Paget’s disease derived in situ from reserve cell hyperplasia, squamous metaplasia, and squamous cell carcinoma of the esophagogastric junction: a case report

Abstract Background Extramammary Paget’s disease (EMPD) of the esophagus is a rare tumor, with most cases originating from invasive adenocarcinoma of the esophagus. Pure esophageal Paget’s disease, in which no underlying invasive carcinoma component is present, is extremely rare. In this report, we...

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Main Authors: Akihiko Sano, Shinji Sakurai, Chika Komine, Yuichi Tabe, Kana Saito, Takaharu Fukasawa, Shinsuke Kiriyama, Hideki Yamamoto, Masachika Tani, Hiroshi Naitoh, Ken Shirabe, Hiroyuki Kuwano
Format: Article
Language:English
Published: Japan Surgical Society 2018-07-01
Series:Surgical Case Reports
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Online Access:http://link.springer.com/article/10.1186/s40792-018-0489-1
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author Akihiko Sano
Shinji Sakurai
Chika Komine
Yuichi Tabe
Kana Saito
Takaharu Fukasawa
Shinsuke Kiriyama
Hideki Yamamoto
Masachika Tani
Hiroshi Naitoh
Ken Shirabe
Hiroyuki Kuwano
author_facet Akihiko Sano
Shinji Sakurai
Chika Komine
Yuichi Tabe
Kana Saito
Takaharu Fukasawa
Shinsuke Kiriyama
Hideki Yamamoto
Masachika Tani
Hiroshi Naitoh
Ken Shirabe
Hiroyuki Kuwano
author_sort Akihiko Sano
collection DOAJ
description Abstract Background Extramammary Paget’s disease (EMPD) of the esophagus is a rare tumor, with most cases originating from invasive adenocarcinoma of the esophagus. Pure esophageal Paget’s disease, in which no underlying invasive carcinoma component is present, is extremely rare. In this report, we describe a case of EMPD of the esophagogastric junction with no evidence of invasive carcinoma. Case presentation An 81-year-old Japanese woman with a 2-week history of abdominal distension presented to our hospital for assessment. Endoscopic examination revealed a mild elevated granular lesion, with a slightly depressed irregular mucosa, in the distal esophagus, with EMPD confirmed by biopsy. Thoracoscopic esophagectomy with lymph node dissection was performed, with Paget cells observed on microscopic examination in the lower part of the esophageal epithelium. Only a few Paget cells stained positively for PAS/Alcian blue. Immunohistochemically, negative staining for CK5 and p63 were identified in the Paget cells, with positive staining for CK7. Furthermore, an intraepithelial squamous cell carcinoma, with squamous metaplasia and reserve cell hyperplasia, was observed in the gastric mucosa of the esophagogastric junction, adjacent to the Paget cells. Conclusions EMPD of the esophagus is a rare disease. We report a case of EMPD that was probably derived from a gastric squamous cell carcinoma, with squamous cell metaplasia and reserve cell hyperplasia, in the esophagogastric junction, which, to our knowledge, is the first report of this type of EMPD in the clinical literature.
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spelling doaj-art-b8e2474e595f47f1a8cfa88dcda6566e2025-08-20T02:51:24ZengJapan Surgical SocietySurgical Case Reports2198-77932018-07-01411710.1186/s40792-018-0489-1Paget’s disease derived in situ from reserve cell hyperplasia, squamous metaplasia, and squamous cell carcinoma of the esophagogastric junction: a case reportAkihiko Sano0Shinji Sakurai1Chika Komine2Yuichi Tabe3Kana Saito4Takaharu Fukasawa5Shinsuke Kiriyama6Hideki Yamamoto7Masachika Tani8Hiroshi Naitoh9Ken Shirabe10Hiroyuki Kuwano11Department of Surgery, Japan Community Healthcare Organization Gunma Central HospitalDepartment of Diagnostic Pathology, Japan Community Healthcare Organization Gunma Central HospitalDepartment of Surgery, Japan Community Healthcare Organization Gunma Central HospitalDepartment of Surgery, Japan Community Healthcare Organization Gunma Central HospitalDepartment of Surgery, Japan Community Healthcare Organization Gunma Central HospitalDepartment of Surgery, Japan Community Healthcare Organization Gunma Central HospitalDepartment of Surgery, Japan Community Healthcare Organization Gunma Central HospitalDepartment of Surgery, Japan Community Healthcare Organization Gunma Central HospitalDepartment of Surgery, Japan Community Healthcare Organization Gunma Central HospitalDepartment of Surgery, Japan Community Healthcare Organization Gunma Central HospitalDepartment of General Surgical Science, Gunma University Graduate School of MedicineDepartment of General Surgical Science, Gunma University Graduate School of MedicineAbstract Background Extramammary Paget’s disease (EMPD) of the esophagus is a rare tumor, with most cases originating from invasive adenocarcinoma of the esophagus. Pure esophageal Paget’s disease, in which no underlying invasive carcinoma component is present, is extremely rare. In this report, we describe a case of EMPD of the esophagogastric junction with no evidence of invasive carcinoma. Case presentation An 81-year-old Japanese woman with a 2-week history of abdominal distension presented to our hospital for assessment. Endoscopic examination revealed a mild elevated granular lesion, with a slightly depressed irregular mucosa, in the distal esophagus, with EMPD confirmed by biopsy. Thoracoscopic esophagectomy with lymph node dissection was performed, with Paget cells observed on microscopic examination in the lower part of the esophageal epithelium. Only a few Paget cells stained positively for PAS/Alcian blue. Immunohistochemically, negative staining for CK5 and p63 were identified in the Paget cells, with positive staining for CK7. Furthermore, an intraepithelial squamous cell carcinoma, with squamous metaplasia and reserve cell hyperplasia, was observed in the gastric mucosa of the esophagogastric junction, adjacent to the Paget cells. Conclusions EMPD of the esophagus is a rare disease. We report a case of EMPD that was probably derived from a gastric squamous cell carcinoma, with squamous cell metaplasia and reserve cell hyperplasia, in the esophagogastric junction, which, to our knowledge, is the first report of this type of EMPD in the clinical literature.http://link.springer.com/article/10.1186/s40792-018-0489-1Paget’s diseaseEsophagogastric junctionReserve cell hyperplasiaSquamous metaplasia
spellingShingle Akihiko Sano
Shinji Sakurai
Chika Komine
Yuichi Tabe
Kana Saito
Takaharu Fukasawa
Shinsuke Kiriyama
Hideki Yamamoto
Masachika Tani
Hiroshi Naitoh
Ken Shirabe
Hiroyuki Kuwano
Paget’s disease derived in situ from reserve cell hyperplasia, squamous metaplasia, and squamous cell carcinoma of the esophagogastric junction: a case report
Surgical Case Reports
Paget’s disease
Esophagogastric junction
Reserve cell hyperplasia
Squamous metaplasia
title Paget’s disease derived in situ from reserve cell hyperplasia, squamous metaplasia, and squamous cell carcinoma of the esophagogastric junction: a case report
title_full Paget’s disease derived in situ from reserve cell hyperplasia, squamous metaplasia, and squamous cell carcinoma of the esophagogastric junction: a case report
title_fullStr Paget’s disease derived in situ from reserve cell hyperplasia, squamous metaplasia, and squamous cell carcinoma of the esophagogastric junction: a case report
title_full_unstemmed Paget’s disease derived in situ from reserve cell hyperplasia, squamous metaplasia, and squamous cell carcinoma of the esophagogastric junction: a case report
title_short Paget’s disease derived in situ from reserve cell hyperplasia, squamous metaplasia, and squamous cell carcinoma of the esophagogastric junction: a case report
title_sort paget s disease derived in situ from reserve cell hyperplasia squamous metaplasia and squamous cell carcinoma of the esophagogastric junction a case report
topic Paget’s disease
Esophagogastric junction
Reserve cell hyperplasia
Squamous metaplasia
url http://link.springer.com/article/10.1186/s40792-018-0489-1
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