Invasive extramammary Paget’s disease of the vulva: a multimodality organ‑preserving approach

Invasive extramammary Paget’s disease of the vulva: a multimodality organ‑preserving approach DOI: https://doi.org/10.18203/2320-6012.ijrms20260645Keywords: Extramammary Paget's disease, Vulva, Invasive Paget's disease of vulvaAbstract Extramammary Paget’s disease (EMPD) of the vulva is an uncommon intraepidermal adenocarcinoma of apocrine gland- bearing skin, typically affecting elderly women. It frequently presents as a chronic pruritic eczematous lesion and is often misdiagnosed as benign dermatoses, leading to delay in diagnosis. While many cases remain intraepidermal, a subset progresses to invasive disease with stromal and visceral involvement, associated with nodal and distant metastasis and worse prognosis. There is no universally accepted standard for locally advanced invasive EMPD; surgery, radiotherapy, and systemic therapy are all used in various combinations. A 70‑year‑old postmenopausal woman presented with invasive vulvar EMPD with bladder base infiltration, staged as T2N1M0 (stage IIIA). She received neoadjuvant paclitaxel- carboplatin chemotherapy followed by definitive external beam radiotherapy to the pelvis with a vulvar boost after declining radical vulvectomy. She achieved a partial response to chemotherapy and remains disease‑free on follow‑up after completion of radiotherapy, illustrating the role of individualized multimodal, organ‑preserving treatment in invasive vulvar EMPD. Metrics References Fanning J, Lambert HC, Hale TM, Morris PC, Schuerer DJ. Paget's disease of the vulva: prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence after surgical excision. Am J Obstet Gynecol. 1999;180(1 Pt 1):24-7. DOI: https://doi.org/10.1016/S0002-9378(99)70143-2 Wilkinson EJ, Brown HM. 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