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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
vulvalcancer-final-190121175026.pdf

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vulvalcancer-final-190121175026.pdf

  • 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