Primary monophasic synovial sarcoma of the cervical esophagus confirmed by detection of the SS18-SSX2 fusion transcripts: case report and literature review

Abstract Background Synovial sarcoma (SS) of the esophagus is extremely rare. Because of the microscopic features of SS, the monophasic type can easily be misdiagnosed as other spindle cell tumors. Here, we present the first case of a primary SS of the esophagus in the presence of SS18-SSX2 fusion t...

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Main Authors: Ken Sasaki, Masahiro Noda, Yusuke Tsuruda, Yasuto Uchikado, Itaru Omoto, Yoshiaki Kita, Takaaki Arigami, Shinichiro Mori, Hiroshi Kurahara, Akihiro Nakajo, Michiyo Higashi, Takao Ohtsuka
Format: Article
Language:English
Published: Japan Surgical Society 2020-07-01
Series:Surgical Case Reports
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Online Access:http://link.springer.com/article/10.1186/s40792-020-00940-8
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author Ken Sasaki
Masahiro Noda
Yusuke Tsuruda
Yasuto Uchikado
Itaru Omoto
Yoshiaki Kita
Takaaki Arigami
Shinichiro Mori
Hiroshi Kurahara
Akihiro Nakajo
Michiyo Higashi
Takao Ohtsuka
author_facet Ken Sasaki
Masahiro Noda
Yusuke Tsuruda
Yasuto Uchikado
Itaru Omoto
Yoshiaki Kita
Takaaki Arigami
Shinichiro Mori
Hiroshi Kurahara
Akihiro Nakajo
Michiyo Higashi
Takao Ohtsuka
author_sort Ken Sasaki
collection DOAJ
description Abstract Background Synovial sarcoma (SS) of the esophagus is extremely rare. Because of the microscopic features of SS, the monophasic type can easily be misdiagnosed as other spindle cell tumors. Here, we present the first case of a primary SS of the esophagus in the presence of SS18-SSX2 fusion transcripts. Case presentation A 47-year-old Japanese woman was initially diagnosed with thyroid papillary carcinoma in the left lobe and leiomyoma of the cervical esophagus and subsequently underwent left thyroid lobectomy and enucleation of the esophageal tumor. Four years after the first surgery, the esophageal tumor recurred. Endoscopic biopsy of the tumor revealed atypical cell proliferation with spindle cell features and mitoses. Immunohistochemistry showed focal positivity for bcl-2 and HHF35. Furthermore, the presence of SS18-SSX2 fusion transcripts was confirmed by reverse transcription-polymerase chain reaction analysis, using a paraffin-embedded tumor specimen. Therefore, the tumor was diagnosed as monophasic SS of the cervical esophagus. We re-evaluated the surgical specimen enucleated 3 years previously, which was initially diagnosed as leiomyoma, and the diagnosis of SS was confirmed. The patient underwent cervical esophagectomy with isolated jejunal interposition reconstruction. Three years after the second surgery, SS recurred in the distal anastomotic site between the jejunum and the esophagus, and the patient underwent thoracoscopic esophagectomy with gastric conduit reconstruction. The pathological grade of the lesion worsened with every recurrence. Conclusions Monophasic SS can be difficult to discriminate from other spindle cell tumors based on microscopy alone, and molecular analysis could be useful for confirming the precise diagnosis of monophasic SS.
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spelling doaj-art-4e1ff1f544154b09acff35bd3949b1582025-08-20T02:52:48ZengJapan Surgical SocietySurgical Case Reports2198-77932020-07-01611610.1186/s40792-020-00940-8Primary monophasic synovial sarcoma of the cervical esophagus confirmed by detection of the SS18-SSX2 fusion transcripts: case report and literature reviewKen Sasaki0Masahiro Noda1Yusuke Tsuruda2Yasuto Uchikado3Itaru Omoto4Yoshiaki Kita5Takaaki Arigami6Shinichiro Mori7Hiroshi Kurahara8Akihiro Nakajo9Michiyo Higashi10Takao Ohtsuka11Department of Digestive Surgery, Breast and Thyroid Surgery, Graduate School of Medical and Dental Sciences, Kagoshima UniversityDepartment of Digestive Surgery, Breast and Thyroid Surgery, Graduate School of Medical and Dental Sciences, Kagoshima UniversityDepartment of Digestive Surgery, Breast and Thyroid Surgery, Graduate School of Medical and Dental Sciences, Kagoshima UniversityDepartment of Digestive Surgery, Breast and Thyroid Surgery, Graduate School of Medical and Dental Sciences, Kagoshima UniversityDepartment of Digestive Surgery, Breast and Thyroid Surgery, Graduate School of Medical and Dental Sciences, Kagoshima UniversityDepartment of Digestive Surgery, Breast and Thyroid Surgery, Graduate School of Medical and Dental Sciences, Kagoshima UniversityDepartment of Onco-Biological Surgery, Graduate School of Medical and Dental Sciences, Kagoshima UniversityDepartment of Digestive Surgery, Breast and Thyroid Surgery, Graduate School of Medical and Dental Sciences, Kagoshima UniversityDepartment of Digestive Surgery, Breast and Thyroid Surgery, Graduate School of Medical and Dental Sciences, Kagoshima UniversityDepartment of Digestive Surgery, Breast and Thyroid Surgery, Graduate School of Medical and Dental Sciences, Kagoshima UniversityDepartment of Pathology, Graduate School of Medical and Dental Sciences, Kagoshima UniversityDepartment of Digestive Surgery, Breast and Thyroid Surgery, Graduate School of Medical and Dental Sciences, Kagoshima UniversityAbstract Background Synovial sarcoma (SS) of the esophagus is extremely rare. Because of the microscopic features of SS, the monophasic type can easily be misdiagnosed as other spindle cell tumors. Here, we present the first case of a primary SS of the esophagus in the presence of SS18-SSX2 fusion transcripts. Case presentation A 47-year-old Japanese woman was initially diagnosed with thyroid papillary carcinoma in the left lobe and leiomyoma of the cervical esophagus and subsequently underwent left thyroid lobectomy and enucleation of the esophageal tumor. Four years after the first surgery, the esophageal tumor recurred. Endoscopic biopsy of the tumor revealed atypical cell proliferation with spindle cell features and mitoses. Immunohistochemistry showed focal positivity for bcl-2 and HHF35. Furthermore, the presence of SS18-SSX2 fusion transcripts was confirmed by reverse transcription-polymerase chain reaction analysis, using a paraffin-embedded tumor specimen. Therefore, the tumor was diagnosed as monophasic SS of the cervical esophagus. We re-evaluated the surgical specimen enucleated 3 years previously, which was initially diagnosed as leiomyoma, and the diagnosis of SS was confirmed. The patient underwent cervical esophagectomy with isolated jejunal interposition reconstruction. Three years after the second surgery, SS recurred in the distal anastomotic site between the jejunum and the esophagus, and the patient underwent thoracoscopic esophagectomy with gastric conduit reconstruction. The pathological grade of the lesion worsened with every recurrence. Conclusions Monophasic SS can be difficult to discriminate from other spindle cell tumors based on microscopy alone, and molecular analysis could be useful for confirming the precise diagnosis of monophasic SS.http://link.springer.com/article/10.1186/s40792-020-00940-8Synovial sarcomaEsophagusSS18-SSX2Monophasic
spellingShingle Ken Sasaki
Masahiro Noda
Yusuke Tsuruda
Yasuto Uchikado
Itaru Omoto
Yoshiaki Kita
Takaaki Arigami
Shinichiro Mori
Hiroshi Kurahara
Akihiro Nakajo
Michiyo Higashi
Takao Ohtsuka
Primary monophasic synovial sarcoma of the cervical esophagus confirmed by detection of the SS18-SSX2 fusion transcripts: case report and literature review
Surgical Case Reports
Synovial sarcoma
Esophagus
SS18-SSX2
Monophasic
title Primary monophasic synovial sarcoma of the cervical esophagus confirmed by detection of the SS18-SSX2 fusion transcripts: case report and literature review
title_full Primary monophasic synovial sarcoma of the cervical esophagus confirmed by detection of the SS18-SSX2 fusion transcripts: case report and literature review
title_fullStr Primary monophasic synovial sarcoma of the cervical esophagus confirmed by detection of the SS18-SSX2 fusion transcripts: case report and literature review
title_full_unstemmed Primary monophasic synovial sarcoma of the cervical esophagus confirmed by detection of the SS18-SSX2 fusion transcripts: case report and literature review
title_short Primary monophasic synovial sarcoma of the cervical esophagus confirmed by detection of the SS18-SSX2 fusion transcripts: case report and literature review
title_sort primary monophasic synovial sarcoma of the cervical esophagus confirmed by detection of the ss18 ssx2 fusion transcripts case report and literature review
topic Synovial sarcoma
Esophagus
SS18-SSX2
Monophasic
url http://link.springer.com/article/10.1186/s40792-020-00940-8
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