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成功进行肝移植治疗不可切除的促结缔组织增生性巢状梭形细胞瘤并发库欣综合征。

Successful liver transplantation for non-resectable desmoplastic nested spindle cell tumor complicated by Cushing's syndrome.

作者信息

Tehseen Sarah, Rapkin Louis, Schemankewitz Erwin, Magliocca Joseph F, Romero Rene

机构信息

Department of Pediatrics, Division of Pediatric Hematology Oncology (Aflac Cancer and Blood Disorders Center), Emory University, Atlanta, GA, USA.

Department of Pediatrics, Division of Pediatric Gastroenterology, Children's Healthcare of Atlanta, Emory University, Atlanta, GA, USA.

出版信息

Pediatr Transplant. 2017 Sep;21(6). doi: 10.1111/petr.13000. Epub 2017 Jun 18.

Abstract

Desmoplastic spindle cell tumors of liver are rare tumors of low malignant potential characterized by well-demarcated nests of spindle and epithelioid cells in a dense desmoplastic stroma. While surgery remains the definitive treatment, there have been reports of tumor recurrence locally and metastasis which respond poorly to chemotherapy. Hepatic transplant has been attempted in cases of recurrence or large size of primary tumor. Long-term follow-up and imaging surveillance are required as these tumors have shown a tendency for recurrence many years after initial therapy.

摘要

肝脏促纤维增生性梭形细胞肿瘤是一种恶性潜能较低的罕见肿瘤,其特征为在致密的促纤维增生性基质中存在界限清楚的梭形细胞巢和上皮样细胞巢。虽然手术仍然是 definitive 治疗方法,但已有肿瘤局部复发和转移的报道,这些对化疗反应不佳。对于复发或原发性肿瘤体积较大的病例,已尝试进行肝移植。由于这些肿瘤在初始治疗多年后仍有复发倾向,因此需要长期随访和影像学监测。

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