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Vulvar Ca³Ì·|µo¥Í©ólabia majora¡A¨ä¦¸¬Olabia minora¡C

Etiology and Risk Factors:

1. No definitive etiologic factor has been identified

2. Risk factors: multiple sexual partners, history of genital warts, smoking, HPV

3. Previous vulva intraepithelial neoplasia (CIS)

Classification of vulvar disease

Nonneoplastic epithelial disorders of skin and mucosa

Lichen sclerosus et atrophicus

Squamous hyperplasia, not otherwide specified (formerly _ hyperplastic dystrophy without atypia_)

Other dermatoses

Mixed nonneoplastic and neoplastic epithelial disorders

Intraepithelial neoplasia

  squamous intraepithelial neoplasia ( formerly _ dystrophies with atypia)

       VIN 1

       VIN 2

       VIN 3 (severe dysplasia or carcinoma in situ)

  Nonsquamous intraepithelial neoplasia

       Paget's disease

       Tumors of melanocytes, noninvasive

Invasive tumors 

Invasive Vulvar Cancer: most often in menopausal female, meas age of diagnosis is 65 y/o 

* Post Radical VulvectomyªºCare:

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Clinical features:

1. Vulvar lump or mass

2. Vulva itching, pruritus (vulvar dystrophy)

3. Vulvar bleeding, discharge, dysuria

4. Groin mass (metastatic lesion)

5. Vulvar wart, fleshy, ulcerated, leukoplakic appearance

6. Site of occurrence: labia majora is most often, labia minora, clitoris, and then perineum

7. 5% of the cases are multifocal

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Diagnosis: pathology is required

1. Wedge biopsy: better include surrounding skin and underlying dermis to determine the invasion depth or stromal invasion, especially in early cancer

2. Excisional biopsy in lesion less than 1 cm in diameter

3. Pelvo-abdominal CT scan: to determine the lymph node status of the inguinal, external iliac and even common iliac and paraaortic area.

4. CXR

5. Tumor markers: only SCC-Ag in some squamous cell carcinoma

6. Fine needle aspiration cytology of suspicious inguinal LN.

Routes of spreading:

1. Direct extension: vagina, urethra, anus

2. Lymphatic spreading:

1) to the regional lymph nodes first, superficial ingunal lymph node ® deep inguinal lymph node (cloquet’s node) ® femoral nodes ® external iliac lymph node

2) The incidence of lymph node spreading is related to the tumor size, stage of disease, and depth of invasion.

3) Pelvic lymph is rare in the absence of groin node metastasis. About 20% patients of with positive groin nodes have positive pelvic nodes.

4) Clinical evaluatin of groin lymph node is inaccurate in about 25 - 30% of the cases. (microscopic metastasis, inflammatory but negative node)

5) The spreading of lymph node is usually limited to the ipsilateral group if the leison does not cross over midline.

3. Hematogenous spread to distant sites: lung, liver and bone.

Staging:

The entire vulva, perineal and inguinal area should be examined carefully and thoroughly to make a exact staging.

FIGO (1995) Staging of vulvar carcinoma 

STAGE                     Clinical Findings                                         

STAGE 0   Carcinoma in situ; intraepithelial carcinoma

STAGE I

Tumor confined to the vulva or perineum; 2 cm or less in greatest dimension; no nodal metastasis

Stage 1A: stromal invasion¡Ø1.0 mm

Stage 1B: stromal invasion¡Ö1.0 mm

STAGE II

Tumor confined to the vulva or perineum; more than 2 cm in greatest dimension; no nodal metastasis

STAGE III

Tumor of any size with adjacent spread to the urethra, vagina, or the anus, or with unilateral regional lymph node metastasis

STAGE IVA

tumor invades upper urethra, bladder mucosa, rectal mucosa, pelvic bone, or bilateral regional node metastases

STAGE IVB  

Treatment:

1. Prior to any surgery or treatment,  all patients should be surveryed thoroughly to rule out the possibility of being a metastatic cancer  or the existence of synchronous second primary cancer.

2. Stage I: No suspicious groin nodes.

1) Primary lesion: radical local excision with the surgical margin of 1 cm at least.

2) Groin lymph node: linear incision to save the skin bridge

If the primary lesion is not periclitorical or not cross midline, do the ipsilateral side node first. The contralateral groin node will be dissected when the froaen section of the ipsilateral node proven positive.

if the invasion depth of stroma is ¡Ù1 mm, groin lymph node dissection is not necessary.

All patients with > 1mm stromal invasion require inguinal-femoral lymph node lymphadenectomy.

For microscopic lymph node involvement: observation

For two or more positive lymph nodes: post-op radiation

3. Stage II:

1) Primary lesion: En Bloc  radical vulvectomy. Partial resection of vagina, urethra, anus is required if they are involved.

2) Groin lymph node:

For early metastatic groin nodules: separate linear incision wound to save the skin bridge is necessary.

For big node or advanced  lesion: butterfly incision to clean the cancer cell in the skin bridge.

3) Myocutaneous graft is better way to decrease the tension of wound and to facilitate the rehabilitatin.

4) Pelvic lymph node dissection: when ¡Ù3 groin lymph nodes including Cloquet’s node (+)

4. Advanced disease Stage III, IV:

1) Large T3 or a T4 primary tumor: Treatment is selected according to patient disease and general condition.

* Pelvic exenteration combined with radical vulvectomy and bilateral groin dissection.

* Palliative radiation: radiation alone is not easy to cure an advanced disease.

* Concurrent chemoradiation: add chemotherapeutic agent (eg cisplatin, 5-Fu) as radiosensitizer to potentiate the effect of radiation.

* Combined radiosurgery: Preoperative radiotherapy + vulvectomy; Modified radical vulvectomy + radiation to eradicate the microscopic lesion.

* Preoperative concurrent chemoradiation  + vulvectomy can be used for cases of locally advanced tumor to save pelvic exenteration.

2) Bulky positive groin nodes:

Full groin dissection combined with groin radiation : often produces severe leg edema.

Limited groin lymph node dissection followed by external groin irradiation in cases.

The positive pelvic lymph nodes should be removed by extraperitoneal approach.

Prognosis:

1. The 5 year survival rate of stage I disease is 90.4%, stage II: 77.1%, stage III: 51.3%, stage IV: 18%.

2. For pateints with negative lymph node: 90%

For patients with positive lymph node: 50%

Complication:

1. Following surgery

1) Early complications:

Wound infection, necrosis and wound breakdown. The incidence can be reduced by separate incision wound. (85% ® 44%)

Urinary infection, seroma in the femoral triangle, deep vein thrombosis, pulmonary embolism, myocardial infarction, hemorrhage and ostitis pubis.

2) Late complications:

leg edema, recurrent cellulitis, urinary stress incontinence, genital prolapse, vaginal introitus stenosis

2. Following radiation:

1) Radiation dermatitis

2) Leg edema

3) Poor healing  and the subsequent infection