RT Journal Article
SR Electronic
T1 Extramammary Paget Disease of the Vulva: A Case Series Examining Treatment, Recurrence, and Malignant Transformation
JF International Journal of Gynecologic Cancer
JO Int J Gynecol Cancer
FD BMJ Publishing Group Ltd
SP 632
OP 638
DO 10.1097/IGC.0000000000001189
VO 28
IS 3
A1 Roni Nitecki
A1 Michelle Davis
A1 Jaclyn C. Watkins
A1 Yiru E. Wu
A1 Allison F. Vitonis
A1 Michael G. Muto
A1 Ross S. Berkowitz
A1 Neil S. Horowitz
A1 Colleen M. Feltmate
YR 2018
UL http://ijgc.bmj.com/content/28/3/632.abstract
AB Objectives Extramammary Paget disease (EMPD) of the vulva is a rare lesion with a high recurrence rate ranging from 12% to 61%. The rate of underlying adenocarcinoma varies, but in the largest series was reported at 4%. Given the rarity of the disease there is a paucity of data to optimize treatment. This study aims to describe the management and recurrence patterns in a tertiary care setting and to offer suggestions for management in a modern-day setting.Methods Patients with pathologically confirmed EMPD treated from 2000 to 2015 were retrospectively identified using an IRB approved database. Clinical data were abstracted from the electronic medical record. Pathology underwent central review.Results Forty-four patients met criteria and underwent central pathology review. Forty-two patients were treated with surgical excision. Alternative treatment modalities included Mohs surgery in 3 patients and medical therapy in 20 patients. The median number of surgical procedures was 1 and the number of procedures ranged from 1 to 16. Twenty-five patients (56.8%) had recurrent disease with a median of 2 (1-6) recurrences per patient. The median disease-free interval was 28.7 months with a median follow up of 45.8 months (1.2-178.9 months). Three patients (7%) had invasive cancer and 7 patients (16%) were diagnosed with a separate malignancy at or following diagnosis of EMPD. Despite radical resection, the majority of patients had positive margins and there was no significant difference in disease recurrence between simple and radical resection (P = 0.69).Conclusions Patients with EMPD in this series have a high rate of recurrence. Many undergo multi-modal therapy often with multiple providers. However, patients experience relatively long disease-free intervals with a low rate of associated malignancy. We propose an algorithm for management that focuses on symptom control and minimizing morbidity of treatment intervention once invasive disease has been excluded.