Management Of Ca Vulva 2

MANAGEMENT OF CARCINOMA OF THE VULVA BY DR. D.O ALLAGOA CLINICAL FEATURES INVESTIGATIONS TREATMENT COMPLICATIONS CONCLUSION

CLINICAL FEATURES Patients usually > 60 years old, may be younger Small lesions may be asymptomatic Most patients present late with 1/3 of the women presenting with stage III or IV disease. Patients present an average of 6-12 months + from onset of symptoms. They present late due to embarrassment, fear or ignorance. Delay in diagnosis may be due to  the non-specific nature of symptoms,  rarity of vulvar Ca compared with nonmalignant lesions  delay in clinical examination by primary carers due to other medical problems the patients may have

PRESENTING SYMPTOMS Pruritus 71% Vulvar lump or swelling 58% Vulvar ulceration 28% (Typical raised ulcer with rolled edges) Bleeding 26% Pain or Soreness 23% Urinary tract symptoms 14% Discharge 13%

PRESENTING SYMPTOMS The lesions may be: -Ulcerated. -Raised. -Pigmented. - warty appearance. IN ORDER OF APPEARANCE the lesions appear mainly on ; - labia majora. (67%) - labia minora. - clitoris. - perineum. - posterior fourchette. The definitive diagnosis depends on biopsy under anaesthesia.

INVESTIGATIONS • • • •

Confirmation of diagnosis Staging the disease Assess the patients fitness Assess the possibility of concurrent disease that might influence management.

INVESTIGATIONS TO CONFIRM DIAGNOSIS/STAGE THE DISEASE  Incisional biopsy  Excisional biopsy (for local lesions)  Colposcopy is useful for diagnosis of smaller

superficial lesions which are clinically difficult to recognize. Colposcopy helps map out areas of invasive disease & plan management. -5% Acetic acid will help to identify areas to biopsy although subtle changes may be difficult to recognize -Toluidine blue can also be used. Cystoscopy/Proctoscopy (bladder/rectal mucosa involvement)

INVESTIGATIONS TO STAGE THE DISEASE (CONT.) Accurate assessment of inguinofemoral lymph node status is the most important feature for staging and management planning. There are limitations to clinical assessment of nodal status. It is notoriously unreliable. Clinical suspicion of nodal involvement may be evaluated with  Fine-needle aspiration – • –ve result does not exclude need for node dissection. • +ve result may prompt neoadjuvant radiation

Lymphangiography Ultrasonography (including Doppler flow study) Magnetic resonance imaging (MRI) Computed tomography (CT) Combination of imaging techniques & fine-needle aspiration improves sensitivity

INVESTIGATIONS TO ASSESS FITNESS & POSSIBLE CONCURRENT DISEASE Full blood count Urinalysis Serum electrolytes/urea Chest radiograph, Intravenous Urography ECG Assessment and possible biopsy of vagina & cervix to detect co-existing VAIN & CIN

Investigation- cont Vulvoscopy Cystoscopy Proctoscopy Pyelography Barium enema

TREATMENT It is a rare tumour hence current guidelines recommend that it is managed in a cancer centre within the context of a multidisciplinary team of experts led by a specialist gynaecological oncologist -SURGERY -CHEMOTHERAPY Neoadjuvant chemotherapy -RADIOTHERAPY

SURGICAL MANAGEMENT The objective of surgery is to remove sufficient tissue to prevent local recurrence as well as excise groin nodes both to stage the disease and to prevent groin recurrence Traditional surgery has been a large en bloc resection of the vulva with the superficial and deep inguinal nodes through a single incision with at least 2-cm margins around the tumor and deep resection to the genitourinary diaphragm The morbidity associated with radical vulvectomy has led to changes in management

REFINEMENTS TO SURGERY (2) Ipsilateral inguinal node dissection in early tumors (3) Using a triple-incision technique instead of an en bloc (4) (5) (6) (7) (8)

approach Using radical local resection with 1-cm margins instead of complete vulvectomy Sparing the saphenous vein in an attempt to prevent lymphedema Omitting deep node dissection Radiation alone for nodal involvement Vs groin node dissection Sentinel lymph node dissection

PRINCIPLES OF SURGERY Maximum individualisation Cautious conservation Restoration by plastic reconstruction Choice of surgery will depend on Age & general state of the patient Size, site and stage of the lesion Histology of the lesion Clinical status & histology of groin lymph nodes Condition of uninvolved vulvar/anal skin

Disease categorization EARLY DISEASE - Small unifocal lesions with no clinical evidence of nodal involvement LATE DISEASE -Advanced vulvar disease and/ or clinical evidence of nodal involvement

SURGERY Wide local excision for Stage I & II Hemivulvectomy & ipsilateral lymph node excision for lateral lesions. - if lesion 2cm or less- ipsilateral lymph adenectomy is appropriate -contralateral dissection done once ipsilateral node is positive. -lesion > 2cm bilateral node dissection is advised Radical vulvectomy - triple incision or enbloc dissection for midline lesions Pelvic exenteration for advanced cancer.(dehumanising)

Radical vulvectomy En bloc significant morbidity Triple incision - better cosmetic outcome -better survival rate - reduced local relapse -improvement in morbidity

GROIN NODE STATUS Groin node negative – no further treatment Groin nodes positive after surgery - one node only involved- observation only - two or more nodes involved inguinal and pelvic radiation - nodes clinically positive before surgery: resection followed by irradiation or irradiation ffed by resection or radiation only

Role of plastic surgery Can be done primarily or as a second stage procedure. - insertion of bilateral myocutaneous tensor fascia lata flap - rectus abdominis myocutaneous flap - gracilis muscle myocutaneous flap - rotational full thickness skin flap taken from the inner thigh or buttocks

POST-OPERATIVE CARE Frequent sitz baths. Patients should dry the vulva completely after each sitz bath. A Foley catheter may be needed for a prolonged period after surgery around the urethra. Early mobilization Heparin or pneumatic compression stockings should be used in all women to prevent postoperative venous thrombosis. Place drains at the time of lymphadenectomy because of the flow through the groin lymphatics. Leave these drains in place until drainage is approximately 25 mL or less per day. In many cases, this may take more than 2 weeks. Antibiotics /analgesics Hospitalization : 17-33 days on the average

RADIOTHERAPY

-External beam megavoltage

-brachytherapy INDICATIONS Primary tumors -squamous vulva cancer -basal cell carcinoma N.B: verrucous cancers (worsened), melanomas not responsive -if surgery will lead to significant functional compromise -cosmesis -frail patients unsuitable for surgery Groin nodal involvement- second line/ recurrence Young women with clitoral cancer Failed local control

chemotherapy No reliable data supports effectiveness of chemotherapy Its enhances tissue sensitivity to radiation Regimen -Cisplatin 100mg /m2 iv on day 1 -Bleomycin 15mg iv on day 1 and 8 -Methotrexate 300mg/m2 with citrovarum factor rescue on day 8

cont This must be repeated every 21 days for 2-3 cycles if patient condition and laboratory results permit Normal: - liver fxn test, -blood urea and electrolyte - HB > 10 g/dl -WBC- > 3x 109/l, platelet > 100 x 109/l

cont Topical 5 fluoro-uracil Cryotherapy Photodynamic therapy Done for stage 0

Immunotherapy- futuristic mgt Vaccine for HPV Use of immuno-modulating agent – topical imiquimod

FOLLOW-UP CARE Watch out for Psychosexual disorders 3 monthly visits for the 1st 2 years is recommended because 80% of recurrence are in this time period. Every 6 months for detection of recurrent dx or 20 primary cancer. At each visit, look for evidence of recurrence of metastasis & possibility of further neoplastic alteration in adjacent skin Local recurrence can be treated by radical surgical excision or radiotherapy

COMPLICATIONS SHORT TERM Haemorrhage, Haematoma Wound infection and breakdown Deep-vein thrombosis Pressure sores LONG TERM Introital stenosis (dyspareunia, Apareunia) Urinary & faecal incontinence Chronic lymphoedema/Lymphocyst formation Cellulitis and Lymphangitis Psychological and psychosexual problems

Differential diagnosis Vulvar dystrophies- lichen sclerosus, squamous cell hyperplasia Paget’s dx Vulval intraepithelia neoplasia Aphthous ulcers Herpes genitalis Lyphogranuloma venerum Tuberculosis Chancroid primary syphilis

PROGNOSIS Early stage disease carries good prognosis Local recurrence at sites other than the vulva carries poor prognosis Short interval between treatment and recurrence is a poor prognostic sign Overall survival = 75% Stage I = 90% Stage II = 81% Stage III = 68% Stage IV = 20%

CONCLUSION A rare tumour Requires expertise and multidisciplinary approach in management Surgery is associated with significant morbidity & therefore needs to be individualized

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