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

Zdroje

1. Breslin, TM., Xu, F. Paget’s disease. In: Singletary, SE., Robb, GL., Hortobagyi, GN., ed. Advanced therapy of breast disease. 2nd ed. London: BC Decker Inc, 2004. p. 694–699.

2. Brett, M., Coldrion, BM., Goldsmith, BA., et al. Surgical treatment of extramammary Paget’s disease. A report of six cases and examination of Mohs micrographic surgery compared with conventional surgical excision. Cancer, 1991, 67, p. 933–938.

3. Cawley, LP. Extramammary Paget’s disease; report of a case. Am J Clin Pathol, 1957, 27, p. 559–566.

4. Fanning, JH., Lambert, CL., Hale, TM., et al. Paget‘s disease of the vulva: prevalence of associated vulvar adenocarcinoma, invasive Paget‘s disease, and recurrence after surgical excision. Am J Obstet Gynec, 1999, 180, 1, p. 24–27.

5. Fien, SM., Oseroff, AR. Photodynamic therapy for non-melanoma skin cancer. J Natl Compr Canc Netw, 2007, 5, p. 531–540.

6. Fishman, A., Lew, S., Altaras, M., et al. A 30s PAS stain for frozen section analysis of surgical margins of vulvectomy in Paget’s disease. Eur J Gynaecol Oncol, 1998, 19, p. 482–483.

7. Guerrieri, M., Back, MF. Extramammary Paget’s disease: role of radiation therapy. Australas Radiol, 2002, 46, p. 204–208.

8. Hendi, A., Brodland, DG., Zitelli, JA. Extrammamary Paget’s disease: surgical treatment with Mohs micrographic surgery. J Am Acad Dermatol, 2004, 51, p. 767–773.

9. Niikura, H., Yoshida, H., Ito, K., et al. Paget‘s disease of the vulva: clinicopathologic study of type 1 cases treated at a single institution. Int J Gynecol Cancer, 2006, 16:1212–5. 10.1111/j.1525-1438.2006.00602.

10. Parker, LP., Parker, JR., Bodurka-Bevers, D., et al. Paget‘s disease of the vulva: pathology, pattern of involvement, and prognosis. Gynecol Oncol, 2000, 77(1), p. 183–189.

11. Pliskow, S. Vulvar Paget‘s disease. Clinicopathological review of 14 cases. J Fla Med Assoc, 1990, 77(7), p. 667–671.

12. Piura, B., Rabinovich, A., Dgani, R. Extramammary Paget‘s disease of the vulva: report of five cases and review of the literature. Eur J Gynaecol Oncol, 1999, 20(2), p. 98–101.

13. Seok-Hyun Son, MD., Jung-Seok Lee, et al. The role of radiation therapy for the extramammary Paget‘s disease of the vulva. Experience of 3 cases. Cancer ResTreat, 2005, 37(6), p. 365–369.

14. Stacy, D., Burrell, MO., Franklin, EW. 3rd. Extramammary Paget’s disease of the vulva and anus: use of intraoperative frozen-section margins. Am J Obstet Gynecol, 1986, 155, p. 519–523.

15. Tanskanen, M., Jahkola, T., Asko-Seljavaara, S., et al. HER 2 oncogene amplification in extramammary Paget‘s disease. Histopathology, 2003, 42, p. 575–579.

16. Zollo, JD., Zeitouni, NC. The Roswell Park Cancer Institute experience with extramammary Paget‘s disease. Br J Dermatol, 2000, 142(1), p. 59–65.