Download CANCER OF THE VAGINA Dr Samar Sarsam

CANCER OF THE
VAGINA
Dr Samar Sarsam
Cancer of the vagina is rare, representing about 1-2% of
gynecological cancers.
It is almost always a squamous cell cancer.
The exception is an adenocarcinoma that occurs in women
who were exposed to DES (diethylstilbestrol) in-utero. One
of the reasons that it is rare is that cancers of the vagina
that also involve the vulva are considered to be vulvar
cancers; if it involves the cervix it is considered to be a
cervical cancer.
There is a premalignant phase for squamous cell cancer of
the vagina similar to the squamous cell cancers of the
vulva and cervix.
The premalignant phase is vaginal intraepithelial neoplasia
grade III (VaIN III). This is also sometimes called
carcinoma-in-situ. The premalignant phase is usually
asymptomatic but can be detected by routine Pap test. It
can be treated by excision, laser evaporation or
occasionally by a chemotherapy type of vaginal cream.
There is no recognized cause for vaginal squamous
dysplasias or cancer, although it is similar to the
squamous dysplasias of the cervix.
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Pathology:
Squamous cell carcinoma may be ulcerative or exophytic, It
usually involves the posterior wall of the upper third of the
vagina, but may be multicentric.
Lymphatic drainage of the vagina consists of meshwork in the
mucosa and sub mucosa, the upper third drainage is as
cervical cancer, the lower third as vulvar cancer, the middle
third may metastasise to inguinal lymph nodes or to the deep
pelvic lymph nodes.
Melanoma rarely occurs in the anterior surface and lower half
of the vagina.
Sarcoma of the vagina occurs in children under 5 years of age,
rabdomyosarcoma in the upper anterior vaginal wall called
sarcoma botryoides.
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Clear cell adenocarcinoma arise in conjunction with vaginal •
adenosis in women who were exposed to DES
(diethylstilbestrol) in-utero. Adenocarcinomas of the vagina
associated with DES exposure were more frequent in the 1970's
and 1980's. DES, diethylstilbestrol, is a synthetic estrogen
hormone that was given to pregnant women in the 1950's to try
to prevent miscarriages. The female infants of these women,
who took the DES, had some developmental abnormalities of
their vaginas and cervices that put them at risk for developing a
particular type of adenocarcinoma called a clear cell
carcinoma.
Metastatic adenocarcinoma to the vagina from adjacent or •
distant organs
Clinical findings: •
Vaginal cancer is often asymptomatic. May •
cause symptoms of abnormal bleeding,
postmenopausal bleeding and foul
discharge. Bleeding after intercourse is a
symptom of cancer of the vagina as well as
cancer of the cervix.
Diagnosis of primary vaginal cancer is •
established after eliminating other sources of
malignancies. History, examination,
cytological examination of the cervix,
endometrial biopsy, colposcopy and biopsy
Staging:
It is clinical not surgical
FIGO staging:
Stage 0 carcinoma in situ, intra
epithelial carcinoma
I
vaginal mucosa
II
to sub vaginal tissue but not to
pelvic wall
III
to pelvic wall
IV
beyond the true pelvis or to
rectum or bladder or distant metastasis
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Treatment: •
Pre treatment evaluation, include chest x-ray, IVU, •
cystoscopy, sigmoidoscopy, CT-scan.
Invasive squamous cell cancer of the vagina is •
usually treated by radiation. Although it can be
removed surgically, the bladder or rectum or both
would have to be removed with it in order to get a
good margin around the cancer. As a general rule
squamous cell cancers of the vagina do not spread
early, so they are usually localized to the pelvic area
on diagnosis. This is a good situation for radiation
since that area can easily be irradiated and
radioactive material can easily be placed into the
vagina next to the cancer. The prognosis for
localized disease is good.
The type of surgery and radiotherapy depend on the
site of the tumor:
In the upper vagina treat as ca cervix.
In lower vagina as cancer of the vulva.
Radiation used is external pelvic irradiation followed
by intracavitary.
A very small tumor may be treated by vaginectomy.
Sarcoma botryoides is treated by chemotherapy plus
radiotherapy.
Melanomas treated with radiation and or surgery.
Women who have had a hysterectomy for non
cancer problems should still have a Pap test every
several years. They can still develop malignant and
pre-malignant vaginal changes.
Prognosis:
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5 year survival rate is 77% in stage I •
Melanomas are very malignant •
Sarcomas associated with recurrence •
Cancer of the vulva
Incidence: representing about 5% of all •
gynecologic cancers, and only about
1% of all female cancers in general.
the incidence has been rising over the •
past several years. The cause for the
growing number of cases is not wellunderstood.
Epidemiology: Predominantly a disease of older
women, but occasional cases in teenagers and not
infrequent in 20-40 yr old age group.
Vulvar cancer is most common in women over 50
years of age, with a median age of 65 – 75 years old
at diagnosis.
Types
85% are squamous cell carcinoma
10% are melanoma
5% are various rarities:
Adenocarcinomas of the vulva Adenocarcinoma:
are also rare, but can develop from glands such as
the Bartholin's glands at the vaginal opening.
Verrucous carcinoma
Sarcomas
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:Site •
About 70% of vulvar cancers involve the labia (mainly the labia •
majora).
15% - 20% involve the clitoris, and another 15% - 20% involve the •
perineum, which is the area of sensitive skin located between the
vagina and the anus.
In about 5% of cases, the cancer is present at more than one site. •
Etiology •
Risk factors
In addition to older age, vulvar cancer has been associated with a
history of:
infection with high-risk HPV types, (i.e.: HPV 16,18,31)
multiple sexual partners/ sexually transmitted diseases
cervical cancer
immunodeficiency
presence of chronic vaginal and vulvar irritation
smoking
Cancer predominantly arises in areas of vulval intra-epithelial
neoplasia.
HPV is thought to be the precursor.
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Clinical Features & Presentation
Pre-malignant lesion = Leukoplakia
characterized by white patches around vulva
due to skin thickening & hypertrophy
it is itchy
biopsy
Malignancies usually present with
lump (hard nodule)
ulcer with sloughing base & raised edges
(indurated ulcer with everted edge strongly
suggests carcinoma)
pain and bleeding from vulva
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The size of the lesion often correlates with its •
progression; 50% present with lymph node
involvement already present. 50% arise on one
labium majus, 25% arise on a labium minus. Some
cases have multiple affected areas. Young women
often present with malignant change in a vulval
condyloma. May have had persistent vulval itching
for months or years.
The classic symptom is vulvar itching (pruritus), •
reported in almost 90% of the women with vulvar
cancer. There can also be associated pain, bleeding,
vaginal discharge, and/or painful urination (dysuria).
Also, women often develop a visible vulvar mass:
the squamous cell subtype can look like elevated
white, pink or red bumps, while vulvar melanoma
characteristically presents as a colored, ulcerated
growth. There can be portions of the tumor that look
sore and scaly or cauliflower-like (similar to HPVrelated warts)
Diagnoses •
thorough gynecological examination should be •
performed using a colposcope (special magnifying
instrument) for better visualization. Any suspicious
areas should be tested by applying a dilute solution
of acetic acid to the region; abnormal areas typically
turn white, making them easier to identify. Also, any
abnormal-appearing area should be sampled along
with surrounding normal tissue using a thick wedgeshaped biopsy (usually under local anesthesia). If
the area is small, it should be entirely removed in the
process of the biopsy (so-called excisional biopsy).
Chest x-ray and CT scan of the abdomen/pelvis can
be done to look for disease spread to lymph nodes
and/or distant organs. If spread to bladder or rectum
is suspected, endoscopy (cystoscopy and
proctoscopy, respectively) should be performed
Pathology •
Progression of vulval intra-epithelial neoplasia (VIN) is a preinvasive phase very like CIN and similarly associated with HPV
infection - types 16, 18, 33.
Even very early stages of invasion through basement
membrane are associated with metastasis. This tumour
spreads rapidly to inguinal lymph nodes by embolisation within
lymphatic (rather than permeation). From there it passes to the
femoral and pelvic nodes.
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Staging •
Unlike vaginal cancer, which is typically clinically staged, the •
International Federation of Gynecology and Obstetrics (FIGO)
uses a surgical staging system for vulvar cancer. This means
that the stage of the cancer is not actually determined until
after surgery is performed and the specimen is examined by
the pathologist. Like vaginal cancer, vulvar cancer has five
main FIGO stages (0, I, II, III, and IV). They are:
Stage 0 - Vulvar intraepithelial neoplasia
Stage I - cancer is limited to the vulva and
perineum, and measures < 2 cm in size
Stage II - cancer is limited to the vulva and
perineum, but tumor is > 2 cm in size
Stage III - cancer spread to vagina, urethra,
anus, and/or the lymph nodes in the groin
Stage IV - cancer spread to bladder, bowel,
pelvic bone, pelvic lymph nodes, and/or
other parts of the body
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T N M STAGING OF VULVAR CANCERS
T-0 pre-malignant change
T-1A a cancer less than 2.0cm in diameter
and less than 1.0mm in depth of invasion
T-1B 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-0 no distant metastases
M-1 any distant metastases
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Vulvar cancer treatment: •
Treatment •
Basis of treatment is excision
small non-invasive VIN may be treated with
laser (esp. good for small multiple lesions) or
modified vulvectomy NB large lesions cannot
be treated with the laser as it is too painful
simple vulvectomy + prophylactic post-op
radiotherapy to inguinal lymph nodes for
small vulval carcinomas of < 2cm
radical vulvectomy if carcinoma > 2cm +
lymphadenectomy (of either just inguinal or
in more advanced disease inguinal, femoral
and pelvic lymph nodes
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Wherever possible vulvectomy is avoided •
because it is so traumatic and
psychologically distressing and a local
excision operation with 5mm borders is
performed.
Topical 5-fluorouracil cream is useful in 50% •
cases but patient tolerance is low as the
cream causes ulceration. In frail elderly
patients palliative care and even palliative
surgery is very important as the late stages
of vulval carcinoma have serious morbidity.
Surgery, radiation therapy and chemotherapy •
are the main treatment options, and are
typically used in various combinations. As
with many cancers, the optimal treatment
depends on the disease stage and patient
factors such as age and other medical
conditions.
Treatment options by stage are as follows:
Stage 0
Wide local excision, laser surgery, or a
combination of both
Skinning vulvectomy
Chemotherapy ointment
Stage I
Wide local excision
Radical local excision with removal of all
nearby groin/ upper thigh lymph nodes
Radical vulvectomy and removal of nearby
groin lymph nodes (and sometimes lymph
nodes on opposite side of the body)
Radiation therapy alone (in selected
patients)
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Stage II
Radical vulvectomy and removal of groin lymph
nodes on both sides of the body, plus postoperative
radiation therapy to the pelvis if lymph nodes are
positive for cancer
Radiation therapy alone (in selected patients)
Stage III
Radical vulvectomy and removal of groin/ upper
thigh lymph nodes on both sides of the body, plus
postoperative radiation therapy to the pelvis and
groin if lymph nodes are positive for cancer or if the
primary vulvar tumor is very large
Radiation therapy and chemotherapy, followed by
radical vulvectomy and removal of lymph node
removal of lymph nodes on both sides of the body.
Radiation therapy (in selected patients) with or
without chemotherapy.
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Stage IV
Pelvic exenteration, which entails radical vulvectomy and
removal of the lower colon, rectum, or bladder (depending on
where the cancer has spread), as well as the uterus, cervix, and
vagina
Radical vulvectomy followed by radiation therapy
Radiation therapy followed by radical vulvectomy
Radiation therapy (in selected patients) with or without
chemotherapy, and possibly following surgery
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Complications •
Wound breakdown (necrosis) and infection are common
problems.
Chronic lymphoedema of lower limbs occurs due to node
dissection in ~ 20%.
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Prognosis •
In early stage disease, when lymph nodes are not involved, the •
overall 5-year survival rate is 90%.
Once cancer has spread to the lymph nodes, the overall 5-year •
survival rate drops to 50% - 70%.