Volume 26, Issue 5 pp. 311-316
Original Article

A Case of Metastatic Extramammary Paget's Disease That Responded to Combination Chemotherapy

Naoya Yamazaki

Corresponding Author

Naoya Yamazaki

Dermatology Division, National Cancer Center Hospital, Tokyo, Japan

Reprint requests to: Naoya Yamazaki M.D., Ph.D., Dermatology Division, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104-0045, Japan.Search for more papers by this author
Akifumi Yamamoto

Akifumi Yamamoto

Dermatology Division, National Cancer Center Hospital, Tokyo, Japan

Search for more papers by this author
Takashi Wada

Takashi Wada

Dermatology Division, National Cancer Center Hospital, Tokyo, Japan

Search for more papers by this author
Masashi Ishikawa

Masashi Ishikawa

Dermatology Division, National Cancer Center Hospital, Tokyo, Japan

Search for more papers by this author
Yoshihiro Moriya

Yoshihiro Moriya

Dermatology Division, National Cancer Center Hospital, Tokyo, Japan

Colorectal Surgery Division, National Cancer Center Hospital, Tokyo, Japan

Search for more papers by this author
Yukihiro Nakanishi

Yukihiro Nakanishi

Dermatology Division, National Cancer Center Hospital, Tokyo, Japan

Pathology Division, National Cancer Center Research Institute, Tokyo, Japan

Search for more papers by this author
First published: 09 April 2015
Citations: 27

Abstract

Extramammary Paget's disease is considered to be a malignant tumor originating from the sweat glands. Some cases of extramammary Paget's disease infiltrate the dermis and metastasize to the regional lymph nodes. No standard treatment has been established for advanced cases. Few previous studies of the treatment for metastatic Paget's disease have revealed an effective regimen. The prognosis of metastasized cases is very poor.

We encountered a patient in whom extramammary Paget's disease had metastasized to the lymph nodes beyond the regional lymph nodes and systemic chemotherapy was partially effective. Combination chemotherapy consisted of mitomycin C 3.5 mg/m2 and epirubicin 50 mg/m2 on day 1, vincristine 0.6 mg/m2 on days 1 and 7, cisplatin 30 mg/m2 from days 1 to 3, and 5-fluorouracil 350 mg/m2 from days 3 to 7. After two courses of chemotherapy, the metastatic lymph nodes decreased in size by more than 90% compared to that before chemotherapy. We defined the treatment effect of this regimen as a partial response (PR). Microscopic examination of the resected lymph nodes revealed replacement of metastatic lesions by fibrous tissue, suggesting a therapeutic effect. Anorexia, alopecia, and leukopenia (neutropenia) have been reported as toxicities, but all were tolerated.

Our results may provide useful indications for the management of this tumor. This particular combination chemotherapy is recommended for extramammary Paget's disease patients with systemic nodal metastases.

The full text of this article hosted at iucr.org is unavailable due to technical difficulties.