Vulvar cancer grows slowly and tends to remain localized for a long period of time, evolving into invasive cancer within an 8-year period [9
]. In our study, almost 90% were diagnosed with early stage (in situ
or FIGO stage I/II) disease. Our data also indicated that older patients were more likely to present with more advanced disease, supporting previous reports of clinical and behavioral barriers that lead to delays in diagnosis [6
]. According to Canavan and Cohen [6
], VNED-related vulvar cancer among women aged 55 and older are less likely to present with cervical neoplasia and a history of condyloma. These characteristics are typically associated with pre-invasive disease [16
] and are a component of screening during routine gynecologic examinations, which older women are less likely to undergo. Additionally, older women are less likely to conduct home self-examinations of the vulva and often fail to seek treatment for VNED symptoms for as long as 16 months [6
]. Physicians also have been reported to contribute to the delay in diagnosis by providing medical treatment for up to 12 months before obtaining a biopsy or consider referral [6
]. It is important, therefore, that women understand the need for continued gynecologic examinations and the importance of timely evaluations of vulvar lesions.
As expected, women with advanced (FIGO Stage III–IV) disease in our study were more likely to undergo CT scans to evaluate for local or metastatic spread, following recommended clinical practice guidelines [8
]. However, the use of radical or total vulvectomy did not differ substantially for women with invasive vulvar cancers, whether stage I/II or stage IV. This may be due, in part, to the individualized approach to treatment as described in the clinical practice guidelines [8
] and other sources [15
] or perhaps to the desire to avoid chemotherapy and/or radiation.
Primary chemoradiation has been shown to shrink vulvar cancers dramatically, thus permitting less extensive surgery [17
] and for women with advanced illness (i.e., close/positive margins or metastatic disease in the lymph nodes), adjuvant chemoradiation is recommended. In our study, however, only about 13% of the patients diagnosed with FIGO Stages III or IV in 1999 received chemoradiation for their advanced stage disease while an additional 8–10% received non-concurrent chemotherapy plus radiation. While some studies have shown that oncologists are considered to be early adopters of new therapies when the evidence is clear [20
], providers may have been hesitant to use less aggressive surgery without additional supporting evidence. Additionally, there may have been insufficient time between publication of the GOG clinical announcement in 1998 [17
] and the current study to reflect changes in treatment practices. The Gynecologic Oncology Group is currently investigating the use of preoperative radiation and chemotherapy for advanced vulvar cancer [22
]. However, it is interesting that even in 2004 one-third of patients with stage I/II vulvar cancer still receive total or radical vulvectomy [1
Surgical recommendations are typically determined by FIGO stage at diagnosis and tumor size [23
]. In situ
(FIGO Stage 0) and IA (≤ 2 cm) may be treated by superficial partial vulvectomy, IB (stromal invasion >1.0) and II (> 2 cm) may be treated with partial vulvectomy and lymphadenectomy, and III (any size with adjacent spread) and IVA (tumor invasion to other organs) may be treated with radical (or en bloc
) vulvectomy and lymphadenetomy. In a study conducted at the University of Mainz, Germany [24
], more than half of the patients treated from 1973–2002 had undergone a radical vulvectomy, a third received hemivulvectomy, and the remaining 12% received exenteration and/or colpectomy. More extensive surgery often results in long-term complications, which may include chronic leg edema, dyspareunia, femoral hernia, genetal prolapse, and urinary stress incontinence for six or more weeks after surgery [6
]. Quality of life is also affected by the surgical treatment of vulvar cancer as women report worsening body image and psychosexual disturbances including a decrease in sexual frequency [25
]. Among women with stage I and II disease in our study, we noted widespread use of radical local excision, which is less disfiguring than radical vulvectomy. Nonetheless, 47% of women with FIGO I/II disease underwent radical or total vulvectomy; while 45% of FIGO III and 54% of FIGO IV underwent a radical or total vulvectomy.
Sentinel lymph node assessment remains experimental among women with vulvar cancer. In our study, we observed that lymph node evaluation was almost always performed with inguinal lymphadenectomy. As data matures from the ongoing studies of sentinel node assessment in vulvar cancer conducted by the Gynecologic Oncology Group and the European Organization for Research and Treatment of Cancer, it is possible that sentinel lymph node assessment may become more common.
We do not have data on the training or board certification of the physicians treating these patients, so we cannot determine how many of these women were treated by gynecologic oncologists. Nonetheless, our data suggest that the majority of patients with vulvar cancer, especially those with late stage disease, were treated at generally large, teaching hospitals with at least one approved residency training program.
Our analysis of mortality from cancer, although limited, mirrors that of previous studies that reported higher risks of death associated with older age and higher stage [19
]. However, after adjusting for stage and age, race/ethnicity was not associated with cancer mortality.
This study has several limitations. We do not have data on whether the entire course of therapy was received, the side-effects of treatment, and quality of life measurements. In addition, we do not have data on the training or board certification of the treating physicians, which might influence treatment. However, the database does have major strengths. It is a population-based dataset that captures characteristics of treatment facilities, therapy provided in the community, patient comorbidity, and interventions confirmed by the treating physicians.
This “snapshot” of vulvar cancer in 1999 demonstrates that it is a disease most often diagnosed at early stages. Radical surgery was still commonly performed for all stages of invasive vulvar cancer, and the use of radiation was more common in women with FIGO III and IV. Although clinical trials data support the use of primary chemoradiation for patients with advanced disease, its use remained limited in the treatment of vulvar cancer among the general population in 1999. Vulvar cancer in most cases is local or loco-regional at the time of diagnosis. Definitive therapy is associated with high cure rates. We must make every effort, therefore, to ensure the treatment will produce the best possible chance at a cure as well as the least impact upon health related quality of life. Future research may collect similar data for 2007 diagnoses to assess more contemporary practice patterns in the United States.
We need to ensure that women with vulvar cancer are referred to specialists with appropriate training. Women must have access to a multidisciplinary treatment team encompassing expertise in gynecologic oncology, radiation oncology, oncology nursing, and psycho-social rehabilitation. In addition, we need to ensure that primary surgery is tailored to the size and location of the lesion, sparing the morbidity and disfigurement of the traditional radical vulvectomy. We need to encourage the use of primary chemoradiation for larger primary tumors, as this approach will permit more limited surgical resection and preservation of function.
The relative rarity of vulva cancer probably precludes phase III trials. We need to encourage participation in available national and international trials aimed at improving cure, local control, and health related quality of life.