Carcinoma of the Vulva Incidence of malignant diseases the vulva: Incidence of malignant diseases the vulva



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Carcinoma of the Vulva


Incidence of malignant diseases the vulva:

  • Incidence of malignant diseases the vulva:

  • 3 - 4% of all gynecologic malignancies.

  • - The incidence increases with age.

  • - Recently there was a rise in the incidence, due to

      • Longevity
      • Increased HPV infections.
      • Increased smoking habits
  • Risk factors for carcinoma of the vulva:

  • 1- Human papillomavirus infection.

    • Genital condylomas: these are detected in 5 % of vulvar cancer.
    • Vulvar intraepithelial neoplasia (VIN) and also CIN.
  • 2- Medical history of:

    • Vulvar dystrophy.
    • Chronic vulvar pruritus.
  • 3- Patients with a history of squamous cell carcinoma of the cervix or vagina.

  • 4- Chronic immunosuppression.

  • 5- Smoking



2 Types / Variants







Vulvar Cancer Stages FIGO System

  • Stage 0 - Carcinoma in situ, VIN 3, severe vulvar dysplasia.

  • Stage I - Tumor 2 cm or less, and confined to the vulva or perineum

    • IA - Less than 1 mm invasion below the surface layer
    • IB - More than 1 mm invasion below the surface layer
  • Stage II - Cancer is confined to the vulva and/or perineum, and larger than 2 cm.

  • Stage III - Cancer has spread to

    • the lower urethra or vagina or anus
    • and / or local lymph nodes on one side.
  • Stage IV

    • A - Cancer has spread to the
    • B - Cancer has spread to the pelvic lymph nodes and/or sites more distant.


T N M STAGING

  • T-0 pre-malignant change

  • T-1

    • A a cancer less than 2.0cm in diameter and less than 1.0mm in depth of invasion
    • B a cancer less than 2.0cm in diameter but greater than 1.0mm in invasion
  • T-2 greater than 2.0 centimeters in diameter

  • T-3 involves vagina, urethra or anus

  • T-4 involves bladder, rectum or pelvic bone N-0 no lymph nodes involved

  • N-1 lymph node metastases to one groin N-2 lymph node metastases to both groins

  • M-1 any distant metastases

  • M-0 no distant metastases





Diagnosis

  • The diagnosis often is delayed:

  • 1- Patients do not ask early consultation. They consider the symptoms as a trivial skin condition.

  • 2- Physicians may neglect small skin lesions.

  • Types of complaints:

  • The most common complaint is a palpable vulvar lesion.

  • Chronic pruritus vulvae.

  • Asymptomatic 20% of patients: the lesion is detected during examination for unrelated condition.

  • Later the lesion becomes necrotic cauliflower or hard ulcerated.

  • Melanomas: frequently appear as bluish black, pigmented, or papillary lesions.



Diagnosis

  • 1) Local examination of the relevant areas: early lesions appears as chronic vulvar dermatitis.

  • 2) Clinical assessment of the lymph nodes is to be performed in the relevant regions.

  • 3) Biopsy: 1- From the suspected lesions:

  • a) Dermal punch biopsy using a local anesthetic: Lesions < 1 cm

  • b) Excisional biopsy under general anesthesia: Lesions > 1 cm:

  • 2- From the lymph nodes in the relevant regions when suspected for metastasis.

  • Differential diagnosis:

  • 1- Venereal diseases: syphilis, chancroid, lymphogranuloma venereum, granuloma inguinale.

  • 2- VIN. An association between invasive and noninvasive lesions is a possibility.

  • 3- Condyloma acuminatum.







Prophylaxis…A high index of suspicion

  • Detection and management of VIN.

  • Proper management of all cases with pruiritus vulvae.

  • All vulval lesions should be diagnosed accurately especially those arising after menopause.

  • All pigmented vulvar lesions should be removed for biopsy.





Treatment Options by Stage



Vulvectomy:

  • There are several operations in which part of the vulva or all of the vulva is removed:

    • A skinning vulvectomy means only the top layer of skin affected by the cancer is removed. Although this is an option for treating extensive VIN3, this operation is rarely done.
    • Simple vulvectomy, the entire vulva is removed.
    • Radical vulvectomy can be complete or partial.
      • When part of the vulva, including the deep tissue, is removed, the operation is called a partial vulvectomy.
      • In a complete radical vulvectomy, the entire vulva and deep tissues, including the clitoris, are removed.
    • An operation to remove the lymph nodes near the vulva is called a en block dissection. It is important to remove these lymph nodes if they contain cancer.










Sentinel Lymph Node



Skinning / Simple Vulvectomy



Radiotherapy

  • Malignant diseases of the vulva are not commonly managed by RT because of the intolerance of surrounding normal tissues.

    • Chemotherapy as radiation sensitizer can improve response of the malignant tissues.
  • Indications of RT in malignant diseases of the vulva:

    • Preoperative RT in stage III and IV:
      • The lesion shrunk and it limits the need for pelvic exenteration.
      • It also improves surgical respectability of tumors.
    • Postoperative RT: can reduce regional recurrences and inguinal lymph node metastases.
      • Multiple positive groin nodes: It decreases the incidence of recurrence.
      • Positive surgical margins as seen on microscopic examination.
      • Multiple focal recurrences.
      • When the tumor size is > 4 cm






Malignant Tumors of the Vagina



Incidence

  • Incidence

    • 1% of gynecologic malignancies.
    • It is the 5th in frequency of primary genital malignant diseases.
    • Average age at diagnosis is 65 years old.
  • High risk factors:

    • VaIN
    • Human papillomavirus infection of the cervix or the vulva.
    • Cervical or vulvar cancer.
    • Exposure to diethylstilbestrol (DES) in utero is associated with the development of vaginal adenosis
      • It might progress to clear cell adenocarcinoma of the vagina and cervix in young wome
      • The mean age at diagnosis of this rare malignancy is 19 year.


Pathological Types:

  • Secondary malignant tumors of the vagina are more common than the primary tumors.

    • The primary lesion may be in the cervix or elsewhere in the body.
  • Primary malignant vaginal tumors:

    • Squamous cell carcinomas: 85% of primary vaginal malignancies.
    • Adenocarcinomas. These occur at younger age group.
    • Clear cell adenocarcinomas secondary to DES exposure.
    • Melanoma.
    • Sarcoma: Sarcoma botryoides (embryonal rhabdomyosarcoma)
      • The peak incidence is in young children at the age of 3 years.


Symptoms of malignant lesions of the vagina:

  • Symptoms of malignant lesions of the vagina:

    • Abnormal vaginal bleeding: may be postcoital, intermenstrual, or postmenopausal.
    • Watery vaginal discharge.
    • Dyspareunia.
    • Vesicovaginal or rectovaginal fistulae are late manifestations of vaginal cancer.
    • Few patients are asymptomatic; a lesion may be discovered during a routine pelvic examination, or a Pap smear may be abnormal.
  • Signs of malignant lesions of the vagina::

    • Local examination: the need to inspect the whole vagina entails modification from the routine speculum examination.
    • A polypoid lesion is the commonest macroscopic appearance.
    • A punch biopsy usually yields a diagnosis, but occasionally wide local excision using an anesthetic is necessary. Most lesions occur in the upper 1/3 of the vagina on the posterior wall.
    • Colposcopy, cystoscopy, and proctosigmoidoscopy and bone scan are needed to detect spread.


Spread:

  • Direct spread: into the local paravaginal tissues, bladder, or rectum.

  • Lymphatic spread:

    • Lesions in the lower vagina: to the inguinal lymph nodes.
    • Lesions in the upper vagina: to the pelvic lymph nodes.
  • Hematogenous spread: late event. Reach liver, lung, bone.



Staging





Treatment:

  • Management of stage 0:

    • Topical fluorouracil for stage 0:
      • It causes intense burning.
      • Long standing benefits is not proven yet
      • Laser therapy
  • Primary localized tumors:

    • RT: a combination of external beam and brachytherapy.
    • Surgery: it is an alternative treatment for early lesions.
      • Radical hysterectomy with upper vaginectomy.
      • Pelvic exenteration: in vesicovaginal or rectovaginal fistulas.


Prognosis:

  • The 5-year survival is as follows:

    • Stage I 65-70%
    • Stage II 47 %
    • Stage III 30%
    • Stage IV 15-20%


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