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[Vulvar Paget's disease. Clinico-pathologic review of the literature]
M Preti1, L Micheletti, B Ghiringhello
1Dipartimento di Discipline Ginecologiche ed Ostetriche, Università degli Studi, Torino. mario.preti@tin.it
Abstract:
In 1986 the International Society For the Study of Vulvar Disease classified vulvar Paget's disease (VPD) as a non-squamous intraepithelial lesion of the vulva. The clinical multiform aspect of VPD, similar to other dermatological lesions, often delays the execution of a biopsy. Paget's cells could be instead easily identified at histological examination and with histochemical reactions. Underlying adenocarcinomas or stromal invasion are present in about 10% of intraepithelial VPD. Patients with VPD are at risk for a second synchronous or metachronous neoplasia: colo-rectal adenocarcinoma (more frequent in perianal localization of VPD), cervical adenocarcinoma, carcinoma of the transitional epithelium from the renal pelvis to urethra and mammary carcinoma. A wide spectrum of frequency of these associations is reported in the literature (0-45%). Therapy for intraepithelial VPD is wide and deep surgical resection comprising all the skin appendages. However VPD has a high frequency of recurrences (15-62%), often irrespective for radicality of surgical excision. When association with underlying invasive adenocarcinoma or stromal invasion is histologically confirmed, vulvar surgical approach must be integrated with inguino-femoral lymphadenectomy. The role of chemotherapy and radiotherapy in the multimodal approach to extensive or recurring VPD is still controversial. Recurrences or progression of intraepithelal VPD are reported more than 10 years from first surgical resection so that long term follow-up is mandatory.
Insights
Vulvar Paget's disease (VPD) is a skin lesion often mistaken for other conditions, delaying diagnosis. Early detection via histology is crucial, as VPD can indicate underlying cancers and frequently recurs, necessitating long-term monitoring.
Area of Science:
- Gynecologic Oncology
- Dermatopathology
- Surgical Pathology
Context:
- Vulvar Paget's disease (VPD) is a rare intraepithelial neoplasm of the vulva.
- Its varied clinical presentation mimics benign dermatological conditions, often delaying diagnosis.
- Histological examination with histochemical reactions is key for accurate identification of Paget's cells.
Purpose:
- To review the classification, diagnosis, associated risks, treatment, and follow-up of vulvar Paget's disease.
- To highlight the diagnostic challenges posed by VPD's clinical presentation.
- To emphasize the importance of recognizing associated malignancies and the high recurrence rates.
Summary:
- VPD is classified as a non-squamous intraepithelial lesion, but approximately 10% of cases involve underlying adenocarcinomas or stromal invasion.
- Patients with VPD have an increased risk of synchronous or metachronous neoplasms, including colorectal, cervical, urothelial, and breast cancers.
- Treatment for intraepithelial VPD involves wide surgical excision, but recurrence rates are high (15-62%).
- Invasive cases require lymphadenectomy, and the role of adjuvant therapies remains debated. Long-term follow-up is essential due to late recurrences.
Impact:
- Accurate diagnosis through histology is vital for appropriate management of vulvar Paget's disease.
- Awareness of associated malignancies can lead to earlier detection and improved patient outcomes.
- Understanding recurrence patterns underscores the need for vigilant, long-term patient surveillance.