A proposal for a TNM staging system for extramammary Paget disease

Retrospective analysis of 301 patients with invasive primary tumors

Kuniaki Ohara, Yasuhiro Fujisawa, Koji Yoshino, Yoshio Kiyohara, Takafumi Kadono, Yozo Murata, Hisashi Uhara, Naohito Hatta, Uchi Hiroshi, Shigeto Matsushita, Tatsuya Takenouchi, Toshihiko Hayashi, Kenichi Yoshimura, Manabu Fujimoto

Research output: Contribution to journalArticle

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Abstract

Background Although extramammary Paget disease (EMPD) usually appears as carcinoma in situ, it sometimes becomes invasive (iEMPD) and fatal. However, a TNM staging system for iEMPD has yet to be established. Objective The aim of this study was to establish a TNM staging system for iEMPD. Methods We retrospectively collected iEMPD patients treated at 12 institutes in Japan. Factors reported to be associated with survival such as distant metastasis, lymph node (LN) metastasis, and primary tumor status were evaluated using the log-rank test. Results We enrolled 301 iEMPD patients, of whom 114 had remote metastases (49 had both distant and LN metastasis; 2, distant metastasis only; and 63, LN metastasis only) and the remaining 187 patients had no remote metastasis. Distant metastasis (M1) showed worse survival (P < 0.00001). In the analysis of the 250 patients without distant metastasis, LN metastasis also showed worse survival (P < 0.00001). Among the patients with LN metastasis, 2 or more LN metastases (N2) showed worse survival than did single LN metastasis (N1, P = 0.02). Lastly, in the analysis of the 187 patients without metastasis, tumor thickness of over 4 mm or lymphovascular invasion showed worse survival (T2, P < 0.05 and P < 0.001, respectively). Patients with neither of these features were defined as T1. From these results, we propose this TNM staging system: stage I, T1N0M0; stage II, T2N0M0; stage IIIa, anyTN1M0; stage IIIb, anyTN2M0; stage IV, anyTanyNM1. Other than stages II and IIIa, each stage had a statistically distinct survival curve. Conclusion We propose a TNM staging system for EMPD using simple factors for classification that could provide important prognostic information in managing EMPD. However, accumulation of more patient data and further revision of the system are required.

Original languageEnglish
Pages (from-to)234-239
Number of pages6
JournalJournal of Dermatological Science
Volume83
Issue number3
DOIs
Publication statusPublished - Sep 1 2016

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Extramammary Paget's Disease
Neoplasm Staging
Tumors
Neoplasm Metastasis
Neoplasms
Lymph Nodes
Survival
Carcinoma in Situ

All Science Journal Classification (ASJC) codes

  • Biochemistry
  • Molecular Biology
  • Dermatology

Cite this

A proposal for a TNM staging system for extramammary Paget disease : Retrospective analysis of 301 patients with invasive primary tumors. / Ohara, Kuniaki; Fujisawa, Yasuhiro; Yoshino, Koji; Kiyohara, Yoshio; Kadono, Takafumi; Murata, Yozo; Uhara, Hisashi; Hatta, Naohito; Hiroshi, Uchi; Matsushita, Shigeto; Takenouchi, Tatsuya; Hayashi, Toshihiko; Yoshimura, Kenichi; Fujimoto, Manabu.

In: Journal of Dermatological Science, Vol. 83, No. 3, 01.09.2016, p. 234-239.

Research output: Contribution to journalArticle

Ohara, K, Fujisawa, Y, Yoshino, K, Kiyohara, Y, Kadono, T, Murata, Y, Uhara, H, Hatta, N, Hiroshi, U, Matsushita, S, Takenouchi, T, Hayashi, T, Yoshimura, K & Fujimoto, M 2016, 'A proposal for a TNM staging system for extramammary Paget disease: Retrospective analysis of 301 patients with invasive primary tumors', Journal of Dermatological Science, vol. 83, no. 3, pp. 234-239. https://doi.org/10.1016/j.jdermsci.2016.06.004
Ohara, Kuniaki ; Fujisawa, Yasuhiro ; Yoshino, Koji ; Kiyohara, Yoshio ; Kadono, Takafumi ; Murata, Yozo ; Uhara, Hisashi ; Hatta, Naohito ; Hiroshi, Uchi ; Matsushita, Shigeto ; Takenouchi, Tatsuya ; Hayashi, Toshihiko ; Yoshimura, Kenichi ; Fujimoto, Manabu. / A proposal for a TNM staging system for extramammary Paget disease : Retrospective analysis of 301 patients with invasive primary tumors. In: Journal of Dermatological Science. 2016 ; Vol. 83, No. 3. pp. 234-239.
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abstract = "Background Although extramammary Paget disease (EMPD) usually appears as carcinoma in situ, it sometimes becomes invasive (iEMPD) and fatal. However, a TNM staging system for iEMPD has yet to be established. Objective The aim of this study was to establish a TNM staging system for iEMPD. Methods We retrospectively collected iEMPD patients treated at 12 institutes in Japan. Factors reported to be associated with survival such as distant metastasis, lymph node (LN) metastasis, and primary tumor status were evaluated using the log-rank test. Results We enrolled 301 iEMPD patients, of whom 114 had remote metastases (49 had both distant and LN metastasis; 2, distant metastasis only; and 63, LN metastasis only) and the remaining 187 patients had no remote metastasis. Distant metastasis (M1) showed worse survival (P < 0.00001). In the analysis of the 250 patients without distant metastasis, LN metastasis also showed worse survival (P < 0.00001). Among the patients with LN metastasis, 2 or more LN metastases (N2) showed worse survival than did single LN metastasis (N1, P = 0.02). Lastly, in the analysis of the 187 patients without metastasis, tumor thickness of over 4 mm or lymphovascular invasion showed worse survival (T2, P < 0.05 and P < 0.001, respectively). Patients with neither of these features were defined as T1. From these results, we propose this TNM staging system: stage I, T1N0M0; stage II, T2N0M0; stage IIIa, anyTN1M0; stage IIIb, anyTN2M0; stage IV, anyTanyNM1. Other than stages II and IIIa, each stage had a statistically distinct survival curve. Conclusion We propose a TNM staging system for EMPD using simple factors for classification that could provide important prognostic information in managing EMPD. However, accumulation of more patient data and further revision of the system are required.",
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T1 - A proposal for a TNM staging system for extramammary Paget disease

T2 - Retrospective analysis of 301 patients with invasive primary tumors

AU - Ohara, Kuniaki

AU - Fujisawa, Yasuhiro

AU - Yoshino, Koji

AU - Kiyohara, Yoshio

AU - Kadono, Takafumi

AU - Murata, Yozo

AU - Uhara, Hisashi

AU - Hatta, Naohito

AU - Hiroshi, Uchi

AU - Matsushita, Shigeto

AU - Takenouchi, Tatsuya

AU - Hayashi, Toshihiko

AU - Yoshimura, Kenichi

AU - Fujimoto, Manabu

PY - 2016/9/1

Y1 - 2016/9/1

N2 - Background Although extramammary Paget disease (EMPD) usually appears as carcinoma in situ, it sometimes becomes invasive (iEMPD) and fatal. However, a TNM staging system for iEMPD has yet to be established. Objective The aim of this study was to establish a TNM staging system for iEMPD. Methods We retrospectively collected iEMPD patients treated at 12 institutes in Japan. Factors reported to be associated with survival such as distant metastasis, lymph node (LN) metastasis, and primary tumor status were evaluated using the log-rank test. Results We enrolled 301 iEMPD patients, of whom 114 had remote metastases (49 had both distant and LN metastasis; 2, distant metastasis only; and 63, LN metastasis only) and the remaining 187 patients had no remote metastasis. Distant metastasis (M1) showed worse survival (P < 0.00001). In the analysis of the 250 patients without distant metastasis, LN metastasis also showed worse survival (P < 0.00001). Among the patients with LN metastasis, 2 or more LN metastases (N2) showed worse survival than did single LN metastasis (N1, P = 0.02). Lastly, in the analysis of the 187 patients without metastasis, tumor thickness of over 4 mm or lymphovascular invasion showed worse survival (T2, P < 0.05 and P < 0.001, respectively). Patients with neither of these features were defined as T1. From these results, we propose this TNM staging system: stage I, T1N0M0; stage II, T2N0M0; stage IIIa, anyTN1M0; stage IIIb, anyTN2M0; stage IV, anyTanyNM1. Other than stages II and IIIa, each stage had a statistically distinct survival curve. Conclusion We propose a TNM staging system for EMPD using simple factors for classification that could provide important prognostic information in managing EMPD. However, accumulation of more patient data and further revision of the system are required.

AB - Background Although extramammary Paget disease (EMPD) usually appears as carcinoma in situ, it sometimes becomes invasive (iEMPD) and fatal. However, a TNM staging system for iEMPD has yet to be established. Objective The aim of this study was to establish a TNM staging system for iEMPD. Methods We retrospectively collected iEMPD patients treated at 12 institutes in Japan. Factors reported to be associated with survival such as distant metastasis, lymph node (LN) metastasis, and primary tumor status were evaluated using the log-rank test. Results We enrolled 301 iEMPD patients, of whom 114 had remote metastases (49 had both distant and LN metastasis; 2, distant metastasis only; and 63, LN metastasis only) and the remaining 187 patients had no remote metastasis. Distant metastasis (M1) showed worse survival (P < 0.00001). In the analysis of the 250 patients without distant metastasis, LN metastasis also showed worse survival (P < 0.00001). Among the patients with LN metastasis, 2 or more LN metastases (N2) showed worse survival than did single LN metastasis (N1, P = 0.02). Lastly, in the analysis of the 187 patients without metastasis, tumor thickness of over 4 mm or lymphovascular invasion showed worse survival (T2, P < 0.05 and P < 0.001, respectively). Patients with neither of these features were defined as T1. From these results, we propose this TNM staging system: stage I, T1N0M0; stage II, T2N0M0; stage IIIa, anyTN1M0; stage IIIb, anyTN2M0; stage IV, anyTanyNM1. Other than stages II and IIIa, each stage had a statistically distinct survival curve. Conclusion We propose a TNM staging system for EMPD using simple factors for classification that could provide important prognostic information in managing EMPD. However, accumulation of more patient data and further revision of the system are required.

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