Interventions for the treatment of Paget's disease of the vulva

Abstract

Background This is an updated version of the original Cochrane Review published in Issue 10, 2013. Extramammary Paget's disease is a rare form of superficial skin cancer. The most common site of involvement is the vulva. It is seen mainly in postmenopausal white women. Paget's disease of the vulva o en spreads in an occult fashion, with margins extending beyond the apparent edges of the lesion. There is a range of interventions from surgical to non-invasive techniques or treatments. The challenges of interventions are to remove or treat disease that may not be visible, without overtreatment and with minimisation of morbidity from radical surgery. There is little consensus regarding treatment. Surgery, by default, is the most common treatment, but it is challenging to excise the disease adequately, and recurrence is common, leading to repeated operations, and destruction of anatomy. Alternative treatments of photodynamic therapy, laser therapy, radiotherapy, topical treatments or even chemotherapy have been mooted, and it is important to evaluate the available evidence. It is essential to assess whether newer cell-specific treatments, such as photodynamic therapy and imiquimod, can reduce the need for radical surgery. Objectives To evaluate the benefits and harms of di erent treatment modalities for the management of Paget's disease of the vulva. Search methods We searched the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE (via Ovid) and Embase (via Ovid) up to 8 May 2018. We also searched registers of clinical trials, abstracts of scientific meetings and reference lists of review articles. Selection criteria We searched for randomised controlled trials (RCTs) and well-designed non-randomised studies that compared di erent interventions in women with Paget's disease of the vulva, Data collection and analysis Two review authors independently assessed whether potentially relevant studies met the inclusion criteria. We found no trials and, therefore, analysed no data. Main results The search for the original version of the review identified 635 unique references. We found 31 references (which reported on 30 studies) in full text a er inspection of titles and abstracts, but we excluded them all as they did not meet the inclusion criteria. However, we have Interventions for the treatment of Paget's disease of the vulva (Review)

Extramammary Paget Disease of the Vulva

“…Vulvar surgery is often disfiguring, resulting in significant morbidity including sexual dysfunction, and psychological disability, 15,16 and often does not cure EMPD. 1,7,9,10,17 These data bring into question the surgical approach to treatment and highlight the importance of considering a less invasive approach to EMPD. In this cohort, we demonstrate that medical management is feasible, with few side effects.…”

Invasive Paget Disease of the Vulva

“…Nevertheless, the majority of authors conclude that surgery is the most common treatment modality, ranging from simple vulvar resection to radical vulvectomy. 19 Even less is known about standard treatment modalities for invasive forms of EPDV. Radical surgical procedures are commonly performed in these cases, and no guidelines have been published about adjuvant therapies or follow-up modalities.…”

“…19 Positivity of margins has been widely discussed, but its role in determining prognosis is still unclear. In this multifocal disease of the vulva, surgical radicality of margins is difficult to achieve.…”

Paget's disease of the vulva: A review of 89 cases

“…The optimal management of Paget's disease of the vulva remains unclear. Surgical excision is usually the primary therapy (Edey et al, 2013). Furthermore, the lesions often extend past clinically apparent borders resulting in positive margins, and surgical excision is limited by the anatomy of the vulva.…”