Abstract
BackgroundPaget's disease is a rare skin disorder occurring in the breast (mammary) or in the groin, genital, peri-anal and axillary regions (extra-mammary). Typical treatment involves surgical excision, which in the case of extra-mammary Paget's disease, can lead to significant morbidity. Photodynamic therapy (PDT) which uses a topical or intravenous photosensitizing agent that is activated by a light source to ablate abnormal tissue, offers a minimally invasive alternative. The purpose of this study was to assess the effectiveness of photodynamic therapy in the treatment of Paget's disease.MethodsFollowing Cochrane guidelines, a comprehensive systematic review of all clinical studies and reports examining the use of PDT for mammary and extra-mammary Paget's disease was conducted. Study quality was assessed using the Oxford Levels of Evidence Scale.Results21 retrospective and 2 prospective non-comparative studies were identified and included in the review: 9 case reports with 1-2 patients and 14 case series with 1-16 patients. These reports totalled 99 patients with 133 extra-mammary Paget's lesions and 3 patients (with 3 lesions) with mammary Paget's disease. Follow-up periods were typically one year or less, with 77/133 extra-mammary lesions exhibiting complete response to PDT. One recurrent mammary skin lesion and two mammary lesions treated concomitantly with surgery also exhibited complete responses.ConclusionsEvidence of the effectiveness of PDT for Paget's disease is promising, but limited. This may, in part, be explained by the rarity of the condition, making controlled comparative clinical trials challenging.
Highlights
Paget’s disease is a rare skin disorder occurring in the breast or in the groin, genital, perianal and axillary regions
Treatment typically involves surgical excision. This can lead to significant morbidity, especially in the case of extra-mammary Paget’s disease, where the lesions are often large
While topical photosensitizing agents are most commonly used (Levulan® Kerastick® and Metvix®), intravenously administered versions are available[11]. The purpose of this project was to assess the clinical effectiveness of photodynamic therapy (PDT) for mammary and extra-mammary Paget’s disease, based on existing published, peerreviewed clinical studies
Summary
Paget’s disease is a rare skin disorder occurring in the breast (mammary) or in the groin, genital, perianal and axillary regions (extra-mammary). Photodynamic therapy (PDT) which uses a topical or intravenous photosensitizing agent that is activated by a light source to ablate abnormal tissue, offers a minimally invasive alternative. Paget’s disease is an extremely rare form of intraepithelial adenocarcinoma that may have an underlying tumour component It primarily affects Caucasian women over the age of 50[1]. While the two types are histologically the same This can lead to significant morbidity, especially in the case of extra-mammary Paget’s disease, where the lesions are often large. Given the potential slow progression of the disease, considerable interest in less invasive approaches exists[8] One such approach is photodynamic therapy (PDT). While topical photosensitizing agents are most commonly used (Levulan® Kerastick® (a type of 5-aminolevulinic acid or ALA) and Metvix® (a type of methyl aminolevulinate or MAL)), intravenously administered versions (hematoporphyrin derivatives, such as porfimer sodium) are available[11]
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