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

Česká verze

Authors: D. Kolářová ¹; A. Havránková ¹; P. Líbalová ¹; Š. Frühaufová ²; Z. Vernerová ²; E. Kučera ¹
Authors place of work: 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 the journal: Ceska Gynekol 2014; 79(5): 394-398

Summary

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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