Extensive extramammary Paget’s disease of the vulva involving the bladder postradical split skin graft reconstruction

摘要

An 84-year-old woman presented to the gynaecology department with a 9-month history of vulval soreness, irritation and bleeding. Her medical history included Dukes C sigmoid adenocarcinoma, thyroid malignancy and multiple medical comorbidities including atrial fibrillation and ischaemic heart disease. The patient was a heavy smoker. On pelvic examination, there was a large erythematous plaque affecting the right labia majora, and biopsies (figure 1) confirmed vulval extramammary Paget’s disease (EMPD). Topical Imiquimod (TLR-7 inhibitor) was initially started with minimal response. The patient then underwent a wide local excision and had several local recurrences excised over the next few years. Finally, the patient required radical excision with a split skin graft reconstruction 6 years after original diagnosis. Three months following the radical procedure, the patient complained of vulval pain, haematuria and urethral spasm. Cystoscopy of the bladder revealed a trabeculated mucosa with a 1 cm raised pale lesion. The lesion was biopsied and confirmed to show recurrent vulval EMPD with disease extension involving Paget’s cells in the urethra (figure 2) and base of the bladder. Following urology review, a decision was made that in view of the patient’s performane status and in keeping with her wishes she was not eligibile for advanced treatment options such as radiotherapy. She was therefore referred to palliative and pain services for best supportive care.