Source 1: WHO Class Female Reproductive Organs
Notes: Chapter 9, Vulva
Reportability/Histology--Vulva: Is Extramammary Paget neoplasm (intraepithelial glandular neoplasm) reportable? See Discussion.
Patient had a vulvar biopsy with final diagnosis of Extramammary Paget neoplasm (intraepithelial glandular neoplasm). No invasion identified. We are unable to contact the pathologist or physician for clarification.
Although this terminology is not listed in the ICD-O-3, web search results refer to this as a possible synonym for Paget disease with associated VIN III, which is reportable.
According to our subject matter expert, vulvar extramammary Paget neoplasm (intraepithelial glandular neoplasm) represents an in situ malignancy and should be reported.
He states "The traditional terminology should be 'extramammary Paget disease' to describe an in situ adenocarcinoma arising from extramammary glands in vulvar mucosa. I am not so sure about "extramammary Paget NEOPLASM", which may include all three Pagetoid processes: the traditional Paget disease, the Pagetoid spreading of an anal adenocarcinoma and a Pagetoid spreading of an urothelial carcinoma from the urethra. Regardless, all these entities are considered at least in situ carcinomas."
We recommend that you review clinical records and imaging for the clinical scenarios mentioned above.