprod Med 1986, 31: Parker RT, Duncan I, Rampone J, et al. Operative management of early invasive epidermoid carcinoma of the vulva. Am J Obstet Gynecol 1975, 123: Anderson BL, Hacker NFG. Psychosexual adjustment after vulvar surgery. Obstet Gynecol 1983, 62: Berek JS, Hacker NF. Practical Gynecologic Oncology, Williams and Wilkins 1989, pp Magrina JF, Webb MJ, Gaffey TA, et al. Stage I squamous cell cancer of the vulva. Am J Obstet Gynecol 1979, 134: Byron RL, Lamb EJ, Yonemoto RM, Kase S. Radical inguinal node dissection in the treatment of cancer. Surg Gyn Obstet 962, 114: Hacker NF et al. Radical vulvectomy and bilateral inguinal lymphadenectomy through separate groin incisions. Obstet Gynecol 1981, 58: Rutledge F, Smith JP, Franklin FW. Carcinoma of the vulva Am J Obstet Gynecol 1970, 106: Calame RJ. Pelvic relaxation as a complication of radical vulvectomy. Obstet Gynecol 1980, 55: Hacker NF et al. Management of regional lymph nodes and their prognostic influence on vulvar cancer. Obstet Gynecol : Homesley H et al. Assessment of current International Federation of Gynecology and Obstetrics staging of vulvar carcinoma relative to prognostic factors for survival (A Gynecologic Oncology Group Study). Am J Obstet Gynecol 1991, 164: Homesley H et al. Radiation therapy versus pelvic node resection for carcinoma of the vulva with positive groin nodes. Obstet Gynecol 1986, 68:

8 16. Boronow RC. Therapeutic alternative to primary exenteration for locally advanced vulvovaginal cancer. Gynecol Oncol 1973, 1: Boronow RC. Combined therapy as an alternative to exenteration for locally advanced vulvovaginal cancer Cancer 1982, 49: Hacker NF et al. Preoperative radiation therapy for locally advanced vulvar cancer. Cancer 1984, 54: Reprints and copies can be ordered by writing to Dr. André Lalonde, SOGC s Executive Vice President, at the following address: 1785 Alta Vista Drive, Suite 102, Ottawa, Ontario, KlG 3Y6.