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

• Main Authors: Satoru Tsuruta, Norihisa Kimura, Keinosuke Ishido, Daisuke Kudo, Kentaro Sato, Tetsu Endo, Tadashi Yoshizawa, Aoi Sukeda, Nobuyoshi Hiraoka, Hiroshi Kijima, Kenichi Hakamada
• Format: Article
• Language: English
• Published: BMC 2018-11-01
• Series: World Journal of Surgical Oncology
• Subjects: Calcifying nested stromal epithelial tumor (CNSET); Hormone imbalance; Klinefelter syndrome; Liver; Neoplasm
• Online Access: http://link.springer.com/article/10.1186/s12957-018-1528-x

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.