Paget’s disease of the vulva: a clinicopathologic institutional review

• Published in: International journal of clinical oncology Vol. 17; no. 6; pp. 569 - 574
• Main Authors: Mendivil, Alberto A., Abaid, Lisa, Epstein, Howard D., Rettenmaier, Mark A., Brown, John V., Micha, John P., Wabe, Marie A., Goldstein, Bram H.
• Format: Journal Article
• Language: English
• Published: Japan Springer Japan 01.12.2012; Springer Nature B.V
• Subjects: Aged; Aged, 80 and over; Bone cancer; Bone diseases; Cancer Research; Disease Progression; Female; Gynecology; Humans; Medicine; Medicine & Public Health; Middle Aged; Neoplasm Recurrence, Local - pathology; Neoplasm Recurrence, Local - therapy; Oncology; Original Article; Paget Disease, Extramammary - pathology; Paget Disease, Extramammary - therapy; Pathology; Surgical Oncology; Vulva - pathology; Vulvar Neoplasms - pathology; Vulvar Neoplasms - therapy; Gynecologic oncology; Extramammary; Vulvar neoplasm; Paget’s disease
• ISSN: 1341-9625; 1437-7772
• DOI: 10.1007/s10147-011-0325-0

Background The aim of this study was to assess the clinicopathologic characteristics of patients with Paget’s disease of the vulva who were treated by our gynecologic oncology service between 1985 and 2010. Methods Vulvar Paget’s disease patient demographics, pathologic diagnosis, treatment and follow-up data were reviewed over a 25-year period. Results The vulvar Paget’s disease patients were primarily (62.5%) treated with a partial simple vulvectomy. Three patients had a history of malignancy, although none of them was intercurrent. Eleven patients had microscopically positive margins, 5 of whom developed progressive disease. Conversely, 5 patients had negative margins, of whom 4 had recurrent disease. There was a significant relationship between the presence of invasive disease and patient progression-free interval (PFI) ( p  = 0.007), but margin status and lesion size did not correlate with PFI ( p  > 0.05). Median patient PFI and follow-up was 30 and 53 months, respectively. Conclusions We found a significant relationship between the presence of invasive disease and patient PFI in vulvar Paget’s disease although the presence of microscopic positive margins and lesion size were not prognostic indicators. In patients with high risk factors, prolonged surveillance should be considered an essential component of optimal patient management.