1. Management of Vulvar Cancer
Tevfik GUVENAL, Prof. Dr
Celal Bayar University, Manisa

2. Vulvar Cancer Vulvar cancer is a rare disease
Vulvar cancer accounts for 5% of all gynecologic malignancies
Incidence of 0.5–1.5 in 100,000 women
It primarily affect women older than 65 years
There is an increasing incidence, particularly among younger women (increased HPV-exposure.
De Sanjose S Eur. J. Cancer 2013
Siegel RL, Cancer J Clin. 2016
Howlader N,
Meltzer-Gunnes CJ, Gynecol Oncol 2017

3. Incidence Over the last five decades,
the incidence rate of vulvar SCC has increased >2.5 fold
Meltzer-Gunnes CJ, Gynecol Oncol 2017

4. Histolojik
doğrulama
Olgu sayısı

5. 2 Types / Variants
(15%)
(85%)

6. Histopathologic Types
Squamous Cell Carcinoma (85%)
Keratinizing SCC
80%, older, related to LS and other vulvar dystrophy
Basaloid SCC
20%, younger, HPV infection, other anogenital lesions, VIN, multifocality
Melanoma (10%)
Adenocarcinoma (5%)

7. Vulvar Adenocarcinoma

8. Vulvar Cancer Management
Standart treatment in the past: Radical vulvectomy and en bloc groin dissection

9. Vulvar Cancer Management
In last 30 years, a number of significant advances have made in the management of vulvar cancer, reflecting a paradigm shift toward a more conservative surgical approach without compromised survival with markedly decreased physical and psychological morbidity
Individualization of the treatment for all patients with invasive disease
Vulvar conservation for patient with unifocal tumors an otherwise normal vulva

10. The proportion of patients receiving surgery as the only
treatment decreased by 25% (from 81 to 61%).
The use of radiation alone and combination therapy
increased >3-fold and almost 3.5-fold, respectively
(from 3 to 11% and 6 to 20%, respectively)

11. (Int J Gynecol Cancer 2017;27: 832- 837)

12. Diagnosis and Referral
The localization of the primary tumor is important
SLN procedure: yes/no, expected unilateral or bilateral lymph drainage, visibility of scar, etc
In any patient suspected for vulvar cancer, diagnosis should be established by a punch/incision biopsy.
Excision biopsy should be avoided for initial diagnosis (may obstruct treatment planning)

13. Diagnosis and Referral
In patients with multiple vulvar lesions, all lesions should be biopsied separately (with clear documentation of mapping)
All patients with vulvar cancer should be referred to a gynecologic oncology center and treated by a multidisciplinary gynecologic oncology team

15. Ontogenetic Staging oT1 Vulvar subcompartment oT2 Vulvar compartment
oT3 Extraembriyonic ectosurface metacompartment
oT4 Ectosurface metacompartment
Michael Höckel

17. Preoperative Investigations Clinical examination
Size of lesion
Distance to the midline,
Palpation of the lymph nodes
Involvement of clitoris, anus/vagina/urethra
Evaluation of cervix
Picture or clinical drawing is advised

18. Preoperative Investigations (Imaging)
Ultrasound, CT or PET/CT, or MRI
Involvement groin and pelvic nodes
Distant metastases
Suspicious nodes (at palpation and/or imaging) should be analyzed by…
fine-needle aspiration or
core biopsy

19. Surgical Management Local Treatment
Wide or Radical local excision is recommended.
In multifocal invasive disease, radical excision of each lesion as a separate entity may be considered.
Vulvectomy may be required in cases with multifocal invasion arising on a background of extensive vulvar dermatosis.

21. Surgical Management Local Treatment
The goal of excision is to obtain tumour-free pathological margins.
Surgical excision margins of at least 1 cm are advised.
It is acceptable to consider narrower margins where the tumour lies close to midline structures (clitoris, urethra, anus)
When invasive disease extends to the pathological excision margins of the primary tumour, re-excision is the treatment of choice.

22. Surgical Management Groin Treatment
Groin treatment should be performed for tumours >pT1a.
no more than 1 mm stromal invasion and is 2 cm or smaller in size.
For unifocal tumours < 4 cm without suspicious groin nodes on clinical examination and imaging (any modality) the sentinel lymph node procedure is recommended.
For tumours ≥ 4 cm and/or in case of multifocal invasive disease, inguinofemoral lymphadenectomy by separate incisions is recommended.

23. SLN Procedure In SLN Procedure
Radioactive tracer is mandatory
blue dye is optional
Intraoperative evaluation and/or frozen sectioning of the SLN
Multiple sectioning (micrometastases)
Immunohistochemistry
When an SLN is not found (method failure), inguinofemoral lymphadenectomy should be performed

24. SLN Procedure
Where metastatic disease is identified in the SLN (any size)
inguinofemoral lymphadenectomy in the groin with the metastatic sentinel lymph node.
For tumors involving the midline, bilateral SLN detection is mandatory.
When only unilateral SLN detection is achieved, an inguinofemoral lymphadenectomy in the contralateral groin should be performed

25. Surgical Management (Groin Treatment)
In lateral tumours (medial border > 1 cm from midline), ipsilateral inguinofemoral lymphadenectomy is recommended.
Contralateral inguinofemoral lymphadenectomy may be performed when ipsilateral nodes show metastatic disease.
When lymphadenectomy is indicated, superficial and deep femoral nodes should be removed.
Preservation of the saphenous vein is recommended.
Where enlarged (> 2 cm) pelvic nodes are identified, their removal should be considered.

26. Surgical Management (Groin Treatment)
In enlarged groin nodes
inguinofemoral lymphadenectomy + RT
groin node debulking + RT
In enlarged pelvic nodes,
debulking of lymph nodes +RT
Radiotherapy alone will probably not sterilize large nodal pelvic disease.

27. Advanced stage vulva cancer
Multiple treatment modalities.
Treatment planning is often individualized..
primary tumor characteristics
presence of regional and/or distant metastases.
Comorbidity and/or frailty of the patient influences treatment planning.
A multidisciplinary setting is needed to optimize treatment planning.

30. Surgical margin Re-excision is preferred in case of positive margins
When possible without damaging structures such as anus, urethra, and clitoris,
Re-excision is not possible, postoperative radiotherapy should be performed
Addition of concomitant, radiosensitizing chemotherapy to adjuvant radiotherapy should be considered

32. Radiation Therapy
Alone has a little role in the primary management, generally indicated in conjuction with surgery
Pre-operatively: in advanced disease
Post-operatively: to treat the pelvic and groin lymph nodes and/or to prevent local recurrences in patients involved or close surgical margin
Adjuvant radiotherapy should start as soon as possible, preferably within 6 weeks of surgical treatment
Radiosensitizing chemotherapy, preferably with weekly cisplatin, is recommended

33. Bilateral groin dissection, RT if positive
Bilateral groin irradiation

34. Bilateral groin dissection, RT if positive
Bilateral groin irradiation

37. Treatment of Recurrent Disease
Local recurrences (vulva or groin)
Wide local excision and inguinofemoral lymphadenectomy (invasion of more than 1 mm and not previously performed groin dissection)
postoperative radiation in radiotherapy-naïve patients
Computed tomography of the thorax/abdomen or PET/CT thorax/abdomen
to examine the presence of additional metastases.

38. Recurrent Disease
Journal of Obstetrics and Gynaecology, 2016

39. Groin recurrence

40. Follow-up There is no evidence for best follow-up schedule.
Local recurrences may occur many years after primary treatment, lifelong follow-up is advised.
Patients with associated VIN or lichen sclerosus/planus have a higher risk of local recurrence, more intensive follow-up may be indicated.

41. Follow-up (After primary surgical treatment)
First follow-up 6 to 8 weeks postoperatively
First 2 years every 3 to 4 months
Third and fourth year biannually
Follow-up should include clinical examination of the vulva and groins
Data do not support routine use of imaging of the groins in follow-up

42. Follow-up (After definitive (chemo)radiation)
First follow-up visit 10 to 12 weeks after completion of definitive (chemo)radiation
First 2 years every 3 to 4 months
Third and fourth year biannually
At first follow-up visit 10 to 12 weeks after definitive (chemo)radiation, CT or PET/CT is recommended to document complete remission

43. Survival
Meltzer-Gunnes CJ, Gynecol Oncol 2017

44. Conclusions
The incidence of vulva cancer has increased in recent years
The treatment of vulval cancer is primarily by surgery.
The treatment of vulvar cancer has become more individualised and conservative (wide local excision)
A tumor-free surgical margin of at least 1 cm decreases the risk of local recurrence

45. Conclusions
Lymph node status is the most important determinant of survival
All tumors larger than 2 cm require pathologic inguinofemoral lymph node evaluation
When lymphadenectomy is indicated, superficial and deep femoral nodes should be removed.
Sentinel node biopsy is a reliable method
Chemoradiation is the treatment of choice in patients with unresectable disease.

46. BAYRAMIMIZ KUTLU OLSUN.