Chi_Principles and Practice of Gynecologic Oncology 8e

20 SECTION 2 ■ Vulvar Cancer

of plastic closures is useful (discussed later). Consultation with a plastic surgeon in such cases can be invaluable. Overall survival (OS) for women following an adequate resec tion of a primary squamous carcinoma limited to the vulva and with uninvolved inguinal LNs is greater than 90%. Patients with stage II who have negative margins and uninvolved nodes but in volvement of the lower vagina or urethra should obtain similar results. Locally Advanced Tumors Locally advanced disease is defined as such by either disease ex tent (eg, T3-T4, or LNs fixed to vessel/muscle) or location (eg, T1 tumor abutting anal verge or urethra), making upfront surgi cal resection not feasible. Many locally advanced vulvar tumors are bulky; however, some are of modest size but are considered high risk because of proximity to critical midline structures. Some primary tumors can be curatively excised by radical vulvectomy. Surgical resection of 1 to 1.5 cm of the distal urethra to achieve a negative surgical margin does not appear to compromise blad der continence (10). Although radical surgery is an option for pa tients with locally advanced tumors, contemporary therapeutic strategies have centered on sequenced radiation therapy (RT) or chemoradiation (CRT) followed by radical surgery as a means to preserve either urinary or fecal continence or both. Vulvar can cers are sufficiently sensitive to therapeutic radiation such that function-sparing operations are feasible in selected patients with advanced disease who receive combined modality treatment (11). For patients with stage IVA tumors, similar experiences have been reported; ultraradical (exenterative) resections may also be con sidered for select patients. Invasive Vulvar Squamous Cell Carcinoma: Management of Lymph Nodes Clinically Node-Negative Patients In the pre-SLNB era, it was determined from Gynecologic On cology Group (GOG) 37 that patients who undergo bilateral in guinofemoral lymphadenectomy as initial therapy and are found to have positive nodes—especially those with more than one pos itive node—benefit from postoperative irradiation to the bilateral groin and lower pelvis (12). RT in this situation is superior to pelvic node dissection. However, the morbidity of combining in guinofemoral lymphadenectomy with radiation can be substantial and includes chronic groin and extremity complications, primarily lymphedema (12). Nowadays, SLNB is standard of care for patients with early-stage clinically node-negative vulvar cancer (13). The ideal management of patients with a microscopically positive SLN who do not undergo inguinofemoral LN dissection was inves tigated in the Groningen International Study on Sentinel Nodes in Vulvar Cancer (GROINSS)-VII/GOG 270 trial (14). In this trial, women with a negative SLN were observed; women with SLN micrometastases ( ≤ 2 mm) received postoperative RT, and women with SLN macrometastases ( > 2 mm) were managed with inguinofemoral lymphadenectomy and RT. Concurrent ra diosensitizing cisplatin was optional. The GROINSS-VII study found that patients with a micrometastasis or ITCs could safely omit inguinofemoral LN dissection and undergo adjuvant RT alone. However, patients with macrometastases had suboptimal outcomes with adjuvant RT alone (20% groin recurrence rate), resulting in a protocol modification to require inguinofemoral lymphadenectomy in addition to RT in these patients. Therefore, at this time, patients with macrometastases on SLNB require completion inguinofemoral LN dissection, followed by adjuvant RT with consideration of concurrent chemotherapy in those with higher risk disease such as two or more involved nodes or extracapsular extension.

Clinically Node-Positive Patients Some women have LN metastases detected by preoperative phys ical examination or diagnostic imaging, or at the time of their primary surgery. An optimal management strategy for clinically apparent node-positive patients is yet to be defined. Surgical resec tion of bulky nodal disease improves regional control and proba bly enhances the curative potential of RT. In multivariate analysis, Hyde et al (15) found that, for patients with clinically positive groin nodes who underwent surgery followed by RT, the method of sur gical groin node dissection (nodal “debulking” vs full groin dis section) had no prognostic significance. On the other hand, some patients present with extensive, unresectable nodal disease. There is increasing early data suggesting a role for upfront chemoradia tion for patients with unresectable locally advanced disease (dis cussed later in Radiation section). Management of Distant Metastatic Disease Treatment for stage IVB vulvar cancer is generally palliative, al though a subset of these patients with pelvic LN metastases as their only site of distant metastases is potentially curable with aggressive locoregional treatment based on retrospective studies (16). Management of Recurrent Squamous Vulvar Cancers Vulvar cancer recurrences can be categorized into three clinical groups: local (vulva), groin, and distant. Historically, it was thought that most localized vulvar recurrences were salvageable, with one study showing recurrence-free survival (RFS) in up to 75% of cases when the recurrence is limited to the vulva and can be excised with a gross clinical margin (17). However, more recent data suggest lower salvage rates than previously thought (13,18). For instance, long-term follow-up of the GROINSSV study showed a 10-year vulva recurrence of 40%, and 10-year disease-specific survival decreased from 90% to 69% for patients without versus with a local recurrence (13). In addi tion, a recent subset analysis of the AGO-CARE-1 study showed that 30% of patients who developed isolated vulvar recurrence had a second recurrence (18). The observation that many of these recurrences arise at sites remote from the initial primary tumor or that they occur years after apparently successful primary treatment suggests that some re currences probably represent new primary tumors rather than the de velopment of new disease. Salvage therapy is individualized based on patient comorbidities and extent of disease but generally consists of multimodality management, with some combination of resection of bulky disease and local radiation, perhaps using intensity-modulated RT (IMRT), with or without systemic therapy. Recurrences in the groin, on the other hand, are almost universally fatal. Patients who develop distant metastases are candidates for palliative systemic cyto toxic or targeted therapy or transition to comfort care. Management of Nonsquamous Vulvar Cancers Malignant Melanoma The primary treatment modality for vulvar melanoma is surgical excision. Radical vulvectomy with bilateral inguinofemoral lymph adenectomy was the historical treatment of choice (19). However, because most failures are distant, radical local resection does not appear to enhance survival. Furthermore, many patients with vulvar melanoma are elderly, with coexisting medical problems, making less radical and morbid surgery compelling. More recent reviews recommend some form of hemivulvectomy or wide local excision along with inguinal lymphadenectomy or SLN mapping (20,21). DOI, mitotic activity, and the presence of ulceration are prognostically significant and should be considered in treatment planning. Based on information derived from large series of pa tients with cutaneous melanomas at nongenital sites, regional lymphadenectomy should probably be considered a prognostic

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