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Vulva

1. Diagnosis

Histological Classification

  1. Squamous carcinoma in situ (VIN III)    
  2. Paget's disease    
  3. Invasive
    1. Squamous cell carcinoma
    2. Basal cell
    3. Malignant melanoma
    4. Adenocarcinoma (Bartholin's gland or Skene's gland)

2. Staging

​1) Classification Criteria (TNM Staging)

T Primary Tumour

TisPreinvasive carcinoma (carcinoma in situ)
T1 ​​Lesions 2 cm or less in size confined to vulva or perineum. No nodal metastasis
T1aLesions 2 cm or less in size confined to the vulva or perineum and with stromal invasion no greater than 1.0 mm.* No nodal metastasis
T1bLesions 2 cm or less in size confined to the vulva or perineum and with stromal invasion greater than 1.0 mm. No nodal metastasis.
** The depth of invasion is defined as the measurement of the tumor from the epithelial-stromal junction of the adjacent most superficial dermal papilla to the deepest point of invasion.
T2Tumour confined to the vulva and/or perineum - >2 cm in greatest dimension
T3Tumour of any size with adjacent spread to the urethra and/or vagina and/or to the anus
T4Tumour of any size infiltrating the bladder mucosa and/or the rectal mucosa, including the upper part of the urethral mucosa and/or fixed to the bone

N Regional Lymph Nodes

N0No nodes palpable
N1Unilateral regional lymph node metastasis
N2Bilateral regional lymph node metastases

M Distant Metastasis

M0No clinical metastasis
M1Distant metastasis (including pelvic lymph node metastasis)

Clinical Stage Grouping (FIGO)

Stage 0

TisCarcinoma in situ; intraepithelial carcinoma

Stage I

T1 N0 M0Tumour confined to the vulva and/or perineum - 2 cm or less in greatest dimension, nodes are not palpable

Stage II

T2 N0 M0Tumour confined to the vulva and/or perineum - more than 2 cm in greatest dimension, nodes are not palpable

Stage III

​T3 N0 M0​​Tumour of any size with
1) adjacent spread to the lower urethra and/or the vagina, or the anus, and/or
2) unilateral regional lymph node metastasis
T3 N1 M01) adjacent spread to the lower urethra and/or the vagina, or the
T1 N1 M0anus, and/or
T2 N1 M02) unilateral regional lymph node metastasis

Stage IVA

T1 N2 M0Tumour invades any of the following:
Upper urethra, bladder mucosa, rectal mucosa, pelvic bone, and/or bilateral regional node metastasis.
T2 N2 M0Upper urethra, bladder mucosa, rectal mucosa, pelvic bone,
T3 N2 M0and/or bilateral regional node metastasis.
T4 Any N M0

Stage IVB

Any TAny distant metastasis including pelvic lymph nodes
Any N, M1

2) Staging Diagr​am

3. Management

​Vulvar Intraepithelial Neoplasia - VIN

(Squamous carcinoma in situ)

Laser surgery is the preferred method of treatment, in young patients and patients with multifocal lesions. Surgery in the form of wide local excision or occasionally simple vulvectomy may also be used for certain patients with intraepithelial neoplasia.

Wide local excision or simple vulvectomy is the usual treatment for basal cell carcinoma or Paget's disease. The use of topical fluorouracil (5-FU) is not recommended.

Invasive Carcinoma

Radical vulvectomy and en bloc regional lympadenectomy is the treatment method of choice for most types of invasive disease.

Modification of this standard treatment policy may be made on the basis of the patient's age, lesion size, depth of invasion, lesion location, and general medical condition. Radiation therapy may be used as primary treatment in inoperable cases and for recurrent disease following surgery, either locally or in nodal areas. In addition, patients with an operable lesion but medically unfit for radical surgery may be treated by primary radiotherapy. A variety of radiotherapeutic methods and techniques may be used.

Certain lesions involving the posterior vulva (fourchette), perineal body or anal margin may be treated with a combined approach using radiation and surgery.

Melanomas

Melanomas are usually treated surgically.

Extent of surgery is individualized based on lesion size and depth of invasion. (Node dissection is no longer part of the routine treatment of these lesions and is only done when suspicious or enlarged nodes are present.)

Chemotherapy Protocols

Estrogen Replacement Therapy - Site Specific Information

No data exist to show a contraindication for hormone replacement in cervical, vaginal or vulvar cancer. Many of these patients are young and at high risk for estrogen deprivation related morbidity. It is the feeling of the group that these patients should be encouraged to take estrogen replacement. In the presence of a uterus we recommend continuous estrogen and progestogen with the estrogen at a moderate dose (0.625-1.25 mg Premarin) and the progestogen at a low dose (2.5 mg Provera). Histologic studies have shown that continuous therapy with combined estrogen and progestogen induces an atrophic bland endometrium. This regime may result in some initial bleeding for the first one to two months but in general the women are amenorrheic.

An alternative regime would be to use cyclical estrogen and progestogen. This has the disadvantage of inducing a withdrawal bleed in some patients who have not had total ablation of the endometrium. Hematometra may develop if there is cervical stenosis present leading to abdominal pain or a mass.

Recommendation: Continuous estrogen and progestogen, e.g.:
Premarin; 0.625-1.25mg
Provera 2.5 mg
(note: these small doses of Provera do not seem to have the same deleterious effects on serum lipids as the higher doses.)

In the absence of a uterus there is no theoretical need for progestogen, therefore estrogen alone in usual doses is recommended.

Radiotherapy - General Information

Site Specific Information

Treatment is individualized. An external beam radiotherapy course involves six to seven weeks of treatment followed possibly by one to two weeks of additional nursing care.

4. Follow-up

​Patients should be reminded that it is their responsibility to keep their recommended follow-up appointments. The objectives of the follow-up visits are as follows: 

  1. To determine the patient's immediate response to the treatment employed    
  2. Early recognition and prompt management of treatment related complications    
  3. Early detection of persistent or recurrent disease    
  4. Collection of meaningful data regarding the efficacy of existing treatment policies and their complications so that any appropriate modifications can be instituted

These objectives are best met by having the initial follow-up examination performed by the Agency medical staff. When appropriate, arrangements will be made for follow-up by the referring physician.

Post Surgical

Year 1every 4 months
Years2-5every 6 months
Years 5+annually

Post​​-Radiotherapy

Year 1first visit at 1 month, then every 2 months
Years2-3every 6 months
Years 3+annually

Note: follow-up consists of general exam, pelvic exam and annual Pap smear screening.

SOURCE: Vulva ( )
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