Treatment Protocols
Primary vulvar cancer is rare; American Cancer Society estimates for 2017 are for 6020 new cases and 1150 deaths.
Vulvar cancers consist largely of squamous cell carcinomas. Other histologies, such as melanoma, adenocarcinoma, sarcoma, and basal cell carcinoma, are less common. The treatment of vulvar cancer in this guideline focuses on squamous cell histology and does not include the treatment of preinvasive disease.
Early-stage vulvar cancer (stage I-II)
Treatment recommendations are as follows:
Early-stage vulvar cancer is primarily treated surgically with radical vulvectomy plus inguinofemoral lymphadenectomy; margins should be ≥1 cm
Reexcision may be considered if margins are positive or < 8 mm
; alternatively, adjuvant radiation may be used rather than reexcision, especially if a repeat procedure would result in excessive morbidity
Inguinofemoral lymphadenectomy is performed based on the risk factors of the primary tumor (see Table 1, below); if the surgeon has adequate experience with sentinel lymph node dissection, this technique may be substituted for complete inguinofemoral lymphadenectomy
Adjuvant radiation is based on pathologic risk factors (see below)
Stage II with extension to the distal third of the urethra or distal third of the vagina or with anal involvement can be treated with radical local excision with bilateral inguinofemoral lymphadenectomy; radiation to these regions can also be considered
Table 1. Summary of Indications for Inguinofemoral Lymphadenectomy
(Open Table in a new window)
|
Inguinofemoral lymphadenectomy |
Tumor size (cm) |
Stromal invasion (mm) |
|
No lymph node dissection required |
≤2 cm |
≤1 (without lymphovascular space involvement) |
|
Ipsilateral |
≤2 cm |
≤1 (plus lymphovascular space involvement) |
|
≤2 cm |
>1 mm |
|
|
>2 cm |
Any |
|
|
Bilateral |
Same as ipsilateral and – Midline tumor (< 1 cm) or – Involves anterior labia minora or – Positive ipsilateral lymph node (if lesion ≥2 cm and depth ≥5 mm) |
|
Indications for adjuvant treatment of metastatic groin lymph nodes include the following
:
One lymph node micrometastasis (<5 mm): No adjuvant radiotherapy
Any lymph node macrometastasis ≥5 mm: Adjuvant radiotherapy
Two or more lymph node micrometastases (<5 mm): Adjuvant radiotherapy, including inguinal and pelvic fields
Any extracapsular spread: Adjuvant radiotherapy, 45 Gy in 25 fractions
Oonk et al examined the size of the metastatic disease in sentinel lymph nodes and found that micrometastasis >2 mm carried a risk of recurrence; the investigators suggested that adjuvant treatment or completion inguinofemoral lymph node dissection be performed for such sentinel lymph nodes.
A National Cancer Data Base (NCDB) analysis of 1797 patients with vulvar cancer who underwent extirpative surgery with confirmed inguinal nodal involvement treated with adjuvant radiotherapy concluded that the addition of adjuvant chemotherapy resulted in a 38% reduction in mortality risk. Unadjusted median survival with and without adjuvant chemotherapy was 44.0 versus 29.7 months, respectively (P=0.001).
Locally advanced vulvar cancer (bulky stage III and stage IV)
Treatment recommendations are as follows:
There is no significant difference in overall survival rates or in treatment-related adverse events when chemoradiation (primary or neoadjuvant) is compared with primary surgery
Radical surgery (radical vulvectomy plus bilateral lymphadenectomy): If partial removal of other involved structures is needed (eg, urethra, vagina, anus, bladder, rectum) and/or pelvic exenteration is necessary, consider preoperative chemoradiation
Chemoradiation (with or without subsequent completion surgery): This approach has been shown to decrease the need for exenterative surgery
; a wide range of regimens have been discussed in the literature, but there is no clear standard of care
Radiation: Total of 47.6-57.6 Gy divided into 28 fractions
Concurrent chemotherapy: Most cancer centers have extrapolated from the cervical cancer literature and use weekly cisplatin as a chemosensitizer
; a phase II study documented the safety and efficacy of weekly cisplatin (40 mg/m2 IV, not to exceed 70 mg/dose)
; previously studied regimens have included cisplatin plus 5FU
and 5FU plus mitomycin C,
but these are not as commonly used
Metastatic vulvar cancer (stage IVB)
There are no standard treatment guidelines for metastatic vulvar cancer; however, the following should be noted:
Chemotherapy regimens for metastatic vulvar cancer are similar to those used for metastatic cervical cancer
Combinations of chemotherapy and radiation can be considered
Chemotherapy options: Cisplatin is an active single agent in vulvar cancer, and cisplatin-based combinations have been reported to yield higher response rates
; erlotinib has also demonstrated some anecdotal responses but is not a standard of care
Recurrent vulvar cancer
Recurrent vulvar cancer can be grouped into local (regional), groin, and distant categories, and the following should be noted:
Local recurrence carries a good prognosis and can be treated with resection or radiation
Recurrences in the groin carry a poor prognosis; a select few of these patients may benefit from surgical resection and radiation
Distant recurrence is treated with chemotherapy; paclitaxel has been used as a single agent