Cancer Res Treat.  2005 Dec;37(6):365-369.

The Role of Radiation Therapy for the Extramammary Paget's Disease of the Vulva ; Experience of 3 Cases

• Son SH
• Lee JS
• Kim YS
• Ryu MR
• Chung SM
• Namkoong S
• Han GT
• Lee HJ
• Yoon SC

Abstract

We have experienced three cases of extramammary Paget's disease (EMPD) of the vulva that received radiation therapy (RT). Here, we analyze the efficacy of RT and include a literature survey. Three patients with EMPD of the vulva were treated with curative RT between 1993 and 1998. One of the patients had associated underlying adenocarcinoma of the vulva. The total doses of radiation administered were 54~78 Gy/6~8 weeks. Radiation fields encompassed 2 to 3 cm outer margins free from all visible disease including or not including the inguinal area using a 9 MeV electron or a 6 MV photon beam. Follow-up durations after radiotherapy were 0.6~11 years. Complete response was obtained in all three patients. Marginal failure occurred in one patient, and another patient with underlying adenocarcinoma treated by vulvectomy with bilateral inguinal lymph node dissection followed by external RT showed no relapse. Radiation induced side effects were transient acute confluent wet desquamation in the treated area resulting in mild late atrophic skin changes. Although surgery is currently considered the preferred primary treatment for EMPD, it has a high relapse rate due to the multifocal nature of the disease. We conclude that RT is of benefit in some selected cases of EMPD.

Keyword

Extramammary Paget's disease of vulva; EMPD; Radiotherapy; RT

MeSH Terms

Adenocarcinoma
Follow-Up Studies
Humans
Lymph Node Excision
Paget Disease, Extramammary*
Radiotherapy
Recurrence
Skin
Vulva*

Figure

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  Fig. 1 Case A. A 78-year-old woman was referred because of a 5-year history of a pruritic, painful, and persistent erythematous lesion in the vulva; 16 years previously she had undertaken a vulvectomy for the same disease. It shows poorly defined infiltrated 5×6 cm sized erythematous plaque with some ulceration on the vulva which extended to the upper medial thigh.

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  Fig. 2 Case C. (A) A 81-year-old woman was referred because of pruritic whitish eczematous vulva lesions with palpable mass. Biopsies showed findings of invasive Paget's disease of the vulva and vaginal intraepithelial neoplasia. (B) After radiation therapy, she showed clinically complete remission.

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  Fig. 3 (A) Microscpic findings of the biopsy from the vulva show that large, pale and vacuolated cells are clustered just above the basal layer (H/E, ×100), (B) They show that pargetoid cells (⇩) are single, or arranged in rows or nests. They show abundant cytoplasm, round nuclei and prominent nucleoli, occasionally with mitosis (H/E, ×400).

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