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Dr Antima Rathore
Incidence
 0.6 % of all malignancies
 4 % of genital malignancies
 Squamous cell carcinoma – 90 %
Types of Vulvar cancer
Anatomy of the vulva
Lymphatic drainage of vulva
Lymphatic drainage of the vulva
Applied Anatomy
 Single most prognostic marker – L N status
 5 yr survival rate
No LN - 90%
LN - 50%
 Superficial Inguinal Lymph node – Sentinal Lymph
Node
Etiology
 HPV
 VIN
 CIN
 Lichen sclerosis
 Squamous hyperplasia
 Immunodeficiency
 History of genital warts
 Smoking
 Alcohol
 Immunosuppression
 H/O Cervical or Vaginal cancer
 Northern Europe ancestry
Squamous cell cancer
90 %
Basaliod
 Multifocal
 Younger pt
 HPV
 VIN
 Smoking
Keratinising
 Unifocal
 Older patient
 Lichen Sclerosis
 Sq. Hyperplasia
(>80% cases)
Itch Scratch Cycle
Clinical features
 Postmenopausal female
(Mean Age – 65 yrs)
 VIN
 Vulval Pruritis
 Lump or mass
 Rare – bleeding/ulcerative lesions, discharge
 Pain or dysuria
 Large metastatic mass in groin
 Other malignancies – HPV and smoking associated
Diagnosis
 Any vulval lesion warrants biopsy
 Punch biopsy
 Wedge biopsy
(include dermis)
 Colposcopy – cervix and vagina
 Labia majora & minora – 60%
 Clitoris – 15%
 Perineum – 10%
 Multifocal – 5%
 Extensive – 10%
Routes of Spread
 Direct extension
 Lymphatic
 Hematogenous
Staging
IA Tumor confined to the vulva or perineum, ≤ 2cm in size
with stromal invasion ≤ 1mm, negative nodes
IB Tumor confined to the vulva or perineum, > 2cm in size or with
stromal invasion > 1mm, negative nodes
II Tumor of any size with adjacent spread (1/3 lower
urethra, 1/3 lower vagina, anus), negative nodes
Staging
IIIA Tumor of any size with positive inguino-femoral lymph
nodes
(i) 1 lymph node metastasis ≥ 5 mm
(ii) 1-2 lymph node metastasis(es) < 5 mm
IIIB (i) 2 or more lymph nodes metastases ≥ 5 mm
(ii) 3 or more lymph nodes metastases < 5 mm
IIIC Positive node(s) with extracapsular spread
Staging
IVA (i) Tumor invades other regional structures (2/3
upper urethra, 2/3 upper vagina), bladder mucosa, rectal
mucosa, or fixed to pelvic bone
(ii) Fixed or ulcerated inguino-femoral lymph nodes
IVB Any distant metastasis including pelvic lymph
nodes
Prognosis
 Lymph nodes status
 Lesion size
 Histologic grade, tumor thickness, depth of stromal invasion, lymph-
vascular space involvement, tumor ploidy
 Stage I – 79%
 Stage II – 59%
 Stage III – 43%
 Stage IV – 13%
Treatment
 Table 33.4
부산백병원 산부인과
 Stage Ia – Microinvasive T1a
Wide Local excision – deep upto dermis
 Stage Ib & II – Early vulval cancer
Radical local excision plus Ipsilateral groin node
dissection
- 1 cm negative margin
- Extending up to inferior fascia of urogenital diaphragm
- Separate incision technique
Treatment
Treatment
 Midline lesions
Anterior – clitoris sparing surgery – 8mm margin
 Periclitoral lesion in young pt – small field RT with
concomitant chemosensitization
 small lesion – 5000 cGy external radiation
f/b biopsy
Treatment
 Stage II involving adjacent srtucture
Radical vulvectomy or radical local excision Plus
LN Dissection
 Advanced disease
Surgery plus RT
plus
concomitant chemo
Treatment
 Clinicaly advanced nodes – debulking of enlarged nodes
&/or chemoradiation
 Metastatic disease - palliation
부산백병원 산부인과
부산백병원 산부인과
부산백병원 산부인과
Closure of large defects
 Small defects – primary closure without tension
 Large – left open to granulate
 Full thickness skin flap – rhomboid or mons pubis flap
 Myocutaneous flap – gracialis
 Tensior fascia lata myocutaneous graft
Management of LN
 > 2 cm diameter
 > 1 mm invasion
 Surgery – trt of choice
 Bilateral dissection - midline lesions, clitoris, post forchet
- unilateral bulky LN / multiple microscopic
LN
 Bulky LN – debulking
 Fixed unresectable LN - chemoradiation
Sentinal Lymph node biopsy
Criteria
1) unifocal primary tumour of 4 cm or less in diameter with >
1 mm invasion
2) no obvious metastatic disease on physical
examination/imaging
Postop management
 Ambulation on day 1 or 2
 DVT prevention
 Subcutaneous heparin
 pneumatic calf compression
 Frequent dressing
 Suction drainage of each side of the groin
 Sitz bath
Early Postoperative Complications
 Groin wound infection, necrosis, breakdown
 En bloc operation – 53-85%
 Separate-incision approach – 44%
 UTI
 Lymphocyst
 DVT
 Pulmonary embolism
 MI
 Hemorrhage
Late Complications
 Chronic lymphedema
 Recurrent lymphagitis or cellulitis
 Usually responds to oral antibiotics
 SUI
 Introital stenosis
 Femoral hernia (uncommon)
 Depression, altered body image and sexual dysfunction
 Pubic osteomylitis
 Fistula
Recurrent Vulvar cancer
 ≥ 3 LN - 2/3 of vulvar cancer recur within first 2 years from initial
Tx.
 Local recurrence
 Margin status
 Closer than 0.8cm -> 50% recur
 Primary lesion larger than 4cm in diameter
 Ipsilateral lymphovascular space invasion
 Deep invasive tumour
 Tx.
 Additional surgery with myocutaneous graft
 External beam therapy + interstitial needles
with chemotherapy
Regional and Distant Recurrence
 Difficult
 Poor prognosis
 Radiation
 Chemotherapy
 Bleomycin and methotrexate & lomustine
 Bleomycin and mitomycin C
 Cisplatin, vincristine & paclitaxel
 Response
 Usually disappointing
 Long-term survival is very uncommon
Role of Radiation Therapy
 primary vulval ca.
 Advanced disease
 LN meta – microscopic, gross
 Possible roles for RTx.
 Involved or close surgical margin
 Small primary tumor - primary Tx.,
 Particularly clitoral or periclitoral lesion
Melanoma
 Rare
 Incidence : 0.1-0.19/100,000women
 Second most common of vulvar malignancy
 Postmenopausal white women
 No symptoms (most)
 Itching, bleeding, groin mass
 Labia minora, clitoris
 Vulvar nevi are junctional, precursor lesion to melanoma; thus,
should be removed
Histopathology
 Mucosal lentiginous melanoma
 Flat freckle, quite extensive, superficial
 Superficial spreading melanoma
 Most common, superficial
 Nodular melanoma
 Most aggressive, raised lesion
 Penetrate deeply
 Metastasize widely
 ¼ of cases of melanomas
 Macroscopically amelanotic -> spread early
Staging
Treatment
 More conservative surgical management
 Invasion
 (<1mm) : Radical local excision alone
 (>1mm) : en bloc resection of the primary tumor and
regional groin node dissection recommended
 1cm surgical margin (<0.76mm)
 2cm surgical margin (1-4mm)
Treatment
 10-year survival rate
 Lateral lesion (61%), medial lesion (37%)
 Superfical lesion (Breslow tumor thickness <0.76mm )
 Lymphadenectomy not indicated
 Intermediate-thickness (1-4mm)
 Observation showed a 5-year survival advantage who
underwent lymph node dissection
 Deeply invasive cutaneous melanoma (>4mm)
 Benefit from regional lymphadenectomy
 Chemotherapy : interferon – α , dacarbazine
Bartholin Gland Carcinoma
 Rare
 Postmenopausal
 Premenopausal
 Honan’s criteria
 The tumor is in the correct anatomic position
 The tumor is located deep in the labium majus
 The overlying skin is intact
 There is some recognizable normal gland present
Bartholin Gland Carcinoma
 Signs and Symptoms
 Vulvar mass or perineal pain
 10% of patients may be mistaken for benign cysts or
abscesses
 Treatment
 Radical vulvectomy with bilateral groin and pelvic LN
dissection
 Fixed, involves adjacent structures-> postop radiation
and chemotherapy is preferable
Bartholin Gland Carcinoma
 Adenoid Cystic Carcinoma of Bartholin Gland
 Other Adenocarcinomas
 Adenosquamous Carcinoma
 Basal cell carcinoma
 Verrucous Carcinoma
 Vulvar Sarcoma
Rare Vulvar Malignancies
 Lymphomas
 Endodermal Sinus Tumor
 Merkel Cell Carcinoma
 Dermatofibrosarcoma Protuberans
 Metastatic Tumors of the Vulva
Vulval cancer   final

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Vulval cancer final

  • 2. Incidence  0.6 % of all malignancies  4 % of genital malignancies  Squamous cell carcinoma – 90 %
  • 7. Applied Anatomy  Single most prognostic marker – L N status  5 yr survival rate No LN - 90% LN - 50%  Superficial Inguinal Lymph node – Sentinal Lymph Node
  • 8.
  • 9. Etiology  HPV  VIN  CIN  Lichen sclerosis  Squamous hyperplasia  Immunodeficiency  History of genital warts  Smoking  Alcohol  Immunosuppression  H/O Cervical or Vaginal cancer  Northern Europe ancestry
  • 10. Squamous cell cancer 90 % Basaliod  Multifocal  Younger pt  HPV  VIN  Smoking Keratinising  Unifocal  Older patient  Lichen Sclerosis  Sq. Hyperplasia (>80% cases) Itch Scratch Cycle
  • 11. Clinical features  Postmenopausal female (Mean Age – 65 yrs)  VIN  Vulval Pruritis  Lump or mass  Rare – bleeding/ulcerative lesions, discharge  Pain or dysuria  Large metastatic mass in groin  Other malignancies – HPV and smoking associated
  • 12. Diagnosis  Any vulval lesion warrants biopsy  Punch biopsy  Wedge biopsy (include dermis)  Colposcopy – cervix and vagina  Labia majora & minora – 60%  Clitoris – 15%  Perineum – 10%  Multifocal – 5%  Extensive – 10%
  • 13. Routes of Spread  Direct extension  Lymphatic  Hematogenous
  • 14. Staging IA Tumor confined to the vulva or perineum, ≤ 2cm in size with stromal invasion ≤ 1mm, negative nodes IB Tumor confined to the vulva or perineum, > 2cm in size or with stromal invasion > 1mm, negative nodes II Tumor of any size with adjacent spread (1/3 lower urethra, 1/3 lower vagina, anus), negative nodes
  • 15. Staging IIIA Tumor of any size with positive inguino-femoral lymph nodes (i) 1 lymph node metastasis ≥ 5 mm (ii) 1-2 lymph node metastasis(es) < 5 mm IIIB (i) 2 or more lymph nodes metastases ≥ 5 mm (ii) 3 or more lymph nodes metastases < 5 mm IIIC Positive node(s) with extracapsular spread
  • 16. Staging IVA (i) Tumor invades other regional structures (2/3 upper urethra, 2/3 upper vagina), bladder mucosa, rectal mucosa, or fixed to pelvic bone (ii) Fixed or ulcerated inguino-femoral lymph nodes IVB Any distant metastasis including pelvic lymph nodes
  • 17. Prognosis  Lymph nodes status  Lesion size  Histologic grade, tumor thickness, depth of stromal invasion, lymph- vascular space involvement, tumor ploidy  Stage I – 79%  Stage II – 59%  Stage III – 43%  Stage IV – 13%
  • 20.
  • 21.  Stage Ia – Microinvasive T1a Wide Local excision – deep upto dermis  Stage Ib & II – Early vulval cancer Radical local excision plus Ipsilateral groin node dissection - 1 cm negative margin - Extending up to inferior fascia of urogenital diaphragm - Separate incision technique Treatment
  • 22. Treatment  Midline lesions Anterior – clitoris sparing surgery – 8mm margin  Periclitoral lesion in young pt – small field RT with concomitant chemosensitization  small lesion – 5000 cGy external radiation f/b biopsy
  • 23. Treatment  Stage II involving adjacent srtucture Radical vulvectomy or radical local excision Plus LN Dissection  Advanced disease Surgery plus RT plus concomitant chemo
  • 24. Treatment  Clinicaly advanced nodes – debulking of enlarged nodes &/or chemoradiation  Metastatic disease - palliation
  • 28. Closure of large defects  Small defects – primary closure without tension  Large – left open to granulate  Full thickness skin flap – rhomboid or mons pubis flap  Myocutaneous flap – gracialis  Tensior fascia lata myocutaneous graft
  • 29. Management of LN  > 2 cm diameter  > 1 mm invasion  Surgery – trt of choice  Bilateral dissection - midline lesions, clitoris, post forchet - unilateral bulky LN / multiple microscopic LN  Bulky LN – debulking  Fixed unresectable LN - chemoradiation
  • 30. Sentinal Lymph node biopsy Criteria 1) unifocal primary tumour of 4 cm or less in diameter with > 1 mm invasion 2) no obvious metastatic disease on physical examination/imaging
  • 31. Postop management  Ambulation on day 1 or 2  DVT prevention  Subcutaneous heparin  pneumatic calf compression  Frequent dressing  Suction drainage of each side of the groin  Sitz bath
  • 32. Early Postoperative Complications  Groin wound infection, necrosis, breakdown  En bloc operation – 53-85%  Separate-incision approach – 44%  UTI  Lymphocyst  DVT  Pulmonary embolism  MI  Hemorrhage
  • 33. Late Complications  Chronic lymphedema  Recurrent lymphagitis or cellulitis  Usually responds to oral antibiotics  SUI  Introital stenosis  Femoral hernia (uncommon)  Depression, altered body image and sexual dysfunction  Pubic osteomylitis  Fistula
  • 34. Recurrent Vulvar cancer  ≥ 3 LN - 2/3 of vulvar cancer recur within first 2 years from initial Tx.  Local recurrence  Margin status  Closer than 0.8cm -> 50% recur  Primary lesion larger than 4cm in diameter  Ipsilateral lymphovascular space invasion  Deep invasive tumour  Tx.  Additional surgery with myocutaneous graft  External beam therapy + interstitial needles with chemotherapy
  • 35. Regional and Distant Recurrence  Difficult  Poor prognosis  Radiation  Chemotherapy  Bleomycin and methotrexate & lomustine  Bleomycin and mitomycin C  Cisplatin, vincristine & paclitaxel  Response  Usually disappointing  Long-term survival is very uncommon
  • 36. Role of Radiation Therapy  primary vulval ca.  Advanced disease  LN meta – microscopic, gross  Possible roles for RTx.  Involved or close surgical margin  Small primary tumor - primary Tx.,  Particularly clitoral or periclitoral lesion
  • 37. Melanoma  Rare  Incidence : 0.1-0.19/100,000women  Second most common of vulvar malignancy  Postmenopausal white women  No symptoms (most)  Itching, bleeding, groin mass  Labia minora, clitoris  Vulvar nevi are junctional, precursor lesion to melanoma; thus, should be removed
  • 38. Histopathology  Mucosal lentiginous melanoma  Flat freckle, quite extensive, superficial  Superficial spreading melanoma  Most common, superficial  Nodular melanoma  Most aggressive, raised lesion  Penetrate deeply  Metastasize widely  ¼ of cases of melanomas  Macroscopically amelanotic -> spread early
  • 40. Treatment  More conservative surgical management  Invasion  (<1mm) : Radical local excision alone  (>1mm) : en bloc resection of the primary tumor and regional groin node dissection recommended  1cm surgical margin (<0.76mm)  2cm surgical margin (1-4mm)
  • 41. Treatment  10-year survival rate  Lateral lesion (61%), medial lesion (37%)  Superfical lesion (Breslow tumor thickness <0.76mm )  Lymphadenectomy not indicated  Intermediate-thickness (1-4mm)  Observation showed a 5-year survival advantage who underwent lymph node dissection  Deeply invasive cutaneous melanoma (>4mm)  Benefit from regional lymphadenectomy  Chemotherapy : interferon – α , dacarbazine
  • 42. Bartholin Gland Carcinoma  Rare  Postmenopausal  Premenopausal  Honan’s criteria  The tumor is in the correct anatomic position  The tumor is located deep in the labium majus  The overlying skin is intact  There is some recognizable normal gland present
  • 43. Bartholin Gland Carcinoma  Signs and Symptoms  Vulvar mass or perineal pain  10% of patients may be mistaken for benign cysts or abscesses  Treatment  Radical vulvectomy with bilateral groin and pelvic LN dissection  Fixed, involves adjacent structures-> postop radiation and chemotherapy is preferable
  • 44. Bartholin Gland Carcinoma  Adenoid Cystic Carcinoma of Bartholin Gland  Other Adenocarcinomas  Adenosquamous Carcinoma  Basal cell carcinoma  Verrucous Carcinoma  Vulvar Sarcoma
  • 45. Rare Vulvar Malignancies  Lymphomas  Endodermal Sinus Tumor  Merkel Cell Carcinoma  Dermatofibrosarcoma Protuberans  Metastatic Tumors of the Vulva

Editor's Notes

  1. The FIGO staging used for squamous lesions is not applicable to melanomas Because the lesions are usually much smaller, and the prognosis is related to the depth of tumor invasion rather than to the diameter of the lesion