eMedicine Specialties > Obstetrics and Gynecology > Gynecologic Surgery

Surgical Treatment of Vulvar Cancer: Treatment

Author: Robert V Higgins, MD, Clinical Associate Professor, Department of Obstetrics/Gynecology, University of North Carolina School of Medicine; Vice-Chairman of Academic Affairs, Associate Director of Gynecologic Oncology, Residency Director, Department of Obstetrics and Gynecology, Carolinas Medical Center
Coauthor(s): R Wendel Naumann, MD, Clinical Assistant Professor of Obstetrics and Gynecology, University of North Carolina at Chapel Hill; Associate Director, Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Carolinas Medical Center; James Hall, MD, Director, Division of Gynecologic Oncology, Clinical Associate Professor, Department of Obstetrics and Gynecology, Carolinas Medical Center, University of North Carolina
Contributor Information and Disclosures

Updated: Nov 20, 2008

Treatment

Medical Therapy

Neoadjuvant therapy for vulvar cancer may be considered for tumors that manifest with bowel or bladder involvement that would require extensive or exenterative surgery. The Gynecologic Oncology Group (GOG) reported its experience with a combination of cisplatin and 5-fluorouracil with hyperfractionated radiation for patients with unresectable stage III or stage IV squamous cell carcinoma of the vulva. After chemotherapy and radiation, 71 of 73 women were candidates for surgery and almost half had no visible disease. Urinary and fecal continence was preserved in all but 3 of these women.⁷

Except in the neoadjuvant setting, chemotherapy for vulvar carcinoma is palliative and often ineffective. Only bleomycin has been reported to produce a complete clinical response. In a series of 11 patients, Trope and colleagues administered bleomycin at a dose of 15 mg twice weekly and noted 2 complete responses (19%) and 3 partial responses (27%), for a total response rate of 46%.¹²

The only other agent that has been reported to be effective in recurrent vulvar cancer is doxorubicin. Deppe et al reported a partial response in 3 of 4 patients treated at a dose of 45 mg/m².¹³

Cisplatin and 5-fluorouracil (5-FU) have been used in the neoadjuvant setting but have not been studied extensively in recurrent vulvar cancer. However, due to a lack of effective chemotherapy agents, these drugs are often used in recurrent vulvar cancer.

Surgical Therapy

Great effort has been devoted to decreasing the morbidity of surgery for vulvar carcinoma. Traditional surgery has been a large en bloc resection of the vulva with the superficial and deep inguinal nodes through a single incision with at least 2-cm margins around the tumor and deep resection to the genitourinary diaphragm (see Media files 3-4). This procedure resulted in significant surgical morbidity and distressing change in body image for the patient.

Refinements to surgery include (1) a definition for microinvasive carcinoma that does not require radical vulvar dissection or inguinal node dissection, (2) unilateral inguinal node dissection for small ipsilateral tumors, (3) using a triple-incision technique instead of an en bloc approach, (4) using radical local resection with 1-cm margins instead of complete vulvectomy, and (5) sparing the saphenous vein in an attempt to prevent lymphedema.^14,15

Many gynecologic oncologists omit dissection of the deep inguinal lymph nodes, although this is controversial. In an attempt to decrease the morbidity from inguinal node dissection, radiation alone has been used to treat the groin lymph nodes but was shown to be inferior to groin node dissection.¹⁶  

Sentinel lymph node (SLN) dissection may eventually replace routine groin node dissection.¹⁷ This idea assumes metastasis occurs first to the SLNs, followed by metastasis to the other inguinal lymph nodes. This orderly metastasis has been validated in breast cancer and cutaneous melanoma. Confirmation of this technique in patients with vulvar cancer continues to be examined. T

he results of lymphoscintigraphy and blue dye used in 59 patients with vulvar cancer was reported by de Hullu. All 37 SLNs interpreted as positive on frozen section were confirmed on final pathologic examination, resulting in a positive predictive value of 100%.¹⁸  A more recent study examined the use of identification of the SLN in patients with squamous cell carcinoma. Patients were divided into 2 groups. Of the women, 28 had a vulvectomy and lymphadenectomy with identification of the SLN using lymphoscintigraphy with technetium-99 colloid albumin and 27 women had a vulvectomy and lymphadenectomy with no attempt to identify the SLN. SLN identification failed in 1 case. There was 1 false-negative SLN. The average number of SLNs identified was 2.2. Recurrent disease was similar in both groups. The authors did not report which patients developed recurrent disease in the inguinal area.¹⁹

The initial proposal for microinvasive lesions was a depth of 5 mm for tumors smaller than 2 cm. However, the risk of groin node metastasis in lesions with 3-5 mm of invasion was noted to possibly be as high as 20%. It was later determined that women with 1-mm invasion or less had a negligible chance of node metastasis and could be treated with wide local excision with a 1-cm clinical margin around the tumor. A GOG study suggested that women with well-differentiated tumors up to 2 mm in depth had a very low risk of lymph node metastasis.

A study by Yoder and colleagues identified 3 features most important in predicting tumor recurrence: depth of invasion, presence of squamous cell carcinoma at the surgical margins, and the histologic grade.²⁰ Omitting the groin node dissection in women with 1-2 mm of invasion can be considered if the tumor is well differentiated and the patient is elderly, debilitated, or at significant risk of lymphedema.

• The risk of lymph node metastasis based on the depth of invasion
  • Invasion to less than or equal to 1 mm - 0% (n = 34)
  • Invasion to 1.1-2 mm - 10% (n = 19)
  • Invasion to 2.1-3 mm - 12% (n = 17)
  • Invasion to 3.1-5 mm - 14% (n = 7)
  • Invasion to deeper than 5 mm - 43% (n = 7)

In stage I lesions, 0 of 177 patients had positive findings from contralateral groin nodes when the ipsilateral node findings were negative and the lesion was not located in the midline. Therefore, contralateral groin node dissection has been omitted when patients present with a primary lesion smaller than 2 cm and have negative ipsilateral lymph node findings.

Since the early part of the 20th century, the traditional surgery has been a radical dissection of the primary lesion with a bilateral groin node dissection performed through a single incision. Although this technique was later modified to remove less skin, the primary wound breakdown rate exceeded 50%. Taussig eventually adopted a triple-incision technique in response to the high wound breakdown rate. Concern was raised over the triple-incision technique because of the possibility of residual disease in the "skin bridges" due to cancer cells in the lymphatics between the primary tumor and the lymph nodes. Hacker et al reported a series of 100 patients who had undergone surgery with a triple-incision technique and reported a 56% primary healing rate. Although 2 patients had metastasis in the skin bridge, neither of these instances were isolated metastasis.⁵

Helm et al reported findings when comparing the cases of 32 women treated with a single incision with 32 similar patients treated with a triple incision. Patients with a triple incision had a significantly shorter operative time, less blood loss, and a shorter hospital stay. No difference was observed in the overall survival or recurrence rate between the 2 groups, and none of the women in the triple-incision group developed skin bridge metastasis. The biggest difference in the 2 groups was that the single-incision group had a 19% complete wound-breakdown rate, compared to only 3% for the triple-incision group. Similar results have been noted for women with larger lesions.²¹

A less-radical approach was adopted following the discovery that the local recurrence risk was low when the pathologic margin around the primary lesion was 8 mm. When taking into account the shrinkage during tissue fixation, this translates to a 1-cm clinical margin. Deep dissection to the urogenital diaphragm is performed, but most of the vulva can be spared if the primary lesion is small.

The traditional description of a groin node dissection includes ligation of the saphenous vein during removal of the superficial groin lymphatics. A review of 139 cases of groin node dissection demonstrated that when the saphenous vein was preserved, the incidence of wound cellulitis and acute and chronic lymphedema was significantly lower. Only one patient in this series with saphenous vein preservation developed chronic lymphedema. This occurred in a patient who received postoperative radiation therapy. No evidence indicated that an attempt to save the saphenous vein significantly increased blood loss or operative time.¹⁴

The idea to implement radiation therapy to treat the groin lymphatics without surgery has been studied by the GOG. The GOG performed a study on the efficacy of groin irradiation compared to surgery. Women with clinically negative findings from groin nodes were randomized to radiation alone or lymphadenectomy with radiation when the groin nodes were pathologically positive. The study was discontinued prematurely as an interim analysis demonstrated a significant decrease in survival in women receiving only groin irradiation.¹⁶  

This study has been criticized due to the radiation dosage. The prescribed dose of radiation was at 3 cm, regardless of body habitus or the actual groin node location. Studies of CT scan data have demonstrated that this technique delivers 100% of the prescribed dose to only 18% of women, and fewer than half the women would have received more than 60% of the prescribed dose to the entire groin lymphatic area.

Many physicians now omit deep groin node dissection. Opening the femoral sheath and removing the deep nodes is not without morbidity. Deep lymph node dissection may increase the incidence of lymphedema. In addition, infection of the groin over the femoral vessels after deep groin dissection can result in catastrophic bleeding. The sartorius muscle historically was transferred to the inguinal ligament to cover the exposed femoral vessels. Judson reported this technique is not beneficial based on a randomized control trial and increased the risk of lymphocyst formation.²²  

Several authors have examined the incidence of unexpected groin failure in the presence of pathologically negative superficial lymph node findings. The incidence rate of unanticipated failure is approximately 0-5%.^23,24,25 These percentages match those of an older series by Stanley Way, in which he examined both nodal groups separately and found that the deep nodes (deep femoral and pelvic) were involved in only approximately 3% of cases when the superficial lymph node findings were negative. He later adopted a technique of using the deep inguinal nodes to predict the need for pelvic lymph node dissection but continued to remove both the superficial and deep inguinal nodes. A survey of a group of gynecologic oncologists found that fewer than 25% of respondents still perform deep inguinal node dissection.²⁶

Intraoperative Details

Surgery for vulvar carcinoma is often performed with the woman's legs in adjustable stirrups to facilitate both the groin node dissection and the perineal phase of the operation. A surgical team can greatly reduce operative time. After the patient is prepared and draped, make an incision approximately 2 cm below the inguinal ligament. The tissue is undermined below the Scarpa fascia. Carry the dissection down to the tensor fasciae latae. Then, dissect the superficial inguinal nodal group off the cribriform fascia, taking care to not injure the great saphenous vein.

If the deep nodes are to be dissected, open the cribriform fascia laterally and take it as part of the specimen. Then, dissect the femoral vein free and remove the nodes from the medial portion of the femoral vein. After a deep groin node dissection, the sartorius muscle can be divided at its insertion on the anterior iliac spine and sutured to the inguinal ligament to cover the femoral vessels. Bring closed suction drains in through a separate incision and suture to the skin. Close Scarpa fascia with 3-0 absorbable sutures, and close the skin with mattress sutures or with staples.

For the vulvectomy, outline the lesion and make an incision to encompass 1-cm margins around the tumor. In contrast to a simple vulvectomy, the dissection is carried deep to the perineal membrane. Care must be taken at the posterior aspects of the incision where the pudendal vessels enter the vulva. The lower portion of the bulbocavernosus muscle should be clamped and ligated to prevent bleeding. Once the lesion is removed, the vagina and vulvar skin can be mobilized to reduce the tension on the incision. It is closed in layers with absorbable suture, and the skin is closed with horizontal or vertical mattress sutures. The authors' preference is to use 2-0 polyglycolic acid for the mattress sutures and to reinforce the incision with a running 3-0 polyglycolic acid suture.

Postoperative Details

After surgery, recommend frequent sitz baths. Patients should dry the vulva completely after each sitz bath. A Foley catheter may be needed for a prolonged period after surgery around the urethra. Low molecular weight heparin or pneumatic compression stockings should be used in all women to prevent postoperative venous thrombosis. A Jackson-Pratt or similar type of drain should be placed in the inguinal space at the time of lymphadenectomy. Leave this drain in place until drainage is approximately 25 mL or less per day. In many cases, this may take more than 2 weeks.

Follow-up

Although surgery for vulvar carcinoma is often curative, it can be disfiguring and may significantly impact sexual function. This type of surgery can have serious psychological sequela, even in the absence of a functional problem after surgery. Sexual dysfunction seems to be related to a disturbance in body image, leading to hypoactive sexual disorder and aversion disorder. Depression and increasing age are risk factors for sexually active women to discontinue intercourse after surgery. Interestingly, few studies have been able to correlate sexual dysfunction with extent of surgery if the clitoris was preserved.

Women who have positive findings from more than one node are likely to benefit from adjuvant radiation therapy to the inguinal and pelvic nodes. The GOG studied the use of pelvic node dissection instead of pelvic and groin radiation and found that radiation was superior. A clear benefit for radiation has not been proven in women with positive microscopic findings from one node, but, because groin recurrence is almost universally fatal, adjuvant radiation is often recommended.

Monitor patients closely after treatment for vulvar carcinoma. Examinations every 3 months for the first 2 years are often recommended because more than two thirds of recurrences are in this time period. Detection of local recurrence of vulvar carcinoma is important because it can be treated by radical surgical excision.

The long-term survival rate after radical excision of a vulvar recurrence has been reported as 50-60%. Survival is better in women who originally presented with early-stage disease. Other factors that diminish the cure rate after local recurrence include disease at sites other than the vulva and a short interval from initial treatment to recurrence. For a large recurrence, an exenterative procedure can be attempted. A long-term survival rate of 38% has been reported after exenterative surgery for vulvar carcinoma.

Resection of a groin recurrence is not usually recommended. Often, this area heals slowly if radiation has already been used. Generally, the procedure should not be considered curative. The only situation in which resection of a groin node recurrence should be attempted is if the groin node is an isolated recurrence and the patient has not been previously irradiated.

Positive groin nodes at the time of initial surgery increase the risk of recurrence in the groin in the first 2 years. After the first 2 years, the risk of groin recurrence is low, regardless of the status of the nodes at the time of the initial surgery. It has been noted that the risk of local failure on the vulva is elevated for many years after the surgery. A report from the Mayo clinic showed that up to 10% of patients treated for vulvar cancer had a local recurrence more than 5 years after the original diagnosis.

For excellent patient education resources, visit eMedicine's Cancer and Tumors Center and Procedures Center. Also, see eMedicine's patient education articles Colposcopy and Cervical Cancer.

Complications

Lymphocyst formation is noted in 7-19% of patients after groin lymphadenectomy. Although cellulitis after vulvectomy has been associated with an increase in the incidence of lymphedema, it has not been associated with an increase in lymphocyst formation. Do not remove drains after inguinal node dissection until the daily output of the drain is less than 25 mL.

Lymphocysts usually manifest as an asymptomatic mass in the groin. To exclude a groin recurrence, aspirate fluid from the cyst and send for cytologic evaluation. Multiple aspirations are often required and may not be curative. If the mass is symptomatic, the lymphocyst can be removed surgically. However, in one small series, lymphocysts were successfully treated by placing a drain in the groin until the output was less than 25 mL/d. The drain was then removed and a pressure dressing was placed to prevent reaccumulation of the fluid. Sclerosis of lymphocysts with Betadine solution has also been described.

Cellulitis and lymphangitis can occur after groin node dissection. The incidence rate of cellulitis requiring antibiotics ranges from 20-40%. Often, patients who develop lymphocysts are at increased risk of lymphangitis. The etiologic agent is most often a streptococcal species, and treatment with penicillin is adequate. If drains are still in place, first-generation cephalosporins may be more appropriate to treat Staphylococcus aureus.

Chronic lymphedema has been reported in 10-20% of women after groin node dissection. This can be a disabling problem and is more common if radiation is required after groin dissection. Limiting groin node dissection in women with early cancers and preserving the saphenous vein decreases the incidence of this problem. The use of graduated compression stockings after lymphadenectomy can help prevent lymphedema. If edema does develop, the use of compression stockings, massage therapy, and limb wraps can help control the accumulation of fluid. However, lymphedema can be chronic and disabling in severe cases. Many major centers offer lymphedema treatment programs.

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