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Case 4

Discussion

VAIN is a rare disease with an incidence of 0.2 to 0.3 per 100,000 women (1). Risk factors include smoking, low social economic status, history of abnormal Papanicolaou smear, genital warts and early hysterectomy (2, 3). A history of cervical dysplasia and cancer is seen in 48% and 9% respectively (3). VAIN may regress or progress to higher grade VAIN and invasive cancer. Among those with no treatment, 87% normalized, 9% persisted and 4% recurred (3). Progression of high grade VAIN to invasive cancer is 8% (4).

 

The majority (94%) of VAIN patients are asymptomatic (1). Around 76% of cases present with abnormal cytology while 22% present with vaginal warts (5). Diagnosis is by colposcopy directed vaginal biopsy. Acetowhite epithelium, absence of iodine uptake, punctuation and mosaicism is seen in 84%, 81%, 14% and 2% respectively (1, 5). The upper third of vagina is involved in 92.4% and is the most commonly affected site (6).

 

Treatment options of VAIN include conservative, medical (imiquimod cream, 5-fluorouracil cream, tricholoroacetic acid) (7, 8), surgical treatment (laser ablation, excision, vaginectomy) (8) and brachytherapy (9, 10). The choice of treatment is influenced by age, comorbidities, number and location of lesions, length of vagina, sexual activity, previous treatment, previous radiotherapy, physician experience and patient preference (1).

 

Conservative treatment and imiquimod cream are mostly for low grade VAIN and is not the treatment of choice for our patient (4, 7). Intravaginal 50% tricholoroacetic acid is successful in 53% of VAIN II/III (11). 5-fluorouracil cream is curative in 46% of VAIN II/III (4). However, it has side effects with 77.8% having vaginal discharge, 44.4% having post-coital bleeding, 8.2% having epithelial ulcers and 5.6% experiencing pain (12).

 

The success rate of surgical methods in treating VAIN II/III is higher. Laser ablation cures 68% of cases (13). It has the advantage of having minimal blood loss, suitable for multifocal lesions and sexual function can be retained. However, it is not desirable for VAIN at the vaginal vault because VAIN can be buried by suture lines and may not be adequately assessed by cytology or colposcopy (8). No histology can be obtained and invasive cancer may be missed. VAIN III in vaginal vault treated with laser ablation is associated with higher risk of recurrence (14). Vaginectomy can provide surgical specimen to assess possibility of invasive cancer and resection margins (1). The cure rate of upper vaginectomy for VAIN II/III is 80% (13). It is associated with risk of bladder or rectal injury, loss of coital function, vesicovaginal fistula and rectovaginal fistula. Upper vaginectomy is more successful than laser treatment in hysterectomized group (46% versus 20% success rate) (13).

 

Brachytherapy can be considered in highly selected patients such as poor surgical candidate with extensive VAIN. A local control rate of 93% has been reported (10).

 

Single treatment results in regression in 70%, 24% recur and 5% progress to invasion (15). Recurrence following partial vaginectomy, laser ablation and 5-fluorouracil was 0%, 38% and 59% respectively (1). Another study found a recurrence rate of 17% after vaginectomy for VAIN III (16). Recurrence rate of multifocal disease is higher than unifocal disease (57% versus 43%) (13). Grade of VAIN does not affect recurrence (1, 3, 15).

 

Our patient had two recurrences. Two recurrences are only seen in 8% of cases (1). The first episode of VAIN was diagnosed with cervical cancer and can be treated concomitantly with surgery. The extent of VAIN can be marked pre-operatively and vagina can be resected beyond the VAIN lesion. The recurrences were at the vaginal vault therefore laser ablation is not a desirable treatment. Medical therapy has lower success rate compared to vaginectomy. VAIN occurring after radiation therapy has been shown to be more refractory than those without history of radiation therapy and may be more likely to progress to invasive cancer (17). Vaginectomy can allow histological examination with higher success rate and is the appropriate treatment. Brachytherapy is an option but is associated with radiation exposure, secondary malignancy, affects sexual function and future surgery.

 

VAIN patients should be followed up with colposcopy and cytology (3). There is no evidence to support optimal interval and duration of follow up. Some suggested annual follow up (3). Late progression to invasive cancer has been reported (4, 9) and therefore VAIN cases should be followed up indefinitely (3).

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