Transcript VULVAR AND VAGINAL CARCINOMA

Vaginal Cancer
Vaginal Cancer
Rare tumor representing only 1-2% of all
gynecologic malignancies
 80-90% are metastatic
 Mean age of patients with primary vaginal
cancer is 60-65 years
 Most primary tumors are squamous cell in
origin
 HPV DNA identified in VAIN
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Vaginal Cancer precursors
VAIN – avg age of VAIN 3 is 53
 Ratio of VAIN to CIN is 1:23
 5% progress to Vaginal Ca
 Hallmark of VAIN
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– cytologic atypia-Pleomorphisim, irreg nuclear
contours and chromatin clumping
– Abnormal maturation
– nuclear enlargement
Vaginal Cancer precursors
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VAIN 3
– usually occurs in upper third of vagina and is
multifocal and diffuse in half the cases.
– 1/3 of patients have a hx/o CIN
– CIN coexists w/ VAIN in 10-20% of pts
– Colposcopic findings are similar to those of CIN
(aceto white epithelium with punctations and
mosaic patterns)
Vaginal Cancer precursors
VAIN 1Proliferation of basal layer
Koilocytotic atypia
Enlarged pleomorphic nuclei
vacuolated cytoplasm
Vaginal Cancer precursors
VAIN 2Proliferation of basal layer,crowding
and loss of polarity
Koilocytotic atypia
Enlarged pleomorphic nuclei
vacuolated cytoplasm
Vaginal Cancer precursors
VAIN 3
Increased proliferation of abnormal basal
and parabasal cells replacing full
thickness of epithelium
Vaginal Cancer precursors
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Treatment Options for VAIN
– Excisional Bx for small lesions
– Partial Vaginectomy
– Laser Vaporization
– Intravaginal 5FU cream
Vaginal Cancer: Predisposing
Factors
Low socioeconomic status
 History of genital warts
 Vaginal discharge or irritation
 Previously abnormal Pap smear
 Early hysterectomy
 Previous pelvic radiation (?)
 In-utero exposure to DES
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Anatomy of the Vagina
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Muscular dilatable tube averaging 7.5 cm in length
Vaginal wall composed of three layers: mucosa,
muscularis, adventitia.
Epithelium normally contains no glands and
changes little during reproductive cycle
Lymphatic drainage of upper vagina via pelvic
nodes while lower vagina drains via femoral and
inguinal nodes.
Natural History and Patterns of
Spread
Lesions usually found in the upper vagina on
the posterior wall
 Vaginal primary tumors may spread along
mucosa to cervix or vulva (changes
diagnosis)
 Direct extension to bladder, parametria,
paracolpos, rectum, cardinal ligaments,
uterosacral ligaments
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Gross and microscopic Findings
50% of Vag Ca ulcerative
 30% are exophytic
 20%are annular and constricting
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Natural History and Patterns of
Spread
Any of the nodal groups may be involved
regardless of the location of the tumor
 Inguinal nodes most often involved if lesion is
in the lower 1/3 of the vagina
 Clinically apparent inguinal node mets seen in
5-20% of patients
 Incidence of pelvic nodes varies with stage
and location of the tumor
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Lymphatic Drainage of Vagina
Clinical Presentation
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Abnormal vaginal bleeding
– 50-75% of patients with primary tumors
Dysuria
 Pain
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Diagnostic Work-up
Complete history and physical
 Speculum examination and palpation of the
vagina
 Bimanual pelvic and rectovaginal examination
 Pap smear, colposcopy, directed biopsies
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Diagnostic Work-up
Cystoscopy
 Proctosigmoidoscopy
 Chest X-ray
 IVP
 Barium enema
 Computed Tomography
 MRI (84% PPV, 97% NPV)
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Staging
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Stage I - Lesions confined to the mucosa
Stage II- Subvaginal tissue involved but no
extension to pelvic sidewall
– IIA: Subvaginal infiltration only
– IIB: Parametrial extension
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Stage III- Pelvic sidewall extension
Stage IV- Bladder or rectal extension and/or direct
extension outside of true pelvis
Staging
Natural History and Patterns of
Failure
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Stage I
– 10-20% pelvic recurrence, 10-20% distant
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Stage II
– 35% pelvic recurrence, 22% distant
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Stage III
– 25-37% pelvic recurrence, 23% distant
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Stage IV
– 58% pelvic recurrence, 30% distant
Pathology
Squamous Cell CA represents 80-90% of
primary tumors
 Vaginal SCCA may be considered primary if
there is neither cervical or vulvar CA at
diagnosis or for 10 years prior
 No correlation between grade and survival
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Verrucous Carcinoma
Variant of well-differentiated SCCA that rarely
occurs in the vagina
 Relatively large, well-circumscribed, soft
cauliflower-like mass
 Cytologic features of malignancy are lacking
 May recur locally after surgery but rarely, if
ever, metastasizes
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Pathology
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Melanoma
– 2nd most common vaginal cancer
– Most frequently found in the lower third
– Cells may be spindle shaped, epithelioid, or small
lymphocyte-like, pigmented or non-pigmented
– Junctional activity helps exclude the possibility of
a metastasis
– Depth of invasion best predictor of survival
Pathology
Smooth muscle tumors
 Small Cell Carcinoma
 Endodermal Sinus Tumor
 Rhabdomyosarcoma (Sarcoma Boytrioides)
 Malignant lymphoma
 Clear Cell Adenocarcinoma
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Management
Radiation therapy is the preferred treatment
for most carcinomas of the vagina
 Surgical therapy
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– Irradiation failures
– Non-epithelial tumors
– Stage I Clear cell adenocarcinomas in young
women
Management
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Surgery
– Stage I tumors of the middle or upper third of
vagina treated with radical hysterovaginectomy
and PLND
– Stage I tumors of the lower third of vagina which
may encroach on the vulva treated with radical
vulvovaginectomy and bilat. groin node
dissection
– Pelvic exenteration possible for more invasive
lesions
Management
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Stage I
– Usually managed with RT
– Superficial lesions (<1cm) may be treated with
vaginal cylinder covering the entire vagina (6-7
Gy mucosal dose + 2-3 Gy dose to tumor)
– Thicker lesions may be treated with vaginal
cylinder + single plane implant
– EBRT reserved for aggressive lesions (infiltrating
or poorly differentiated)
Vaginal Cylinder + Single Plane
Implant
Management
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Stage I
– Radical hysterectomy, partial vaginectomy, PLND
may be used for lesions of the posterior and
lateral vaginal fornices
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Stage IIA
– WPRT (2000cGy) + parametrial boost for
4500cGy-5,000cGy total
Management
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Stage IIA
– WPRT (2000cGy) + parametrial boost for
4500cGy-5,000cGy total
– WPRT + combination of intracavitary and
interstitial implants for 5000 to 6000 cGy total
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Stage IIB, III, IVA
– WPRT (4000 cGy) + parametrial boost (2500
cGy)
Management
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Small Cell Carcinoma
– Reasonable local control may be obtained with
surgery or irradiation followed by systemic chemo
– Cyclophosphamide, Adriamycin, Vincristine (CAV)
X 12 cycles (some prior to initiation of RT)
– Doses of RT similar to SCCA
Management
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Rhabdomyosarcoma
– Generally treated with a combination of surgery,
RT, and chemotherapy
– Vincristine, Dactinomycin, Cyclophosphamide
(VAC) X 1-2 years effective adjuvant treatment
for stage 1 dz
– Local excision + interstitial/intracavitary RT +
systemic chemo has replaced radical pelvic
surgery as therapy of choice
Sarcoma Botryoides
Sarcoma Botryoides
Strap cell
Management
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Malignant Lymphoma
– Vaginectomy and radical hysterectomy or pelvic
exenteration has been used for localized vaginal
tumors
– Satisfactory results with pelvic RT (tele and
brachytherapy) + systemic chemo
– Cyclophosphamide, adriamycin, vincristine,
prednisone (CHOP) X 6 cycles most often used
Clear Cell Adenocarcinoma and
DES Exposure
Incidence is between 0.14 to 1.4/1000
women exposed to DES
 Median age at diagnosis 19 years
 Lesions found mainly in the upper 1/3 of the
anterior vaginal wall
 90% of patients with early stage disease (I
and II) at diagnosis
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Management
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Clear Cell Adenocarcinoma
– Surgery for stage I lesions has advantage of
ovarian preservation and better vaginal function
following skin graft
– Vaginectomy, radical hysterectomy PLND,
paraaortic LNBx (frozen section of distal margin)
– Intracavitary or transvaginal radiation can be
used for small lesions
– More extensive lesions: EBRT
Clear cell adenocarcinoma
FAVORABLE FACTORS IN SURVIVAL OF
PATIENTS WITH CLEAR CELL
ADENOCARCINOMA
Low stage
 Older age
 Tubulocystic Pattern
 Small tumor diameter
 Reduced depth of invasion
 Negative nodal mets
 Positive ho/o DES
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Radiation Therapy Techniques
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EBRT delivered through AP:PA portals or using 4
field “box technique”
15 cm X 15 cm or 15 cm X 18 cm portals usually
adequate
Inguinal nodes should be electively covered (45005000cGy) for tumors of the lower 1/3 of vagina
Additional 1500cGy (4-5cm depth) delivered for
palpable inguinal nodes
Radiation Therapy Techniques
Portal for pelvic RT and
elective groin coverage
Portal for groin coverage
with palpable inguinal
nodes
Radiation Therapy Techniques
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Intracavitary therapy utilizes vaginal cylinders
(Burnett, Bleodorn, Delclos, or MIRALVA
applicators)
Upper 1/3 lesions can be treated with tandem and
ovoids
Interstitial therapy with 137Cs, 226Ra, or 192Ir needles
have been used
High dose rate brachytherapy (>1200cGy/hour)
also used
Summary
Superficial stage I lesions may be treated
with RT or radical hysterovaginectomy
 Stage IIA-IVA treated with WPRT and
intracavitary RT
 Role of chemotherapy in advanced SCCA
presently unknown
 Pelvic failures and distant metastases occur
in 1/2 of pts with advanced dz
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5 Year Survival
80
70
60
50
40
30
20
10
0
Stage I
Stage II Stage III Stage IV
The End