Extramammary Paget’s disease (EMPD) of the vulva is a rare vulvar neoplasm but commonly arises during the postmenopausal period. Intraepithelial Paget’s disease may persist for prolonged periods without demonstrating invasion but with high rates of recurrence. Appearance of Paget’s disease in a split-thickness skin graft, is associated with an occurrence outside the grafted area. It demonstrates retrodissemination as the pathologic process hypothesized in the spread of the disease within the skin via lymphatics and vessels creating tissue bridges between sites of involvement. We present a case of an 81-year-old female, the patient came in for complaints of vulvar pruritus beginning at the left inguinal area three years prior to her diagnosis. She consulted with a dermatologist and was initially treated with steroids and emollients. Persistence of symptoms and enlargement of the lesion prompted a vulvar punch biopsy which showed Paget’s disease and referral to the Gynecologic Oncology service. Wide local excision with split-thickness skin grafting was performed. However, one year after her surgery, patient noted vulvar pain and palpable vulvar lesions. Biopsy was done which showed Extramammary Paget’s Disease recurrence. Patient underwent repeat wide local excision with frozen section, and split-thickness skin grafting. With the aid of frozen section, the intraepithelial involvement was noted to spread beyond the grossly apparent lesion. After 6 months post re-excision, patient noted vulvar pruritus and palpable vulvar lesions. Biopsy was done which showed Extramammary Paget’s Disease recurrence. Due to the proximity of the lesion to the sphincter and need for a colostomy, the patient did not consent for re-excision. Imiquimod 5% was chosen as the mode of treatment. The challenges of interventions are to remove or treat disease that may not be visible, without overtreatment and to minimize morbidity from radical surgery. Surgery remains the primary management for EMPD of the vulva. Imiquimod 5% can be used in recurrences. Despite the advances in the knowledge and management of vulvar Paget’s disease the high rate of recurrent disease remains a challenge for optimal management and would require frequent and long-term follow-up.
Paget Disease, Extramammary ; Female ; vulvar neoplasms ;

Background: Ovarian, fallopian tube, and peritoneal cancer patients with advanced-stage diagnosis or recurrences spread to the peritoneal surface of the abdomen. Hyperthermic intraperitoneal chemotherapy (HIPEC) can penetrate and eradicate tumors that are microscopic up to those with a diameter of 2.5 cm from the peritoneal surface following cytoreductive surgery (CRS). Objectives: The study aimed to determine the efficacy and safety of CRS with HIPEC versus CRS alone for patients with epithelial ovarian, fallopian tube, and peritoneal cancer. Materials and Methods: This retrospective cohort study included 50 patients (20 patients underwent CRS + HIPEC, while 30 patients underwent CRS alone). Records of these patients from January 2014 to June 2020 were reviewed, tabulated, and analyzed. Results: The difference in recurrence rate between CRS with HIPEC and CRS alone was not statistically significant (50% vs. 43%, P = 0.774). The median time to recurrence was 10 and 9 months, respectively (P = 0.636). Five percent in the HIPEC group succumbed to the disease, while 13% died in the CRS alone group (P = 0.636). More post-operative complications were noted in the HIPEC group (45% vs. 10%, P = 0.007), but among these, only 2 cases had grade 3 to 4 complications (10%). The addition of HIPEC in the management of these patients resulted in a longer operative time (360 vs. 240 min, P < 0.001) and postoperative hospital stay (8 vs. 6 days, P = 0.026). There were no intra- or peri-operative mortalities in both groups. Conclusion CRS with HIPEC and CRS alone showed similar time to recurrence and recurrence rate. CRS with HIPEC had low risk of grade 3-4 complications and may still be considered as a treatment option for advanced, progressive, and recurrent epithelial ovarian, fallopian tube, and peritoneal cancer.
Cytoreduction Surgical Procedures ; Hyperthermic Intraperitoneal Chemotherapy ; Ovarian Neoplasms