Extramammary Paget´s disease of the vulva – a case report


Authors: D. Kolářová 1;  A. Havránková 1;  P. Líbalová 1;  Š. Frühaufová 2;  Z. Vernerová 2;  E. Kučera 1
Authors‘ workplace: Gynekologicko-porodnická klinika 3. LF UK a FNKV, Praha, přednosta doc. MUDr. E. Kučera, CSc. 1;  Ústav patologie 3. LF UK a FNKV, Praha, přednosta prof. MUDr. V. Mandys, CSc. 2
Published in: Čes. Gynek.2014, 79, č. 5 s. 394-398

Overview

Objective:
Analysis of one case of vulvar extramammary Paget´s disease (EMPD) and associated well differentiated endometrial adenocarcinoma.

Desing:
A case report.

Methods and results:
On this case report we present current theoretical knowledge about EMPD, difficulty of diagnostics, treatment and dispensarization the patients with this rare intraepithelial non-squamous neoplasia. We report a rare case of a 62 years old female patient with extramammary Paget´s disease of vulva and associated well differentiated endometrial adenocarcinoma.

Conclusions:
EMPD is a rare intraephitelial non-squamous neoplasia, which represents less then 1% vulvar tumors. Predominantly it affects white women between 60 and 80 years of age. EMPD occurs in cutaneous areas bearing apocrine glands – vulva, perineum, perianal area, axilla, penis, scrotum and rarely region of tights or buttocks. It is characterized microscopically by the presence of specific tumor cells called Paget´s cells – atypical large cells with pale clear cytoplasm and large round nuclei.

Keywords:
extramammary Paget´s disease, EMPD, Paget´s cells, vulvectomy, imiquimod 5%, photodynamic therapy, imunotherapy, radiotherapy


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Labels
Paediatric gynaecology Gynaecology and obstetrics Reproduction medicine

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Czech Gynaecology

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2014 Issue 5

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