Chronic vulvar Paget’s disease: a therapeutic challenge with a review of the literature

Journal of Mind and Medical Sciences Volume 6 | Issue 2 Article 25 2019 Chronic vulvar Paget’s disease: a therapeutic challenge with a review of the literature Bouhani Malek Zemni Ines Fertani Yasmine Bouzaiene Hatem Rahal Khaled Follow this and additional works at: https://scholar.valpo.edu/jmms Part of the Obstetrics and Gynecology Commons, Oncology Commons, Plastic Surgery Commons, and the Surgery Commons Recommended Citation Malek, Bouhani; Ines, Zemni; Yasmine, Fertani; Hatem, Bouzaiene; and Khaled, Rahal (2019) "Chronic vulvar Paget’s disease: a therapeutic challenge with a review of the literature," Journal of Mind and Medical Sciences: Vol. 6 : Iss. 2 , Article 25. DOI: 10.22543/7674.62.P356360 Available at: https://scholar.valpo.edu/jmms/vol6/iss2/25 This Case Presentation is brought to you for free and open access by ValpoScholar. It has been accepted for inclusion in Journal of Mind and Medical Sciences by an authorized administrator of ValpoScholar. For more information, please contact a ValpoScholar staff member at . https://scholar.valpo.edu/jmms/ https://proscholar.org/jmms/ ISSN: 2392-7674 J Mind Med Sci. 2019; 6(2): 356-360 doi: 10.22543/7674.62.P356360 Received for publication: August 12, 2018 Accepted: September 16, 2019 Case report Chronic vulvar Paget’s disease: a therapeutic challenge with a review of the literature Bouhani Malek1, Zemni Ines1, Fertani Yasmine1, Bouzaiene Hatem1, Rahal Khaled1 1 Department of oncologic surgery, Salah Azaiz Institute, Tunis Tunisia Abstract Vulvar Paget’s disease is an uncommon adenocarcinoma of the female genital tract, occuring more often in postmenopausal Caucasian women and accounting for 1-2% of all vulvar malignancies. A 39-year-old female patient was examined for the erythematous and pruritic vulvar lesion, with biopsy and histological examination revealing vulvar Paget’s disease. We did not diagnose any associated neoplasm. The patient underwent four surgical resections for relapsed disease, varying from local excision to extended excision, including radical vulvectomy, with esthetic coverage. The margins were involved. Due to the extended target volumes, radiotherapy was rejected and the patient underwent a local topical therapy with Imiquimod 5%. The patient was free of disease, with three years of regular follow-ups. This case was challenging due to the long recurrence of the disease for approximately 26 years and due to the therapeutics issues that occurred. Keywords  Highlights ✓ Vulvar Paget’s disease is a therapeutic challenge for physicians due to its chronicity and its high rate of recurrence. The management of local recurrence is associated with mutilating surgery. vulvar neoplasm, reconstructive surgical procedures, extramammary Paget’s disease, Imiquimod ✓ Currently, new conservative approaches such as local cream use, laser therapy, and many other treatments are being adopted. Imiquimod 5% cream is an alternative for consideration in the case of invaded margins. To cite this article: Bouhani Malek, Zemni Ines, Fertani Yasmine, Bouzaiene Hatem, Rahal Khaled. Chronic vulvar Paget’s disease: a therapeutic challenge with a review of the literature. J Mind Med Sci. 2019; 6(2): 356-360. DOI: 10.22543/7674.62.P356360 *Corresponding author: Bouhani Malek, Department of oncologic surgery, Salah Azaiz Institute, Boulevard 9th April, Tunis 1009, Tunisia E-mail: Bouhani Malek et al. Introduction In 1876, Sir James Paget observed chronic eczematous disease on the skin of the nipple and areola that was associated with a breast intraductal carcinoma (1). This entity was defined as mammary Paget’s disease. Later, in 1901, William Dubreuilh described vulvar Paget’s disease (VPD) (2). VPD is an uncommon adenocarcinoma of the female genital tract (3). It occurs more often in postmenopausal Caucasian women (3) and accounts for 12% of all vulvar malignancies (3, 4). Given its chronicity, the disease is under-diagnosed, thus leading to a real therapeutic challenge for physicians. Surgery remains the treatment of choice; however, many new therapeutic approaches also seem appealing. We cite radiotherapy, topical therapy, laser therapy, photodynamic therapy, and radiotherapy as possibilities (5-10). Despite these varied therapies, VPD still has high relapse rates, ranging from 15 to 72% (5, 7, 8, 11). Figure 1. General overview showing multiple erythematous lesions located in mons veneris, in the flap and in the peri-anal region (red circles). Case report In 1990, a 39-year-old Caucasian woman presented with an erythematous lesion on the left hemi vulva at another medical facility. She underwent local excision of the lesion and the histological report revealed VPD, but the limits and the invasion were not determined. She did not undergo adjuvant therapy, and her case was subsequently lost over time. Twenty-two years later (in 2012), she presented with a new though similar lesion in the same localization. The patient underwent mammography, coloscopy, gastric endoscopy, and cystoscopy, and all results were negative. She then underwent left hemivulvectomy. The final histological examination concluded VPD again, and the limits were free with a 2mm clearance. Figure 2. Closeup overview of multiple erythematous lesions located in mons veneris, in the flap and in the peri-anal region (red arrows). We performed punch biopsies for each suspicious lesion, and the histological examination confirmed the VPD recurrence. She underwent a large excision and coverage by a V-Y flap of the inner thigh (Figures 3 and 4). Three years later (in 2015), after the first relapse, she presented with an erythematous lesion on the right hemivulva and the perianal region. The inguinal areas were free of lymph nodes. The punch biopsy with histological examination confirmed the recurrence of VPD. She had a totalization of the vulvectomy with excision of the perianal lesion and an advanced flap of the inner thigh. We performed a left iliac colostomy to improve the peri-anal scarring. The limits were not free, but the patient was lost again. One year later (in 2016), she was referred to our institute for an evolutive disease. The physical examination revealed multiple erythematous lesions located in mons veneris, in the flap and in the perianal region (Figures 1 and 2). Figure 3. Large excision of the perineal areas, mons veneris and the flap. 357 Chronic vulvar Paget’s disease Figure 4. Coverage by a V-Y flap of the inner thigh Frozen section examination pointed to free limits, but final histology exams showed involved margins. Due to the multiple recurrences of the disease and the involved margins, the patient was proposed for external beam therapy. But radiotherapy was rejected due to the extent of the target volumes. The multidisciplinary committee indicated a topical therapy with Imiquimod 5%. The patient underwent local therapy with imiquimod 5% twice a week for 6 months. At the time of this publication, the p (...truncated)