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Open Access 01-12-2018 | Case report

Calcifying nested stromal epithelial tumor of the liver in a patient with Klinefelter syndrome: a case report and review of the literature

Authors: Satoru Tsuruta, Norihisa Kimura, Keinosuke Ishido, Daisuke Kudo, Kentaro Sato, Tetsu Endo, Tadashi Yoshizawa, Aoi Sukeda, Nobuyoshi Hiraoka, Hiroshi Kijima, Kenichi Hakamada

Published in: World Journal of Surgical Oncology | Issue 1/2018

Abstract

Background

Calcifying nested stromal epithelial tumor (CNSET) is a primary neoplasm of the liver, characterized by well-demarcated nests consisting of spindle and epithelioid cells with calcification and bone formation. An association of Cushing syndrome with CNSET has drawn attention, but the origin of CNSET has not been clarified.

Case presentation

We report here the case of a 20-year-old male with Klinefelter syndrome who underwent liver resection for an increasing liver tumor that was pathologically diagnosed with CNSET. He was postoperatively followed up and received several examinations, and recurrences and extrahepatic lymph node metastases were detected on the 64th day after surgery. Chemoembolization and chemotherapy were not effective, leading to tumor progression with development of progressive liver failure, and the patient finally died 164 days after hepatectomy.

Conclusions

This case suggests that an imbalance of hormones affects the genesis and progression of CNSET, and indicates the importance of closely following patients with CNSET by imaging with attention to hepatic recurrence and extrahepatic metastases.

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Title: Calcifying nested stromal epithelial tumor of the liver in a patient with Klinefelter syndrome: a case report and review of the literature
Authors: Satoru Tsuruta
Norihisa Kimura
Keinosuke Ishido
Daisuke Kudo
Kentaro Sato
Tetsu Endo
Tadashi Yoshizawa
Aoi Sukeda
Nobuyoshi Hiraoka
Hiroshi Kijima
Kenichi Hakamada
Publication date: 01-12-2018
Publisher: BioMed Central
Published in: World Journal of Surgical Oncology / Issue 1/2018
Electronic ISSN: 1477-7819
DOI: https://doi.org/10.1186/s12957-018-1528-x

ACC 2024 Congress

Springer Medicine

Calcifying nested stromal epithelial tumor of the liver in a patient with Klinefelter syndrome: a case report and review of the literature

Abstract

Background

Case presentation

Conclusions

ACC 2024 Congress

Springer Medicine

Abstract

Background

Case presentation

Conclusions

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