Paget's disease of the vulva: A review of 89 cases

Abstract

The purpose of this study was to retrospectively review the clinical characteristics and outcomes of a series of women with Paget's disease of the vulva. A retrospective review was performed of 89 women with Paget's disease of the vulva evaluated at a single institution between 1966 and 2010. Medical records were reviewed for demographic information, clinical data, pathologic findings, treatment modalities and outcomes. We found that the primary treatment was surgery for 74 (83.1%) patients, with positive margins noted in 70.1% of cases. Five patients (5.6%) underwent topical treatment with imiquimod and/or 5-fluorouracil, one patient (1.1%) underwent laser ablation and treatment was unknown in 9 patients (10.1%). The majority of patients had multiple recurrences, with 18% having four or more recurrences. There were no significant differences in recurrence rates between patients who underwent surgery and those who did not. Furthermore, there was no association between positive margins following primary surgery and recurrence. Forty-one patients (46.1%) were diagnosed with 53 synchronous or metachronous cancers. Seven patients (7.9%) were found to have invasive vulvar cancer with 1 mm or more depth of invasion, but none of the patients died of Paget's disease or associated vulvar/vaginal cancer. Our findings suggest that the majority of patients with Paget's disease of the vulva develop multiple recurrences regardless of treatment modality or margin status. Alternatives to surgery are needed to better care for women with this disease.

A Case of Chronic and Relapsing Paget Disease of the Vulva

“…1 The treatment for PDV is challenging due to frequent relapses, regardless of the treatment option. 12 Surgery has been the standard treatment for PDV but, because of its multifocal and subclinical skin involvement, surgical margins are frequently positive. As such, and although the results have not been encouraging (since involved surgical resection margins are neither associated with recurrence rates nor with overall survival), surgery with intraoperative margin control (Mohs microsurgery) is the preferred surgical method.…”

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

“…Diagnosis is made by skin biopsy and or immunohistochemistry staining of GCDFP-15, CK-7, CK-20 and CEA. Pathologically, EMPD of the vulva resembles mammary Paget’s of the nipple and areola 7 8…”

“…Patients with EMPD of the vulva are at increased risk of synchronous or metachronous neoplasms including cervical, colorectal, and transitional epithelium carcinomas 7 8 10. Therefore, routine screening in some cases and prolonged surveillance has been recommended, although there are no clear guidelines.…”

“…Therefore, routine screening in some cases and prolonged surveillance has been recommended, although there are no clear guidelines. Recommended screening tests to rule out co-existing malignancies include, colonoscopy, cervical cytology, mammogram, cystoscopy, and/or urinary cytology to rule out coexisting or underlying malignancies 7–10…”

Vulvovaginal conditions remain difficult and unresolved

“…[1] Studies by himself have since given a different emphasis, and a study published by the MD Anderson Cancer Center in 2017 showed that, of 89 patients who were followed up over 44 years, 46% had a synchronous or metachronous underlying carcinoma supporting the current recommended management of colonoscopy and cystoscopy on diagnosis, and stringent follow-up. [2] This represents an about-face on a condition fairly recently considered less significant than the more well-known Paget's disease of the breast. …”