Vulvar Cancer   外陰癌  

 

顧名思義Vulvar Carcinoma是指由會陰部長出來的cancer,部分vulvarcancer事實上的原發部位是子宮頸,會陰部份的病灶是轉移而來的,所以診斷vulvar cancer必須排除cervical cancer

Vulvar Ca最會發生於labia majora,其次是labia minora

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 VulvectomyCare:

1. 術後前3天除藥物外須禁食(術前須colon prepare以利術後延遲解便時間)

2. 使用paregonic tincture使病人在前5天不排便,不免污染傷口,減少infection機會。

3. 6天起給軟便劑,以防病用力排便傷口裂開。

4. 術後病人的腳不要張得太開,以減少傷口縫合的張力。

5. 術後一週內病人不穿褲子,而以"罩子"(打燈籠)外面蓋被子蓋住下半身。

6. 每日記錄每一個Vaccum ball的日排出量,小於5cc才能拔除。

7. 術後立即給two combined antibiotics,一般是選用一種broad spectrum加上一種aminoglycoside (eg cefamezine + GM)

8. Vaccum ball流出液體若有異味或異色則立即採集液體作嗜氧及厭氧之細菌培養,並依細菌培養報告之藥物敏感性測驗結果更改消炎藥物之選用。

9. 每日檢查傷口看植皮或皮瓣部分有無壞死,若有紅腫等發炎徵兆,則以生理食鹽水紗布作wet dressing, q4hr

10. 若傷口癒合良好,第8天起可以拆線鼠蹊部淋巴腺分離切法傷口部份,至於外陰的傷口視療合情況由主治醫師決定。

11. 4天須查病理報告,以便規劃後續治療。

 

* Adjuvant R/T: 以下情況術後須追加放射治療。

1. Pelvic lymph node有轉移。

2. Groin lymph node有轉移(包括cloquet's node)

3. 會陰部之surgical margin仍有cancerprecancer lesion

4. 會陰部的病灶已深及骨膜(periostum)而無法全部拿乾淨。

5. 淺部之轉移淋巴腺≧2個以上。

 

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

8. 早期會陰癌的臨床表現都只有搔癢及破皮或潰瘍,所以,如果有會陰部潰瘍或破皮經治療後(一個月後)仍然不會好,一定要考慮會陰癌,並安排切片檢查。 

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 (cloquets 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 invasion1.0 mm

Stage 1B: stromal invasion1.0 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  

Any distant metastasis, including pelvic lymph nodes                            

 

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 1 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 3 groin lymph nodes including Cloquets 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