Laboratory Studies
Women with a small vulvar cancer and nonpalpable inguinal lymph nodes require little preoperative testing, except what is indicated for preoperative clearance.
Imaging Studies
Imaging studies are useful to rule out metastatic disease for patients with large vulvar lesions and/or palpable inguinal lymph nodes.
A CT scan may be useful to help evaluate nodal spread in the pelvis in women with evidence of groin node metastasis, but the sensitivity of a CT scan to help detect pelvic lymphadenopathy is approximately 30%. Positron emission tomography (PET) scanning is the most accurate modality to detect pelvic or para-aortic adenopathy in patients with vulvar cancer. [1]
Other Tests
Perform an ECG prior to surgery, if indicated.
Pulmonary function tests may be appropriate in women who smoke and are older than 50 years to help in perioperative management. Evaluation should also include an arterial blood gas analysis.
Diagnostic Procedures
Colposcopy can be performed on the vulva but is more difficult than colposcopy of the cervix because of the large surface area of the vulva and the variability in premalignant lesions. Because of the keratinized skin, acetic acid should be placed for at least 5 minutes prior to colposcopy. To facilitate biopsy, EMLA (ie, eutectic mixture of local anesthetics) cream may be applied to ameliorate the pain from lidocaine injection. A punch biopsy tool can be used to take a representative sample of the vulva. A biopsy should be performed on all lesions to ensure that a cancer is not missed when multiple dysplastic lesions are present.
Histologic Findings
Squamous carcinoma is the most common pathologic type of vulvar carcinoma. Various grading systems are described and may be prognostic. Other prognostic features include confluent growth patterns and lymph vascular involvement.
Melanoma accounts for approximately 10% of vulvar cancers. The staging and treatment is similar to other melanomas. Clark defined a classification system that describes prognosis based on invasion of melanoma to certain tissue levels. [13] This system has been modified by Chung for use in vulvar melanoma. [14] Similarly, the depth of invasion, as described by Breslow, can be used to predict prognosis. [15]
Sarcoma is relatively uncommon. Subtypes include leiomyosarcoma, malignant fibrous histiocytoma, and epithelioid sarcoma. In addition, a sarcoma can arise from any structure in the vulva; including blood vessels, skeletal muscle, and fat.
Basal cell carcinoma of the vulva is uncommon, but it can occur in elderly women. As with other basal cell carcinomas, local excision is usually curative.
Verrucous carcinoma resembles condylomata acuminata and is also called a Buschke-Lowenstein giant condyloma. This type of carcinoma is locally aggressive but does not have a propensity to spread via lymphatics. These tumors are thought to be associated with HPV type 6.
Adenocarcinoma may arise in the Bartholin gland, and it represents approximately 40% of tumor types from this location. This type of tumor may attain considerable size before detection. Removal of the Bartholin gland to exclude an underlying carcinoma is indicated for recurrent Bartholin gland abscesses or cysts or if asymptomatic enlargement occurs in persons older than 50 years.
Paget's disease usually manifests as a red, raised, pruritic lesion. Histologically, the lesion is noted to contain cells with prominent nuclei and an increased amount of cytoplasm. Paget's disease has been associated with underlying adenocarcinoma of the colon or sweat glands in 15% of cases. Although Paget's disease does not metastasize, because the histologic changes often extend past the gross extent on the skin, it is known to have a high rate of local recurrence. For this reason, a clinical margin of 2 cm is recommended at the time of excision.
Other carcinomas of the vulva are rare. Tumors can occur in the apocrine sweat glands, and breast carcinoma can also develop from ectopic breast tissue contained within the milk line that extends down into the vulva.
Staging
The International Federation of Gynecology and Obstetrics (FIGO) revised staging for carcinoma of the vulva in 2021. [16] The FIGO committee used a new approach by analysis of prospective collected data.
Table 1. Staging of vulvar cancer by FIGO 2021 (Open Table in a new window)
TNM Categories |
FIGO |
Definition |
|
|
Primary tumor cannot be assessed |
|
|
No evidence of primary tumor |
|
|
Carcinoma in situ (preinvasive carcinoma) |
|
IA |
Tumor size < 2 cm and stromal invasion ≤1 mm (a) |
|
IB |
Tumor size >2 cm or stromal invasion > 1 mm |
|
II |
Tumor of any size with extension to lower one-third of the urethra, lower one-third of the vagina, lower one-third of the anus with negative nodes
Stage III Tumor of any size with extension to upper part of adjacent perineal structures, or with any number of nonfixed, nonulcerated lymph node Stage IIIA Tumors of any size with disease extension to upper two-thirds of the urethra, upper two-thirds of the vagina, bladder mucosa, rectal mucosa, or regional (b) lymph node metastases < 5 mm Stage IIIB Regional (b) lymph node metastases > 5 mm Stage IIIC Regional (b) lymph node metastases with extracapsular spread |
|
IV |
Tumor of any size fixed to bone, or fixed, ulcerated lymph node metastases, or distant metastases IVA - Disease fixed to pelvic bone, or fixed or ulcerated regional (b) lymph node metastases IVB - Distance metastases |
(a) Depth of invasion is measured from the basement mambrane of the deepest, adjacent, dysplastic, tumor-free rete ridge (or nearest dysplastic rete peg) to the deepest point of invasion. (b) Regional refers to inguinal and femoral lymph nodes. Important notations and changes 1. Lymph node positivity should reflect the consensus utilized in cervical cancer staging, which is micrometastasis and macrometastasis. 2. Individual tumor cells (ITC) will not count toward lymph node metastasis. 3. This staging is to be used for all morphological types of vulvar cancer and not just the most common squamous cell carcinoma. The only exception to this is vulvar carcinoma. 4. Documentation of HPV status of the carcinoma of the vulva (HPV-associated or HPV-independent) is strongly recommended. This is assessed by p16 block-type immunoreactivity and/or positive molecular testing for HPV. |
||
Table 2. Tumor Regional Lymph Node Involvement and Metastasis Categories and FIGO Stages (Open Table in a new window)
Regional lymph nodes (N)* |
|||
TNM categories |
FIGO stages |
Definition |
|
NX |
|
Regional lymph nodes cannot be assessed |
|
N0 |
|
No regional lymph node metastasis |
|
N1 |
|
1or 2 regional lymph nodes with the following features: |
|
N1a |
IIIA |
1 lymph node metastasis each ≤5 mm |
|
N1b |
IIIA |
1 lymph node metastasis ≥5 mm |
|
N2 |
IIIB |
Regional lymph node metastasis with the following features: |
|
N2a |
IIIB |
≥3 lymph node metastases each < 5 mm |
|
N2b |
IIIB |
≥2 lymph node metastases ≥5 mm |
|
N2c |
IIIC |
Lymph node metastasis with extracapsular spread |
|
N3 |
IVA |
Fixed or ulcerated regional lymph node metastasis |
|
Distant metastasis (M) |
|||
TNM categories |
FIGO stages |
Definition |
|
M0 |
|
No distant metastasis |
|
M1 |
IVB |
Distant metastasis (including pelvic lymph node metastasis) |
|
Anatomic stage/prognostic groups |
|||
Stage 0 |
Tis |
N0 |
M0 |
Stage I |
T1 |
N0 |
M0 |
Stage IA |
T1a |
N0 |
M0 |
Stage IB |
T1b |
N0 |
M0 |
Stage II |
T2 |
N0 |
M0 |
Stage IIIA |
T1,T2 |
N1a, N1b |
M0 |
Stage IIIB |
T1, T2 |
N2a, N2b |
M0 |
Stage IIIC |
T1, T2 |
N2c |
M0 |
Stage IVA |
T1, T2 |
N3 |
M0 |
T3 |
Any N |
M0 |
|
Stage IVB |
Ant T |
Any N |
M1 |
* An effort should be made to describe the site and laterality of lymph node metastases. |
|||
-
Diagram of lymphatic drainage of a lateral lesion. Most lymphatics flow through the superficial inguinal nodes, deep inguinal nodes, and the node of Cloquet to the pelvic lymph node chains. Deep inguinal node findings are positive approximately 3% of the time when superficial inguinal node findings are negative. Lymphatic mapping studies indicate that 13% of cases demonstrate findings consistent with flow to the pelvis that does not involve the node of Cloquet. If the lesion is in the anterior labia minor, then contralateral flow is demonstrated in 67% of cases.
-
A large T2 carcinoma of the vulva crossing the midline and involving the clitoris. (Photograph courtesy of Tom Wilson)
-
Specimen after removal with at least 1 cm margins around the tumor. (Photograph courtesy of Tom Wilson)
-
The surgical defect after a radical vulvectomy specimen is removed. (Photograph courtesy of Tom Wilson)
-
The surgical defect is closed after a radical vulvectomy. (Photograph courtesy of Tom Wilson)
-
A large squamous cell carcinoma of the vulva. Note the small contralateral "kissing lesion" that can be seen with vulvar carcinomas. (Photograph courtesy of James B. Hall, MD)
-
A specimen from a traditional single-incision radical hysterectomy. Most radical vulvectomies are now performed through 3 incisions, with the groin nodes removed separately from the vulvectomy specimen. (Photograph courtesy of James B. Hall, MD)
-
Closure of a large single-incision radical vulvectomy. The complete wound breakdown rate from this procedure is often greater than 50%. (Photograph courtesy of James B. Hall, MD)
-
A specimen from a primary exenteration for a stage IVA vulvar cancer involving the rectum. Many of these large tumors are now treated with adjuvant chemotherapy and radiation prior to surgery, with preservation of rectal sphincter function. (Photograph courtesy of James B. Hall, MD)
Tables
TNM Categories |
FIGO |
Definition |
|
|
Primary tumor cannot be assessed |
|
|
No evidence of primary tumor |
|
|
Carcinoma in situ (preinvasive carcinoma) |
|
IA |
Tumor size < 2 cm and stromal invasion ≤1 mm (a) |
|
IB |
Tumor size >2 cm or stromal invasion > 1 mm |
|
II |
Tumor of any size with extension to lower one-third of the urethra, lower one-third of the vagina, lower one-third of the anus with negative nodes
Stage III Tumor of any size with extension to upper part of adjacent perineal structures, or with any number of nonfixed, nonulcerated lymph node Stage IIIA Tumors of any size with disease extension to upper two-thirds of the urethra, upper two-thirds of the vagina, bladder mucosa, rectal mucosa, or regional (b) lymph node metastases < 5 mm Stage IIIB Regional (b) lymph node metastases > 5 mm Stage IIIC Regional (b) lymph node metastases with extracapsular spread |
|
IV |
Tumor of any size fixed to bone, or fixed, ulcerated lymph node metastases, or distant metastases IVA - Disease fixed to pelvic bone, or fixed or ulcerated regional (b) lymph node metastases IVB - Distance metastases |
(a) Depth of invasion is measured from the basement mambrane of the deepest, adjacent, dysplastic, tumor-free rete ridge (or nearest dysplastic rete peg) to the deepest point of invasion. (b) Regional refers to inguinal and femoral lymph nodes. Important notations and changes 1. Lymph node positivity should reflect the consensus utilized in cervical cancer staging, which is micrometastasis and macrometastasis. 2. Individual tumor cells (ITC) will not count toward lymph node metastasis. 3. This staging is to be used for all morphological types of vulvar cancer and not just the most common squamous cell carcinoma. The only exception to this is vulvar carcinoma. 4. Documentation of HPV status of the carcinoma of the vulva (HPV-associated or HPV-independent) is strongly recommended. This is assessed by p16 block-type immunoreactivity and/or positive molecular testing for HPV. |
||
Regional lymph nodes (N)* |
|||
TNM categories |
FIGO stages |
Definition |
|
NX |
|
Regional lymph nodes cannot be assessed |
|
N0 |
|
No regional lymph node metastasis |
|
N1 |
|
1or 2 regional lymph nodes with the following features: |
|
N1a |
IIIA |
1 lymph node metastasis each ≤5 mm |
|
N1b |
IIIA |
1 lymph node metastasis ≥5 mm |
|
N2 |
IIIB |
Regional lymph node metastasis with the following features: |
|
N2a |
IIIB |
≥3 lymph node metastases each < 5 mm |
|
N2b |
IIIB |
≥2 lymph node metastases ≥5 mm |
|
N2c |
IIIC |
Lymph node metastasis with extracapsular spread |
|
N3 |
IVA |
Fixed or ulcerated regional lymph node metastasis |
|
Distant metastasis (M) |
|||
TNM categories |
FIGO stages |
Definition |
|
M0 |
|
No distant metastasis |
|
M1 |
IVB |
Distant metastasis (including pelvic lymph node metastasis) |
|
Anatomic stage/prognostic groups |
|||
Stage 0 |
Tis |
N0 |
M0 |
Stage I |
T1 |
N0 |
M0 |
Stage IA |
T1a |
N0 |
M0 |
Stage IB |
T1b |
N0 |
M0 |
Stage II |
T2 |
N0 |
M0 |
Stage IIIA |
T1,T2 |
N1a, N1b |
M0 |
Stage IIIB |
T1, T2 |
N2a, N2b |
M0 |
Stage IIIC |
T1, T2 |
N2c |
M0 |
Stage IVA |
T1, T2 |
N3 |
M0 |
T3 |
Any N |
M0 |
|
Stage IVB |
Ant T |
Any N |
M1 |
* An effort should be made to describe the site and laterality of lymph node metastases. |
|||
