Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan.
Department of Gastroenterology and Hematology, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan.
World J Surg Oncol. 2018 Nov 19;16(1):227. doi: 10.1186/s12957-018-1528-x.
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.
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.
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.
甲状旁腺巢状间质上皮瘤（CNSET）是一种肝脏原发性肿瘤，其特征为边界清楚的巢状结构，由梭形和上皮样细胞组成，伴有钙化和骨形成。库欣综合征与 CNSET 之间的关联引起了人们的关注，但 CNSET 的起源尚未阐明。
我们在此报告一例 20 岁的克氏综合征男性患者，因进行性增大的肝肿瘤行肝切除术，术后病理诊断为 CNSET。患者术后接受了随访和多次检查，在术后第 64 天发现复发和肝外淋巴结转移。化疗栓塞和化疗均无效，导致肿瘤进展并出现进行性肝功能衰竭，患者最终在肝切除术后 164 天死亡。
本病例提示激素失衡可能影响 CNSET 的发生和进展，因此对于 CNSET 患者，通过影像学密切随访对于关注肝内复发和肝外转移非常重要。
