1. EMERGING TRENDS IN THE MANAGEMENT OF VULVAL CARCINOMA
Dr. PAULAMI GUHA, M.D

2. INCIDENCE 4th COMMON MALIGNANCY OF FEMALE GENITAL TRACT
Incidence = 3740 / year in USA
1.7 / 100,000 women

3. TYPES OF VULVAL CANCER TYPE PERCENT SQUAMOUS 90 - 92 MELANOMA 2 - 4
BASAL CELL
2 - 3
BARTHOLIN GLAND ( adenoca, squamous, transitional, adenoid cystic) 1
METASTATIC
VERRUCOUS
<1
SARCOMA
APPENDAGE (hidradeno ca)
Rare

4. SQUAMOUS CELL CARCINOMA
Most common type…….90-92%
Histologic &
environmental factors
Basaloid or warty
Keratinising

5. FIGO staging for vulvar cancer (1994)
Stage Carcinoma in situ, intraepithelial carcinoma
Stage I Tumour 2 cm in greatest diameter, confined to the
vulva or perineum; nodes are negative
IA As above with stromal invasion
1B As above with stromal invasion 1 mm
Stage II Tumour confined to the vulva and/or perineum, 2cm
in greatest dimension, nodes are negative
Stage III Tumour of any size with
1. Adjacent spread to the lower urethra and/or the vagina
and or anus.
2. Unilateral regional lymph node metastasis
Stage IVA Tumour invades any of the following: Upper urethra,
bladder mucosa, rectal mucosa, pelvic bone, or
bilateral regional node metastasis
Stage IVB Any distant metastasis including pelvic lymph nodes

6. DIAGNOSIS History Asymptomic Vulvar pruritus Vulval lump or mass
Less frequent symptoms:
Bleeding/ discharge from ulcerative lesion
Pain
Dysuria
Mass in the groin

7. DIAGNOSIS (contd…) PHYSICAL EXAMINATION On vulval inspection :
Mass – fleshy, ulcerated, leukoplakic or warty, pigmented, red or white
Ulcer – sloughing base with everted edges
Discharge
Site – labia majora & minora (60%),clitoris (15%), perineum(10%), multifocal (5%)

8. DIAGNOSIS (contd…) On palpation :
Lesion may be tender or painless
Bleeds to touch
Extent of the lesion
Surrounding tissue edematous or indurated
Careful examination of cervix, vagina, urethra & rectum to rule out co-existent primary lesions

9. DIAGNOSIS (contd…) Examination of inguinal lymph nodes
Lesion may be clinically indistinct esp. in presence of VIN or other vulval dystrophies
Confirmation of diagnosis by wedge biopsy of the growth.
Excisional biopsy if lesion is only 1 cm in diameter

10. PRE-TREATMENT INVESTIGATIONS
Routine :
Complete hemogram
Skiagram of chest
LFT etc.
Depending on the extent of the disease:
Cystoscopy
IVP
Proctoscopy
CT scan
MRI etc.

11. MANAGEMENT
Available modalities:
Surgery
Radiotherapy
Chemotherapy

12. MANAGEMENT Pioneering work by Taussig & Way in 1940
En bloc radical vulvectomy & bilateral dissection of inguino-femoral & pelvic nodes became the standard treatment
5- year survival rate improved from % to 60 – 70 %
BUT
Several factors led to modifications …..

13. MANAGEMENT (contd…)
Concerns regarding increased post operative morbidity
Increasing proportion of patients presenting with early stage disease
Increased awareness about the psychosexual consequences of radical vulvectomy

14. MANAGEMENT (contd…)
Hence individualisation of treatment is needed to determine appropriate therapy

15. MANAGEMENT (contd…) MANAGEMENT OF PRIMARY LESION
EARLY VULVAL CANCER ( T1 )
Traditional treatment was en bloc radical vulvectomy with bilateral lymph node dissection
Disadvantages :
Wound breakdown rate upto 85%
Severe disfigurement & distortion of body image

18. MANAGEMENT (contd…)
Triple incision technique developed to overcome these shortcomings
Described by Byron et al in 1965 but
Gained popularity only after Hacker et al

20. MANAGEMENT (contd…) Radical local excision
Main concern – risk of local recurrence
Microscopic metastasis occurs in close vicinity of macroscopic tumor
Hence adequate excision margin of at least 1 cm to 2 cm is required

21. MANAGEMENT (contd…)
Most appropriate for lesions on the lateral or posterior aspects of vulva
Variety of local excisions reported: hemivulvectomy, anterior or posterior vulvectomy etc.
Midline lesions pose challenges
Small periclitoral lesions in a limited no. of young pts may be treated with a small field of radiation (5000cGy).

22. MANAGEMENT (contd…) T2 &EARLY T3 TUMORS
Radical local excision with at least 1 cm margin suitable in selected patients with lesions in posterior half of vulva
Majority of patients, however, need radical vulvectomy with bilateral inguino-femoral lymphadenectomy

23. MANAGEMENT (contd…) ADVANCED VULVAL CANCER (LARGE T3 & T4 TUMORS )
Radical vulvectomy & inguino-femoral lymphadenectomy with some form of pelvic exenteration required for primary surgical clearance
Combined radiation & surgical therapy is the preferred approach

24. MANAGEMENT (contd…)
Pre-operative external beam radiotherapy with selective brachytherapy usually with concurrent chemotherapy
cGy delivered to whole pelvis, groin & vulva
Surgery performed after 2-6 weeks in a more limited fashion
5-year survival rate as high as 76% with improved quality of life

25. MANAGEMENT (contd…) MANAGEMENT OF LYMPH NODES
Single most important prognostic factor is lymph node status
Only patients with virtually no risk of LN metastasis – stage Ia ( stromal invasion < 1mm)
Patients who develop recurrent disease in an undissected groin have > 90 % mortality

26. MANAGEMENT OF LYMPH NODES
Microinvasive carcinoma
Groin dissection not required if stromal invasion < 1 mm , no lymph-vascular space invasion & no clinically suspicious groin lymph nodes

27. MANAGEMENT OF LYMPH NODES
Radical inguino-femoral lymphadenectomy recommended for all stages beyond Ia
Post-operative radiotherapy given to the groin & pelvis if there is one grossly positive or 2 or more microscopically positive inguinal lymph nodes
Dose – 4000 – 5000 cGy in daily 200 cGy fractions

28. UNILATERAL vs BILATERAL GROIN DISSECTION
Unilateral groin dissection adequate in case of
Well-lateralised primary lesion (T1)
Negative ipsilateral inguinal lymph nodes
Risk of contralateral LN metastasis is only 0.4%

29. UNILATERAL vs BILATERAL GROIN DISSECTION
Bilateral groin dissection is indicated in patients with :
Bulky ipsilateral groin LNs
Multiple microscopically positive ipsilateral groin LNs
Midline lesions ( clitoris,anterior labia minora, fourchette, vagina )
Lesions within 2 cm of midline

30. SUPERFICIAL INGUINAL LYMPHADENECTOMY
Only % of stage I & II patients have positive inguino-femoral nodes
So a recent trend towards less radical groin dissection
Unilateral lymphadenectomy & removal of nodes superficial to cribriform fascia ( Di Saia et al ,1979)
If nodes found positive on frozen section then complete bilateral inguino-femoral LND performed

31. MANAGEMENT OF PELVIC LNs
In the past pelvic lymphadenectomy was a routine procedure
But overall incidence of :
Inguino-femoral LN metastasis – 30%
Pelvic LN metastasis %
Pelvic LN metastasis in presence of :
Clinically suspicious groin nodes -33%
3 or more pathologically +ve groin nodes -50%

32. MANAGEMENT OF PELVIC LNs
In the absence of groin node involvement , pelvic nodal metastasis is rare – 0.6%
So a selective approach is preferred
Patients at risk of pelvic nodal metastasis are given postoperative radiotherapy
Improved survival compared to those undergoing pelvic lymphadenectomy

33. SENTINEL LYMPH NODE STUDIES
Based on the fact that superficial inguinal LNs act as sentinel group so that skip lesions to deep nodes are rare
Intra-operative lymphatic mapping using lymphoscintigraphy with technetium 99m labelled nanocolloid or isosulfan blue dye
Unnecessary extensive lymphadenectomy may be avoided in those with –ve sentinel node
Currently under trial

34. POST-OPERATIVE MANAGEMENT
Early ambulation
Suction drainage of each side of groin continued till output is minimal
Frequent dressing changes to keep the vulvar wound dry
Meticulous perineal hygiene
Proper antibiotic coverage
Foley´s catheter removed once patient is ambulatory

35. POST-OPERATIVE COMPLICATIONS
EARLY
Groin wound infection & breakdown
En bloc technique -85%
Triple incision technique – 44% , major breakdown in 14%
UTI
Seromas of femoral triangle
Deep vein thrombosis

36. POST-OPERATIVE COMPLICATIONS
Pulmonary embolism MI
Anaesthesia of anterior thigh from femoral nerve injury
Osteitis pubis, rarely

37. POST-OPERATIVE COMPLICATIONS
LATE
Chronic lymphedema of legs (30%)
Recurrent lymphangitis or cellulitis of leg (10%)
Urinary stress incontinence with / without genital prolapse (10%)
Dyspareunia
Sexual dysfunction, altered body image
Femoral hernia
Pubic osteomyelitis
rectovaginal/ /rectoperineal fistula

38. ROLE OF RADIATION THERAPY
Treatment of vulval carcinoma is primarily surgical
Radiation therapy frequently with concurrent chemotherapy is gaining an increasingly important role
Generally indicated in conjunction with surgery

39. ROLE OF RADIATION THERAPY
INDICATIONS
Pre-operatively, in patients with advanced disease to shrink the tumor who would otherwise require pelvic exenteration
Post-operatively, to treat the pelvic LNs & groin of patients with 2 or more microscopically positive or one grossly positive groin node
May be used as primary therapy in technically inoperable or unresectable cases of advanced vulval carcinoma

40. ROLE OF RADIATION THERAPY
Possible role :
Post-operatively to help prevent local recurrence in patients with involved or close surgical margins
As primary therapy for patients with small primary tumors particularly clitoral or periclitoral lesions in young women

41. ROLE OF CHEMOTHERAPY Very limited, only palliative
Usually in conjunction with radiotherapy as radiation sensitiser
Chemoradiation may have an important role in sphincter preserving surgery in advanced vulval carcinoma
Agents used – 5FU, cisplatin, bleomycin, doxorubicin & mitomycin C

42. PROGNOSIS & SURVIVAL 5-YEAR SURVIVAL RATE Negative nodes 90%
LYMPH NODE STATUS
5-YEAR SURVIVAL RATE
Negative nodes
90%
Positive inguino-femoral nodes
50%
Positive pelvic nodes
11%
Single most important prognostic factor is lymph node status
Patients with 3 or more positive nodes have a poor prognosis

43. PROGNOSIS & SURVIVAL Other prognostic factors : Histologic grade
Tumor thickness
Depth of stromal invasion
Lymph vascular space invasion
Tumor ploidy status

44. PROGNOSIS & SURVIVAL FIGO STAGE CORRECTED 5-YEAR SURVIVAL RATE STAGE I
98%
STAGE II
85%
STAGE III
74%
STAGE IV
31%
FIGO S

45. RECURRENT VULVAL CANCER
Two-thirds of recurrences within first 2 years from initial therapy
Groin recurrences occur sooner than vulvar recurrences
Patients with 3 or more positive nodes are at increased risk

46. RECURRENT VULVAL CANCER
LOCAL RECURRENCE
Margin status at the time of radical resection is the most powerful predictor of local recurrence
When detected early, isolated local recurrence is usually salvageable by surgery
Other options : radiation therapy often with concurrent chemotherapy

47. RECURRENT VULVAL CANCER
REGIONAL OR DISTANT RECURRENCE
Poor prognosis
Groin recurrence – radiation therapy with surgery
Distant recurrence – chemotherapy with
bleomycin,methotrexate,cisplatin,mitomycin C

48. MANAGEMENT OF RARE TYPES OF VULVAL CANCER
MELANOMA
Clinical features :
Asymptomatic – majority
Pigmented lesion
Itching
Bleeding
Groin mass
Diagnosis confirmed by biopsy & microstaging (Breslow) done according to tumor thickness

49. MANAGEMENT OF RARE TYPES OF VULVAL CANCER
For lesions < 1 mm invasion – radical local excision
For more invasive lesions – en bloc resection of primary tumor with inguino-femoral lymphadenectomy
Other modalities:
Radiotherapy – palliative
Chemotherapy – dacarbazine most active
Immunotherapy – interferon alpha

50. MANAGEMENT OF RARE TYPES OF VULVAL CANCER
BARTHOLIN´S GLAND CARCINOMA
Radical local excision / hemivulvectomy / radical vulvectomy with bilateral groin dissection
Post-operative radiotherapy if nodes are positive
VERRUCOUS CARCINOMA
Radical local excision is the basic treatment
If nodes found positive by FNAC, radical vulvectomy with inguino-femoral lymphadenectomy

51. MANAGEMENT OF RARE TYPES OF VULVAL CANCER
BASAL CELL CARCINOMA
Radical local excision is usually adequate

52. CONCLUSION
Early cancer of the vulva is now managed by less radical surgery.
Treatment of advanced tumours requires a multimodality approach.
Appropriate treatment requires an understanding of tumour biology and growth, and vulvar and pelvic anatomy,and relevant oncological consultation where indicated.

53. Thank You !