2. Epidemiology
• Vulvar cancer is a rare malignancy
• Represents < 1% of all the cancers diagnosed
in women and < 5% of all gynecologic
neoplasms
• More common with increasing age ; Median
age – 68 years
2
3. Risk Factors
• Older age
• Precancerous changes (dysplasia) in vulvar tissues
• Lichen sclerosus, which causes persistent itching and scarring of the
vulva
• Human papillomavirus (HPV) infection
• Cancer of the vagina or cervix
• Heavy cigarette smoking
• Chronic granulomatous disease (a hereditary disease that impairs the
immune system)
3
4. Pathogenesis
• Two primary mechanisms involved in the
carcinogenesis:
– Human papillomavirus (16, 18, and 33)
– Vulvar dystrophy, including lichen sclerosus (LS)
and squamous hyperplasia
• Usual-type VIN (uVIN) is often HPV driven and occurs
in younger women with HPV risk factors
• Differentiated-type VIN (dVIN) arises in the setting of
LS and other chronic inflammatory processes and is
associated with older age
4
5. Pathogenesis
• The differing precursor lesions may transform
into two distinct classifications of squamous
carcinoma:
Keratinizing squamous carcinomas (KSCs) arising
from dVIN
Basaloid squamous carcinomas (BSCs) arising
from uVIN
5
8. Distant Metastasis
• Hematogenous dissemination generally occurs
late in the natural history of the disease
• The most common sites are-
Lungs
Liver
Bones
• Overall survival is limited in these cases to a
median of approximately 6 months
8
16. Evaluation
• Clinical history
• Examination of the vagina, cervix, perianal skin,
and anal canal - to delineate the extent of disease
and to identify synchronous lesions
• Examination of inguinofemoral basins for clinical
detection of lymphatic spread
• Complete blood cell count
• Blood chemistry
• Biopsy
16
17. Imaging
Imaging in locally advanced disease –
• Cystoscopy
• CECT - suspicious lymphadenopathy in the inguinofemoral,
pelvis, or para-aortic regions
• MRI - to delineate the primary lesion and evaluation of
inguinal lymph node
• PET - to evaluate the groin prior to surgical evaluation
Sensitivities of all imaging modalities available are
insufficient to omit surgical evaluation in women with
a high risk of nodal involvement
17
23. Surgery
• In tumors clinically confined to the vulva or perineum, radical
local excision with a margin of at least 1 cm has replaced
radical vulvectomy
• Separate incision has replaced en bloc inguinal node
dissection
• Ipsilateral inguinal node dissection is preferred over bilateral
dissection for laterally localized tumors
• Lymph node dissection has been omitted in many cases by
employing Sentinel Lymph Node Biopsy
23
24. Surgery In Locally Advanced Cases
• Radical vulvectomy is reserved for patients with large
or multifocal lesions in whom preservation of normal
vulvar tissue is not possible or would not serve a
functional or reconstructive benefit
• When the anus, vagina, or urethra is involved by
malignancy, extended radical vulvectomy or pelvic
exenteration is required to clear the disease
surgically
24
26. Adjuvant Radiotherapy
• In the setting of early invasive disease treated with wide local
excision, radiation to the tumor bed may be advised to
prevent local recurrence
• Indications for adjuvant RT to the primary site include-
Lymphatic–vascular invasion (LVI)
Depth of invasion >5 mm
Margins <8 mm
Microscopically positive margins
26
27. Adjuvant RT to Inguinal and Pelvic Nodes
• All women with ECE of tumor in the nodes or with residual
disease in the inguinal areas should receive postoperative RT
to the pelvis and inguinal areas
• There are consensus to support adjuvant radiation to the
pelvis and both groins for all patients with nodal involvement
27
28. Dose
• The elective volume (grossly uninvolved nodal basins) can be
treated to 45 to 50.4 Gy
• When microscopic disease in the primary tumor area is
suspected, a dose of 55 Gy is recommended
• When there is ECE of tumor in the lymph nodes, the dose to
the groins can be carried up to 60 Gy
• If there is gross residual disease post surgery, the dose to the
area should be brought to a minimum of 65 to 70 Gy, a dose
used for definitive therapy.
28
29. Neo-Adjuvant Radiotherapy
• Given with the goals of decreasing the sequelae of radical
surgery and to maximize functional outcome
• After initial concurrent chemoradiation and healing of the
reaction, the response to the therapy at the primary site and
the lymph nodes is assessed
• If there is complete clinical regression of the disease,
resection may be omitted for negative biopsies from primary
site with groin dissection
29
30. Dose
• Dose to primary and gross nodal disease-
Upto 55.8 to 59.4 Gy with 1.8 Gy daily fraction
• Dose to elective nodal sites-
45 to 50.4 Gy with 1.8 Gy daily fraction
• With or without concurrent chemotherapy
• Surgery is performed for any residual disease 6 to
8 weeks post treatment
30
31. Definitive Radiotherapy
• In locally advanced disease with-
– Anorectal, urethral, or bladder involvement (in an effort to
avoid colostomy and urostomy)
– Disease that is fixed to the bone
– Gross inguinal or femoral node involvement (regardless of
whether a debulking lymphadenectomy was performed)
31
32. Dose :
• With appropriate field reductions, radiation dose should be brought
up to 60 to 70 Gy
• Total dose to certain areas is dependent upon location and extent
of the disease, response to therapy, and estimated tolerance of the
area requiring the high radiation dose
• Often, tolerance of normal tissue limits the dose to ≤65 Gy
• Chemotherapy should be continued throughout the entire course
of radiation for radiosensitization
Weekly Cisplatin @ 40 mg/m2
32
34. CT Simulation
• Oral contrast
• Bladder protocol
• Frog leg position in a vacuum-
evacuated device
• Hands over chest
• Fiducials
• IV contrast
• Customised bolus of 0.5 cm
thickness for the vulvar region at
simulation
• Images taken from L2 to mid thigh
with slice thickness of 3 mm
34
35. Contouring
Target Volume :
Entire Vulva / Post-op Bed
B/L Inguinofemoral nodes
Pelvic lymph nodes
Consensus recommendations for radiotherapy contouring and treatment
of vulvar carcinoma David K. Gaffney
35
36. GTV + CTV Primary
• GTV :
All gross disease on physical examination and imaging (see above
regarding the importance of PET-CT and MRI)
• CTV Primary :
GTV + 1 cm including the entire vulva; exclude uninvolved bone,
muscle, and adjacent organs
Include entire vagina if vaginal involvement and generate ITV for full
and empty bladder if feasible
Include 2 cm of urethra proximal to primary GTV for anterior
periurethral lesion or to the bladder neck if extensive urethral
involvement
Include a 1–2 cm margin if anal verge is involved or the entire
mesorectum if anal canal involvement
36
46. 3D-CRT Planning
• Solutions :
Wide anterior field, narrow posterior field, anterior electron
field
Narrow anterior and Narrow posterior field and anterior
electron field
Wide anterior, Narrow posterior, and anterior inguinal
photon fields
46
50. Challenges with 3DCRT technique
• Abutting photon-electron fields at the groin result in
significant hotspots and dose inhomogeneity at the match
line, which contribute to patient morbidity
• The depth of inguinal node necessitates the use of high
energy electron that result in severe skin reaction
• The irradiation of large volumes of normal tissue, especially
small bowel exposes patients to treatment related toxicities
and limits the total deliverable dose
50
51. IMRT
• Often used to treat the pelvis and inguinal nodes
• Careful quality assurance is required when using IMRT
• In particular, care should be placed to ensure that the surface
of the vulvar tumor receives adequate dose
• Virtual Flash –
During treatment planning, the PTV is expanded out of the skin by 1 to
2 cm, with additional placement of virtual tissue equivalent to allow
for dose buildup
This protects against underdosing the tumor surface if there is
displacement of the patient during treatment 51
52. IMRT
Dose constraints :
o Small bowel : V45 < 195 cc
o Rectum : V50 < 50%
o Bladder : Dmax < 65 Gy
o Femoral heads : <15 % to
receive > 35 Gy
52
54. • AP-PA fields with the patient in the supine position are
recommended for the delivery of external beam
• Depending on whether the inguinofemoral lymph nodes
and/or pelvic lymph nodes are to be included in the radiation
volume, different field configurations may be used
• Although there are no data regarding scar recurrences, it is
common practice to include the inguinal node dissection scars
in the radiation field
54
55. Superior Border-
middle of the sacroiliac joints to cover the external and internal iliac
nodes
If a patient has internal or external iliac node involvement, the
superior border should be extended to the L3/L4 interspace
Inferior Border –
Should cover the entire vulva and the most superficial, inferior
inguinal nodes
Lateral Border -
Posterior field : The pelvic field extends 2 cm laterally to the widest
point of the pelvic inlet
Anterior field : The pelvic field extend laterally to lateral edge of lesser
trochnter
55
57. Portal for irradiation of pelvic and inguinofemoral lymph nodes
and vulvar area
A final boost to the positive inguinal lymph nodes may be given
with a reduced field
57
59. Brachytherapy
• Brachytherapy has been used for inoperable vulvar cancer,
disease recurrence and as a boost to the primary tumor
• Advocated for primary vulvar cancer if the patient refuses
surgery or if surgery is contraindicated
• Because of the significant risk of necrosis, the use of
brachytherapy should be limited to very selected cases and
performed by experienced practitioners
59
60. Procedure
• Patient hospitalized a day before the procedure for parts and
bowel preparation
• Procedure performed under spinal or general anesthesia with
patient placed in a lithotomy position
• After thorough clinical examination under anesthesia and
mapping of disease, radioopaque silver markers are implanted
to assist delineation of tumor on planning imaging
• BT procedure involves insertion of plastic catheters or bevel-
edged stainless-steel hollow rigid needles into the vulvar
tissues with safety margin
60
61. Procedure
• Implantation techniques are guided by the Paris
system rules in terms of spacing and margins
accounting for the tumor size in all three
dimensions
• Usually, free-hand implant is done to treat the
superficial vulvar lesions while template-based
straight or curved needles are employed for
treatment of deep vulvar lesions extending into
vagina
61
64. • Organs at risk –
Urethra
Bladder
Rectum
• Dose –
Definitive :
o 3.5 to 4 Gy per fraction
o 10 to 13 fractions atleast 6 hours apart
o EQD2 of 30 to 40 Gy
Boost :
o 3.5 to 4 Gy per fraction
o 4 to 5 fractions atleast 6 hours apart
o EQD2 of 16 to 20 Gy
64
65. Sequelae of Treatment
ACUTE -
• Muco-cutaneous reaction
• Wound infection and hematoma
• Hematological toxicity
• Deep vein thrombosis
• Osteitis pubis
• Sensory loss in thigh (Femoral nerve injury)
65
66. Sequelae of Treatment
CHRONIC –
• Avascular vulvar necrosis
• Edema of lower extremeties
• Chronic cellulitis of inguinal area
• Introitus stenosis
• Recto-vaginal or recto-perineal fistula
• Psycho-sexual dysfunction
66