VULVA AND VAGINA

MALIGNANCIES
ANGELA A. EMBESTRO
OBGYN RESIDENT

COMPREHENSIVE GYNECOLOGY 7TH EDITION

SGOP CPG 2018, 2019
UP TO DATE
ANATOMY

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BLOOD SUPPLY
INTERNAL PUDENDAL ARTERY

INNERVATION
Ilioinguinal nerve
Genitofemoral nerve

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VULVA LYMPH DRAINAGE
• Rich in lymphatics with numerous
cross connections
• Tumors of either labium – drain
initially to the IPSIlateral femoral-
inguinal nodes . >98%
Ipsilateral ,< 2% contralateral
• Perineal nodes can spread to
either left or right side
• Anterior labial

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INTRODUCTION
 CANCER OF THE VULVA ACCOUNTS FOR APPROXIMATELY 5% OF MALIGNANCIES
OF THE LOWER FEMALE GENITAL TRACT
 4TH IN FREQUENCY AFTER ENDOMETRIUM, OVARY AND CERVIX
 Well-defined predisposing factors for the development of vulvar carcinoma have not been
identified.
 HPV INFECTION HAS BEEN NOTED IN ALMOST 70% OF PATIENTS WITH CARCINOMA
OF VULVA
 MOST COMMON PRESENTING SYMPTOMS; VULVAR PRURITUS, IRRITATION AND
PAIN

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PRIMARY VULVAR MALIGNANCIES
 Squamous Cell Carcinoma- MOST COMMON
 Adenocarcinoma (including Bartholin's gland)
 Verrucous carcinoma
 Basal cell carcinoma
 Melanoma
 Sarcoma

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SQUAMOUS CELL PRECURSORS
(WHO 2014)
▪ LOW GRADE- SIL (VIN 1)
▪ HIGH GRADE –SIL (VIN 2/3)
▪ DIFFERENTIATED TYPE

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8
PRIMARY PREVENTION (SGOP 2019
▫ IMMUNIZATION WITH HPV VACCINE CAN
PREVENT DEVELOPMENT OF VIN AND
VULVAR CA.

SECONDARY PREVENTION
▫ THERE IS NO RECOMMENDED SCREENING
METHOD FOR THE DETECTION AND
PREVENTION
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 SQUAMUS CELL
CARCINOMA
• comprise approximately 90%
of primary vulvar
malignancies
• GROSSLY : Usually appear
as raised, flat, ulcerated,
plaquelike, or polypoid
masses on the vulva
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SQUAMUS CELL
CARCINOMA

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DIAGNOSIS • COMPLETE PHYSICAL EXAMINATION
• assessment for tumor diameter, local
extension to vagina, urethra, anus,
bladder, rectum, and enlarged
inguinofemoral lymph nodes

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COLPOSCOPY (SGOP 2019)

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BIOPSY
▪ VULVAR BIOPSY
SHOULD BE DONE FOR
ALL VULVAR LESIONS
▪ WIDE LOCAL EXCISION

SGOP 2019
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 2009 FIGO STAGING CARCINOMA OF THE VULVA
STAGE DEFINITION
I-T1/2, N0, M0 TUMOR CONFINED TO THE VULVA
IA Lesions ≤2 cm in size, confined to the vulva or perineum and with stromal invasion ≤1.0 mm⁎, no
nodal metastasis
IB Lesions < 2 cm in size, with stromal inasion <10mm confined to the vulva, or perineum, with
negative nodes
II-T3,N0,MO TUMOR OF ANY SIZE WITH EXTENSION TO ADJACENT PERINEAL STRUCTURE
(1/3 lower urethra, 1/3 lower vagina, anus) no negative nodes
III-T1-3,N1/2,M0 TUMOR OF ANY SIZE WITH EXTENSION TO ADJACENT PERINEAL STRUCTURE
(1/3 lower urethra, 1/3 lower vagina, anus) with positive inguino femoral lymph nodes

IIIA (i.) With 1 lymph node metastasis(>5mm) or

(ii.) 1-2 lymph nodes metastasis(<5mm)
IIIB (i.) With 2 or more lymph nodes metastasis(>5mm) or
(ii) .3 or more lymph nodes metastasis(<5mm)

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 2009 FIGO STAGING CARCINOMA OF THE VULVA
STAGE DEFINITION

III

IIIIC With positive nodes, with extracapsular spread

IV- any T any N,M1 TUMOR INVADES OTHER REGIONAL(2/3 upper urethra, 2/3 upper vagina,) or distant structure

IVA Tumor invades any of the following

(i.) Upper urethral and or/ vaginal mucosa, bladder mucosa, rectal mucosa, or fixed to pelvic bone, or

( ii. ) Fixed or ulcerated inguino femoral lymph nodes

IVB Any distant metastasis including pelvic lymph nodes

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*The depth of invasion is defined as the measurement of the tumor from the epithelialstromal junction of the adjacent most
superficial dermal papilla to the deepest point of
TNM SYSTEM (TUMOR,
NODES, METASTASES)
• T: SIZE AND EXTENT OF
TUMOR
• N: CLINICAL STATUS OF
THE NODES
• M: PRESENCE OR
ABSENCE OF
METASTATIC DISEASE
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 TNM
T PRIMARY TUMOR

T1 TUMOR CONFINED TO THE VULVA OR PERINEUM, <2CM

T2 TUMOR CONFINED TO THE VULVA OR PERINEUM >2CM

T3 TUMOF OF ANY SIZE WITH ADJACENT SPREAD TO THE URETHRA, VAGINA

ANUS OR ALL OF THESE
T4 TUMOR OF ANY SIZE INFILTRATING THE BLADDER MUCOSA, RECTAL
MUCOSA, OR BOTH INCLUDING THE UPPER PART OF THE URETHRAL
MUCOSA OR MUCOSA OR FIXED TO THE ANUS

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 TNM
N REGIONAL LYMPH NODES

N0 NO NODES PALPABLE

N1 UNILATERAL REGIONAL LYMPH NODE METASTASES

N2 BILATERAL REGIONAL LYMPH NODE

M DISTANT METASTASES

M0 NO CLINICAL METASTASES

M1 DISTANT METASTASES INCLUDING PELVIC LYMPH NODES

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 VULVA LYMPH
DRAINAGE
• Rich in lymphatics with numerous cross
connections
• Tumors of either labium – drain initially
to the IPSIlateral femoral-inguinal nodes .
>98% Ipsilateral ,< 2% contralateral
• Perineal nodes can spread to either left or
right side
• Anterior labial

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 VULVA LYMPH
DRAINAGE
• Technetium 99m colloid
• Anterior labial, 92% IPSI, 8%
Contralateral side
• Clitoral and perineal , bilateral
nodal distribution
• No direct connection from the
clitoris to the deep nodes

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Natural History, Spread, and Prognostic Factors

• The prognosis of a patient with vulvar carcinoma is related to the

• stage of the disease,
• lesion size, as well as the
• presence or absence of cancer in regional nodes
• The status of the regional lymph nodes is the most important factor
prognostically and therapeutically

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Natural History, Spread, and Prognostic Factors
• Tumor stage, location on the vulva, microscopic differentiation, presence
or absence of vascular space involvement, and tumor thickness are all
important prognostic factors

• Additional independent predictors of positive nodes were

• tumor grade
• suspicious, fixed, or ulcerated lymph nodes
• lymphvascular space involvement
• older age of the patient;
• tumor thickness

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MANAGEMENT (SQUAMOUS CELL CACINOMA)
2018 SGOP
• EARLY STAGE VULVAR CANCER
• TUMORS CONFINED TO THE VULVA WITHOUT SUSPICIOUS LYMPH
NODES AS DETERMINED BY CLINICAL EXAMINATION WITH OR
WITHOUT ULTRASONIC OR RADIOLOGICAL ASSESSMENT

• ADVANCED STAGE VULVAR CANCER

• TUMORS EXTENDING BEYOND THE VULVA, OR BULKY POSITIVE
GROIN NODES

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 MANAGEMENT OF GROIN NODES

• BOTH INGUINAL AND FEMORAL NODES BE REMOVED AS

INGUINAL NODE DISSECTION ALONE IS ACCOCIATED WITH A
HIGHER INCIDENCE OF GROIN RECURRENCE

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 CLINICALLY SUSPICIOUS NODES
• IF CLINICALLY POSITIVE
• A COMPLETE LYMPHADENECTOMY SHOULD BE
AVOIDED BECAUSE FULL GROIN DISSECTION
TOGETHER WITH POSTOPERATIVE GROIN
IRRADIATION MAY RESULT IN SEVERE
LYMPHEDEMA.

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29
 CLINICALLY OBVIOUS NODES
• ULCERATED OR FIXED GROIN NODES
• RESECTED IF NOT INFILTRATING MUSCLE OR
FEMORAL VESSELS
• IF NOT RESECTABLE THEY SHOULD BE BIOPSIED TO
CONFIRM THE DIAGNOSIS THEN TREATED WITH
PRIMARY RADIATION

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31
 ROLE OF SENTINEL LYMPH NODE SAMPLING
• CRITERIA
• UNIFOCAL TUMOR CONFINED TO THE VULVA LESS THAN 4CM IN
DIAMETER
• >1MM INVASION
• RADIOACTIVE TRACER AVAILABLE- USE IS MANDATORY BLUE DYE USE IS
OPTIONAL
• NO CLINICALLY SUSPICIOUS LYMPH NODES
• NO PREVIOUS GROIN NODE DISSECTION
• NO PRIOR GROIN IRRADIATION
• NOT FOR MULTIFOCAL DISEASE
• NOT GROSSLY INFLAMED PRIMARY TUMOR
• NOT FOR RECURRENT DISEASE
• LARGE VOLUME SURGEON
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33
 EARLY STAGE
SGOP 2018
STAGE PRIMARY TREATMENT ADJUVANT TREATMENT

1A RADICAL LOCAL EXCISION OBSERVATION

1B RADICAL LOCAL EXCISION WITH OBSERVATION

UNILATERAL INGUI-FEMORAL
LYMPHADENECTOMY

RADICAL LOCAL EXCISION WITH BILATERAL

INGUI-FEMORAL LYMPHADENECTOMY IF:
1. LWSION WITHIN 1.0CM FROM MIDLINE
2. LABIA MINORA INVOLVED
3. IPSILATERAL POSITIVE NODES

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 EARLY STAGE SGOP 2018
STAGE PRIMARY TREATMENT ADJUVANT
STAGE II RADICAL LOCAL EXCISION WITH BILATERAL INGUI- IF WITH POOR PROGNOSTIC
FEMORAL LYMPHADENECTOMY FACTORS:

RADICAL LOCAL EXCISION AND SENTINEL NODE

DISSECTION; GROIN NODE DISSECTION DONE ONLY FOR +LINES OF RESECTION
THOSE WITH METASTASIS TO THE SENTINEL NODE SURGICAL VMARGINS <1.0CM
HISTOLOGICAL MARGINS <5MM
RADICAL VULVECTOMY WITH BILATERAL INGUI-FEMORAL THICKNESS >5MM
LYMPHADENECTOMY BY TRIPLE INCISION TECHNIQUE FOR +LVSI
LARGE TUMORS
DO: PELVIC, VULVAR AND
EN BLOC SINGLE INCISION (STANLEY-WAY) TECHNIQUE BILATERAL INGUINAL
RECOMMENDED FOR RADIOTHERAPY +CHEMOTHERAPY
1. INGUINAL NODES SUSPICIOUS OR POSITIVE
2. LESIONS WITHIN 1CM OF MIDLINE

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 ADVANCED STAGE
STAGE III TUMOR RESECTABLE WIHOUT REQUIRING IIIA: OBSERVATION
STROMA IIIB, IIIC OR IIIA WITH POOR
PROGNOSTIC FACTORS
RADICAL VULVECTOMY WITH BILATERAL 1. LINES OF RESECTION
INGUI FEMORAL LYMPHADENECTOMY 2. SURGICAL MARGINS
<1CM
3. HISTOLOGICAL
TUMOR RESECTION THAT WOULD REQUIRE MARGINS <5MM
STOMA 4. THICKNESS >5MM
5. +LVSI
PRI,ARY RADIATION PLUS CHEMOTHERAPY PELVIC, VULVAR AND
FOLLOWRED BY CONSIDERATION OF BILATERAL INGUINAL
SURGICAL RESECTION RADIOTHERAPY

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 ADVANCED STAGE

STAGE IV

PRIMARY RADIATION (PELVIC, VULVAR AND

BILATERAL INGUINAL RADIOTHERAPY)
PLUS CHEMOTHERAPY FOLLOWED BY
CONSIDERATION OF SURGICAL RESECTION

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Vulvectomy with
operative
incision lines –
TRIPLE
INCISION

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Radical Vulvectomy with operative incision lines – TRIPLE
INCISION

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Radical Vulvectomy with operative incision lines – TRIPLE
INCISION

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Radical Vulvectomy with GND

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Radical Vulvectomy Specimen

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BARTHOLIN'S
GLAND
CARCINOMA
• Enlargement of Bartholin's
gland in a postmenopausal
woman should raise
suspicion for this malignancy

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TREATMENT

• Treated similarly to primary squamous cell carcinoma of the vulva

• Radical vulvectomy with bilateral inguinal-femoral

lymphadenectomy
• treatment of choice
• If the regional lymph nodes are free of tumor, the prognosis is good

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 BASAL CELL
CARCINOMA
• arise in the vulva as it can arise in the skin elsewhere in the body

• MANAGEMENT
• Wide local excision of the lesion
• if the surgical resection margins are free of tumor, the disease is cured

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 VERRUCOUS
CARCINOMA
• special variant of squamous cell cancer with
distinctive histologic features
• appear as a large condylomatous mass on
the vulva
• Histologically, they consist of mature
squamous cells and extensive keratinization
with nests that invade the underlying vulvar
tissue

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 VERRUCOUS
CARCINOMA
• It is often necessary to perform multiple biopsies of the condylomatous lesion to establish a
diagnosis of malignancy
• Radiation therapy is ineffective and can worsen the prognosis by causing anaplastic
change in the tumor and is therefore contraindicated

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 VERRUCOUS CARCINOMA:
Management
• Wide excision
• Depending on the size and location of the tumor, simple vulvectomy may be
needed
• Radical vulvectomy or inguinal node dissection is not indicated.

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 VERRUCOUS CARCINOMA:
Management
• In the presence of areas of squamous cell carcinoma, local excision is inadequate
therapy
• Verrucous tumors with squamous cell carcinoma elements can metastasize to
regional nodes, and should not be treated as true verrucous carcinomas

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PAGET’S DISEASE OF THE
VULVA • Commonly seen in post
menopausal women
• Average age is 65years old
• CLINICAL PRESENTATION
• Complaint: Itchiness or
• Irritation for a prolonged time
• Grossly : eczematoid and
• reddish in appearance
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 PAGET’S
•
DISEASE
a rare intraepithelial disorder that occurs in the vulvar skin

• histologically resembles Paget's disease in the breast

• large pale cells

• tends to spread, often in an occult fashion, and recurrences are frequent after
treatment

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 PAGET’S
•
DISEASE
Once a diagnosis of Paget’s disease of the vulva is made, it is important to rule
out the presence of breast and GI malignancy.
• There is an increased association of Paget's disease of the vulva with underlying
invasive adenocarcinoma of the vulva, vagina, and anus as well as distant sites
including the bladder, cervix, colon, stomach, and breast

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PAGET’S DISEASE:
Microscopic Findings

 Paget cells are large pale cells, with prominent cytoplasm (granular or vacuolated)
within the epithelium
 Large nuclei, prominent nucleoli

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 PAGET’S
DISEASE
PRIMARY VPD (CUTANEOUS) TYPE 1A- CUTANEOUS VULVAR NON-INVASIVE PAGETS
DISEASE
TYPE 1B- CUTANEOUS VULVAR INVASIVE DISEASE:
DERMAL INVASION OF PAGETS CELLS
TYPE 1C: CUTANEOUS VULVAR DISEASE AS A
MANIFESTATION OF AN UNDERLYING VULVAR
ADENOCARCINOMA
SECONDARY VPD (NON TYPE 2- VPD ORIGINATES FROM RECTAL OR ANAL
CUTANEOUS) ADENOCARCINOMA
TYPE 3- VPD ORIGINATES FROM UROGENITAL
NEOPLASIA

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TREATMENT

• WIDE LOCAL EXCISION WITH OR WITHOUT INGUINOFEMORAL LYMPH

NODE DISSECTION
• FOR SECONDARY PAGETS:>1MM DEPTH IS REQUIRED
• IF AN UNDERLYING ADENOCARCINOMA HAS NOT BEEN RULED OUT
REMOVAL OF A SMALL AMOUNT OF SUBCUTANEOUS TISSUE
• FROZEN SECTION OF SURGICAL MARGINS IS RECOMMENDED TO
ENSURE COMPLETE REMOVAL OF TUMOR

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 MELANOMA
• Account for approximately 4% to 5% and the other types for the remainder
• Most frequent nonsquamous cell malignancy of the vulva
• Arise from junctional or compound nevi
• Pigmented lesions of the vulva are usually junctional nevi, and all such lesions should be
removed by excision
• Most occur on the labia minora or the clitoris
• Use the same FIGO classification used for squamous carcinomas

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MELANOMA

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 Nodular
melanoma
arising directly
from glans
clitoris

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 MELANOMA
• Staging is not as useful a prognostic indicator as is the depth of invasion
• Superficial spreading is more common and has a better prognosis

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MELANOMA: TREATMENT & PROGNOSIS

• Primary Treatment
• Wide excision of the primary tumor
• Prognosis is poor for patients with melanomas more than 3 mm thick
• If the regional nodes are negative, the survival rate is greater than 60%,
but decreases to less than 30% if the regional nodes are involved with
tumor

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MALIGNANT DISEASES
OF THE VAGINA
▪ BLOOD SUPPLY
▫ CERVICAL BRANCH
OF THE UTERINE
ARTERY
▫ VAGINAL ARTERY
▫ MIDDLE RECTAL
ARTERY
▫ INTERNAL
PUDENDAL ARTERY
LYMPHATIC DRAINAGE
OF THE VAGINA
• The lymphatics of the vagina envelop the
mucosa and anastomose with lymphatic
vessels in the muscularis
• Middle to upper vagina – drain into the
pelvic nodes of the obturator and internal
and external iliac chains

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LYMPHATIC DRAINAGE
OF THE VAGINA
• Distal third of the vagina – drain to both the
inguinal nodes and the pelvic nodes
• The posterior wall lymphatics
• anastomose with the rectal lymphatic
system and
• then to the nodes that drain the rectum,
such as the inferior gluteal, sacral, and
rectal nodes

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GENERAL GUIDELINES
• DIAGNOSED BY BIOPSY AND CLINICALLY STAGED USING THE
2009 FIGO
• COLPOSCOPY, CYSTOSCOPY AND PROCTOSIGMOIDOSCOPY, IF
CLINICALLY INDICATED
• RADIATION THERAPY IS THE TREATMENT OF CHOICE
• FOR YOUNG PATIENTS WHO REQUIRE RT: PRE TREATMENT
LAPAROTOMY , SURGICAL STAGING AND RESECTION OF ANY
BULKY POSITIVE LN

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 PRIMARY VAGINAL CANCER
• Rare ( less than 2% ), most are METASTATIC
• Malignant transformation of endometriosis has been described in the
vagina and rectovaginal septum
• Survival: overall 5yr SR – 45%
• Stage I 82%, Stage II 53%
• Stage of the tumor is the most important predictor of prognosis.

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 Common Primary Vaginal Cancers
Predominant
Tumor Type Age (years) Clinical Correlations
Endodermal sinus tumor <2 Extremely rare, α-fetoprotein secretion,
(adenocarcinoma) often fatal, multimodality therapy

Sarcoma botryoides <8 Aggressive malignancy, multimodality

therapy
Clear-cell adenocarcinoma >14 Associated with intrauterine exposure to
diethylstilbestrol
Melanoma >50 Very rare, poor survival
Squamous cell carcinoma >50 Most common primary vaginal cancer

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 MALIGNANT DISEASE OF THE
VAGINA
• Symptoms and Diagnosis
• Primary vaginal cancers usually occur as squamous cell carcinomas in
women older than age 60
• To be considered a primary vaginal tumor, the malignancy must arise in
the vagina and not involve the external os of the cervix superiorly or the
vulva inferiorly

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 MALIGNANT DISEASE OF THE
VAGINA
• Most common symptom: abnormal bleeding or discharge
• Pain is usually a symptom of an advanced tumor
• Urinary frequency is reported in the case of anterior wall tumors
• Constipation or tenesmus may be reported when the tumors involve the
posterior vaginal wall
• Usually diagnosed by direct biopsy of the tumor mass

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TUMORS
OF ADULT VAGINA
 SQUAMOUS CELL
CARCINOMA

• Most common of the vaginal malignancies

• Occurs primarily in those older than age 60

• Most occur in the upper third of the vagina

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SQUAMOUS CELL
CARCINOMA

• Grossly, the tumor appears as

a fungating, polypoid, or
ulcerating mass, often
accompanied by a foul smell
and discharge related to a
secondary infection

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SQUAMOUS CELL
CARCINOMA

• Microscopically, the tumor

demonstrates the classic
findings of an invasive
squamous cell carcinoma
infiltrating the vaginal
epithelium

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 SQUAMOUS CELL
CARCINOMA
• Treatment is based on the size, stage, and location
• Therapy is limited by the proximity of the bladder anteriorly and the
rectum posteriorly
• It is also influenced by the location of the tumor in the vagina, which
determines the area of lymphatic spread

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 2009 International Federation of Gynecology and Obstetrics
Staging Classification for Vaginal Cancer

Stage Characteristics
I The carcinoma is limited to vaginal wall
II The carcinoma has involved the subvaginal tissue but has not extended to the pelvic wall

III The carcinoma has extended to the pelvic wall

IV The carcinoma has extended beyond true pelvis or has involve the mucosa of bladder or
rectum; bullous edema as such does not permit a case to be allotted to stage IV)
IVA Tumor invades bladder and/or rectal mucosa and/or direct extension beyond the true
pelvis
IVB Spread to distant organs

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Staging of Vaginal Cancer

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Staging of Vaginal Cancer

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 MANAGEMENT
STAGE TREATMENT OPTIONS
STAGE 1 1. COMPLETE RADIOTHERAPY:PELVIC EBRT
FOLLOWED BY INTERSTITIAL OR
INTRACAVITARY RADIOTHERAPY
2. WIDE LOCAL EXCISION OR TOTAL
VAGINECTOMY WITH VAGINAL
RECONSTRUCTION
3. FOR LESIONS INVOLVING UPPER POSTERIOR
THIRD OF THE VAGINA, RH WITH PELVIC
LYMPHADENECTOMY
4. FOR LESIONS OF THE LOWER THIRD OF THE
VAGINA
• COMPLETE RT + INGUINAL BOOST
• RADICAL VAGINECTOMY WITH INGUINAL
LYMPHADENECTOMY
5. FOR POORLY DIFFERENTIATED TUMORS:
COMPLETE RT +- INGUINAL EBRT
ADJUVANT THERAPY
FOR THOSE WHO UNDERWENT
PRIMARY SURGERY:
• IF WITH <1CM OR POSITIVE
SURGICAL MARGIN,
INTERSTITIAL OR VAGINAL
BACHYTHERAPY
• IF WITH INCOMPLETE
EXCISION OR WHEN LN WERE
HISTOPATHOLOGICALLY
POSITIVE FOR METASTASIS,
PEBRT WITH INTERSTITIAL OR
VAGINAL BRACHYTHERAPY
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 MANAGEMENT
II-IVA 1.COMPLETE RADIOTHERAPY:
PELVIC EBRT FOLLOWED BY INTERSTITIAL INTACAVITARY
BRACHYTHERAPY
2. CONCURRENT CHEMORADIATION
3. RADICAL SURGERY WITH PELVIC LYMPHADENECTOMY WITH PRE-
OPERATIVE OR POST OP RT
FOR LESIONS INVOLVING LOWER 3RD OF VAGINA ADD BILATERAL
GROIN NODE DISSECTION
4. FOR STAGE II TUMORS, NEOADJUVANT CHEMOTHERAPY
FOLLOWED BY SURGERY (RHBSO AND RADICAL VAGINECTOMY WITH
BLND MAY BE AN OPTION

83
 MANAGEMENT
IV B

COMPLETE RADIOTHERAPY: PELVIC EBRT

FOLLOWED BY INTERSTITIAL OR INTRACAVITARY
BRACHYTHERAPY

CONCURRENT CHEMORADIATION

SYSTEMIC CHEMOTHERAPY

PALLIATIVE TREATMENT
84
 CLEAR CELL
ADENOCARCINOMA
• Seen more frequently in young women since 1970 as a result of the
association of many of these cancers with intrauterine exposure to DES
• Therapeutic considerations are similar to those for squamous cell
carcinoma, taking into account the young age of the patients undergoing
therapy
• The survival rate is related directly to the stage of the tumor

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CLEAR CELL ADENOCARCINOMA

Tubulocystic pattern Solid pattern Papillary pattern

CLEAR CELL ADENOCARCINOMA

Surgery is the primary treatment modality because of young age

STAGE TREATMENT OPTIONS

I, II • Radical hysterectomy with partial or complete

vaginectomy, pelvic lymphadenectomy &
reconstruction of vagina
• Retroperitoneal lymph node dissections are
usually performed
• Local excision of tumor - <2cm diameter
- Tubulocystic pattern
- Invasion <3mm
CLEAR CELL ADENOCARCINOMA

Surgery is the primary treatment modality because of young age

STAGE TREATMENT OPTIONS

III • Full pelvic irradiation + Intracavitary implant

• No single agent or combination of
chemotherapeutic agents has emerged as an
effective therapy
CLEAR CELL ADENOCARCINOMA

• > 1yo
• Tubulocystic pattern
GOOD PROGNOSIS • Smaller tumor diameter
• Superficial depth of invasion
• (-) pelvic lymph node spread
MALIGNANT
MELANOMA
• Rare and highly malignant
• Most common presenting
symptoms: vaginal discharge,
bleeding, and a palpable mass
• Darkly pigmented, irregular areas and
may be flat, polyoid, or nodular

90
MALIGNANT MELANOMA:
TREATMENT & PROGNOSIS

• Metastasize early, via the bloodstream and lymphatics, to the iliac and/or
inguinal nodes, lungs, liver, brain, and bones
• Surgery with wide excision of the vagina and dissection of the
regional nodes (pelvic or inguinal-femoral, or both)
• Worse prognosis than those with vulvar melanoma
• Both local and distant recurrences are common, and the disease is usually
fatal

91
 MALIGNANT MELANOMA

STAGE TREATMENT OPTIONS

I-IV • Wide local excision

• Radiation therapy using high-
dose fractions
• Pelvic exenteration

Local and distant recurrences are

common, and the disease is usually
fatal.
 MALIGNANT MELANOMA

PROGNOSIS
• 5-year survival rate is 8.4%, with
an overall median survival of 20
months

IMPROVED SURVIVAL
• Tumors w/ fewer than six mitoses/hpf
• <3cm in diameter
 VAGINAL ADEMOCARCINOMA ARISING
IN ENDOMETRIOSIS

• Malignant transformation of extraovarian endometriosis

• Rare but with increasing incidence
• MC extragonadal location: Rectovaginal septum
• Typical presentation • Risk Factors
- Pain - Use of unopposed
- Vaginal bleeding estrogen
- Vaginal mass - Tamoxifen use
VAGINAL ADEMOCARCINOMA
ARISING
IN ENDOMETRIOSIS

TREATMENT
• Surgery
• Radiation + Chemotherapy
VAGINAL TUMORS
OF INFANT & CHILDREN
ENDODERMAL SINUS TUMOR
(Yolk-Sac Tumor)
• A rare germ-cell tumor that usually occurs in the ovary
• Secretes α-fetoprotein, which provides a useful tumor marker to monitor
patients treated for these neoplasms
• Aggressive, and most patients have died

97
ENDODERMAL SINUS TUMOR
(YOLK SAC TUMOR)

TREATMENT

Surgery + Irradiation +
Vincristine
Actinomycin D
VAC Chemotherapy Cyclophosphamide

Bleomycin
BEP Chemotherapy Etoposide
Cisplatin
SARCOMA BOTRYIODES
(Embryonal Rhabdomyosarcoma)
• Usually diagnosed in the vagina of a young female
• Rarely does it occur in a young child older than 8 years of age
• most common symptom: abnormal vaginal bleeding, with an occasional
mass at the introitus
• Begin in the subepithelial layers of the vagina and expand rapidly to fill the
vagina
• Often are multicentric

99
 SARCOMA BOTRYIODES
(Embryonal Rhabdomyosarcoma)

Grossly resemble a
cluster of grapes forming
multiple polypoid masses

100
SARCOMA BOTRYIODES
(Embryonal Rhabdomyosarcoma)

101
SARCOMA BOTRYIODES
(Embryonal Rhabdomyosarcoma)
Histologically, they have a
loose myxomatous stroma with
malignant pleomorphic cells
and occasional eosinophilic
rhabdomyoblasts that often
contain characteristic cross-
striations (strap cells)

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TREATMENT
• Pelvic exenteration
• Effective control with a multimodality approach consisting of
multiagent chemotherapy (VAC therapy), usually combined with
surgery
• Radiation therapy

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PSEUDOSARCOMA BOTRYIODES
• A rare, benign vaginal polyp that resembles sarcoma botryoides found in the
vagina of infants and pregnant women
• Although large atypical cells may be present microscopically, strap cells are
absent
• Grossly, these polyps do not resemble the grapelike appearance of sarcoma
botryoides
• Treatment : Local excision

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Thank You!

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