Treatment outcomes of advanced/metastatic extramammary Paget's disease in Korean patients: KCSG‐RC20‐06

Abstract Background Extramammary Paget's disease (EMPD) is rare. There are no standard treatments due to its rarity and few clinical trials. Methods The objective of this multicenter study was to investigate treatment outcomes of Korean patients with advanced/metastatic EMPD. Data were collecte...

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Main Authors: Byeong Seok Sohn, Jeongeun Kim, Miso Kim, Jung Yong Hong, Jieun Lee, Song Ee Park, Hyojeong Kim, Hyo Jin Lee, Eun Joo Kang, Soon Il Lee, In Hee Lee, Seok Jae Huh, Jeongmin Jo, Ho Young Kim
Format: Article
Language:English
Published: Wiley 2023-07-01
Series:Cancer Medicine
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Online Access:https://doi.org/10.1002/cam4.6190
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author Byeong Seok Sohn
Jeongeun Kim
Miso Kim
Jung Yong Hong
Jieun Lee
Song Ee Park
Hyojeong Kim
Hyo Jin Lee
Eun Joo Kang
Soon Il Lee
In Hee Lee
Seok Jae Huh
Jeongmin Jo
Ho Young Kim
author_facet Byeong Seok Sohn
Jeongeun Kim
Miso Kim
Jung Yong Hong
Jieun Lee
Song Ee Park
Hyojeong Kim
Hyo Jin Lee
Eun Joo Kang
Soon Il Lee
In Hee Lee
Seok Jae Huh
Jeongmin Jo
Ho Young Kim
author_sort Byeong Seok Sohn
collection DOAJ
description Abstract Background Extramammary Paget's disease (EMPD) is rare. There are no standard treatments due to its rarity and few clinical trials. Methods The objective of this multicenter study was to investigate treatment outcomes of Korean patients with advanced/metastatic EMPD. Data were collected retrospectively from 14 institutions participating in Korean Cancer Study Group (KCSG) Rare Cancer Committee. Results A total of 37 patients were identified. Of these 37 patients, 6 received locoregional therapy as a first‐line treatment. In 31 patients who received systemic chemotherapy as a first‐line treatment, platinum‐based chemotherapy (n = 22) achieved an objective response rate (ORR) of 45.5% and a median progression‐free survival (PFS) of 7.89 months. Taxane‐based chemotherapy (n = 8) achieved an objective response rate of 62.5% and median PFS of 9.73 months. In second‐line chemotherapy, platinum‐based chemotherapy (n = 4) had a disease control rate (DCR) of 75.0% and median PFS of 3.45 months. Taxane‐based chemotherapy (n = 8) had a DCR of 75.0% and a median PFS of 8.67 months. Six patients received anti‐human epidermal growth factor receptor 2 (HER2) antibody during first‐ and second‐line chemotherapy. Overall, systemic chemotherapy combined with anti‐HER2 antibody had an ORR of 100% and a median PFS of 13.31 months. The ORR and PFS with systemic chemotherapy combined with trastuzumab was better than platinum‐ and taxane‐based chemotherapy only. Conclusions Due to its rarity, advanced or metastatic EMPD still has no established standard treatment. Results of our study indicate that the combination of trastuzumab with taxane has longer survival than trastuzumab monotherapy or conventional platinum‐ or taxane‐based chemotherapy.
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spelling doaj.art-df826c92a4414bae9cbf04d45015e3ab2023-08-11T14:51:17ZengWileyCancer Medicine2045-76342023-07-011214151591517510.1002/cam4.6190Treatment outcomes of advanced/metastatic extramammary Paget's disease in Korean patients: KCSG‐RC20‐06Byeong Seok Sohn0Jeongeun Kim1Miso Kim2Jung Yong Hong3Jieun Lee4Song Ee Park5Hyojeong Kim6Hyo Jin Lee7Eun Joo Kang8Soon Il Lee9In Hee Lee10Seok Jae Huh11Jeongmin Jo12Ho Young Kim13Department of Internal Medicine, Sanggye Paik Hospital Inje University College of Medicine Seoul South KoreaDepartment of Oncology, Asan Medical Center University of Ulsan College of Medicine Seoul South KoreaDepartment of Internal Medicine Seoul National University College of Medicine and Seoul National University Hospital Seoul South KoreaDivision of Hematology‐Oncology, Department of Medicine Samsung Medical Center, Sungkyunkwan University School of Medicine Seoul South KoreaDivision of Medical Oncology, Department of Internal Medicine Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea Seoul South KoreaDivision of Hematology‐Oncology, Department of Medicine Chung‐Ang University College of Medicine Seoul South KoreaDivision of Hemato‐Oncology, Department of Internal Medicine Pusan National University School of Medicine Busan South KoreaDivision of Hematology/Oncology, Department of Internal Medicine College of Medicine, Chungnam National University Daejeon South KoreaDivision of Hematology‐Oncology, Department of Internal Medicine Korea University Guro Hospital, Korea University College of Medicine Seoul South KoreaDivision of Hematology‐Oncology, Department of Internal Medicine Dankook University College of Medicine Cheonan South KoreaDepartment of Oncology/Hematology Kyungpook National University Chilgok Hospital, School of Medicine, Kyungpook National University Daegu South KoreaDepartment of Internal Medicine Dong‐A University College of Medicine Busan South KoreaDepartment of Hematology‐Oncology Ewha Womans University Medical Center Seoul South KoreaDepartment of Hematological Oncology Hallym University Sacred Heart Hospital, Hallym University College of Medicine Anyang‐si South KoreaAbstract Background Extramammary Paget's disease (EMPD) is rare. There are no standard treatments due to its rarity and few clinical trials. Methods The objective of this multicenter study was to investigate treatment outcomes of Korean patients with advanced/metastatic EMPD. Data were collected retrospectively from 14 institutions participating in Korean Cancer Study Group (KCSG) Rare Cancer Committee. Results A total of 37 patients were identified. Of these 37 patients, 6 received locoregional therapy as a first‐line treatment. In 31 patients who received systemic chemotherapy as a first‐line treatment, platinum‐based chemotherapy (n = 22) achieved an objective response rate (ORR) of 45.5% and a median progression‐free survival (PFS) of 7.89 months. Taxane‐based chemotherapy (n = 8) achieved an objective response rate of 62.5% and median PFS of 9.73 months. In second‐line chemotherapy, platinum‐based chemotherapy (n = 4) had a disease control rate (DCR) of 75.0% and median PFS of 3.45 months. Taxane‐based chemotherapy (n = 8) had a DCR of 75.0% and a median PFS of 8.67 months. Six patients received anti‐human epidermal growth factor receptor 2 (HER2) antibody during first‐ and second‐line chemotherapy. Overall, systemic chemotherapy combined with anti‐HER2 antibody had an ORR of 100% and a median PFS of 13.31 months. The ORR and PFS with systemic chemotherapy combined with trastuzumab was better than platinum‐ and taxane‐based chemotherapy only. Conclusions Due to its rarity, advanced or metastatic EMPD still has no established standard treatment. Results of our study indicate that the combination of trastuzumab with taxane has longer survival than trastuzumab monotherapy or conventional platinum‐ or taxane‐based chemotherapy.https://doi.org/10.1002/cam4.6190chemotherapymetastatic extramammary Paget's diseaseoverall survivalprogression‐free survivaltrastuzumab
spellingShingle Byeong Seok Sohn
Jeongeun Kim
Miso Kim
Jung Yong Hong
Jieun Lee
Song Ee Park
Hyojeong Kim
Hyo Jin Lee
Eun Joo Kang
Soon Il Lee
In Hee Lee
Seok Jae Huh
Jeongmin Jo
Ho Young Kim
Treatment outcomes of advanced/metastatic extramammary Paget's disease in Korean patients: KCSG‐RC20‐06
Cancer Medicine
chemotherapy
metastatic extramammary Paget's disease
overall survival
progression‐free survival
trastuzumab
title Treatment outcomes of advanced/metastatic extramammary Paget's disease in Korean patients: KCSG‐RC20‐06
title_full Treatment outcomes of advanced/metastatic extramammary Paget's disease in Korean patients: KCSG‐RC20‐06
title_fullStr Treatment outcomes of advanced/metastatic extramammary Paget's disease in Korean patients: KCSG‐RC20‐06
title_full_unstemmed Treatment outcomes of advanced/metastatic extramammary Paget's disease in Korean patients: KCSG‐RC20‐06
title_short Treatment outcomes of advanced/metastatic extramammary Paget's disease in Korean patients: KCSG‐RC20‐06
title_sort treatment outcomes of advanced metastatic extramammary paget s disease in korean patients kcsg rc20 06
topic chemotherapy
metastatic extramammary Paget's disease
overall survival
progression‐free survival
trastuzumab
url https://doi.org/10.1002/cam4.6190
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