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Ex situ hepatectomy and liver autotransplantation for a treating giant solitary fibrous tumor: A case report.

ABSTRACT: A solitary fibrous tumor (SFT) is a rare mesenchymal tumor. Ex situ hepatectomy and liver autotransplantation are novel methods for the treatment of complicated liver tumors, for example, those involving vascular structures, including the inferior vena cava, which are unresectable by conventional approaches. The present study describes a rare case of a massive hepatic SFT in a 32-year-old female who underwent ex situ hepatectomy and liver autotransplantation to achieve a radical resection. The surgery was without complications. Post-operative histopathological and immunohistochemical examinations revealed an SFT of the liver. The patient was discharged 29 days after the surgery with fully recovered liver function. The routine check-up 3 months after surgery indicated normal liver function and no evidence of recurrence. Additionally, an exhaustive review of available literature was performed to provide a complete overview of the current status of SFTs. In summary, the present study found that ex situ hepatectomy and liver autotransplantation are suitable surgical techniques for treating a giant SFT, as well as other liver neoplasms that are considered unresectable by conventional surgery.

SUBMITTER: Sun Z

PROVIDER: S-EPMC6312964 | biostudies-other | 2019 Jan

REPOSITORIES: biostudies-other

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Project description:Solitary fibrous tumors (SFTs) are NAB2-STAT6 fusion-associated neoplasms. There are several subtypes of NAB2-STAT6 fusions, but their clinical significances are still unclear. Moreover, the mechanisms of malignant progression are also poorly understood. In this study, using 91 SFT cases, we examined whether fusion variants are associated with clinicopathological parameters and also investigated the molecular mechanism of malignant transformation using whole-exome sequencing. We detected variant 1b (NAB2ex4-STAT6ex2) in 51/91 (56%) cases and variants 2a/2b (NAB2ex6-STAT6ex16/17) in 17/91 (19%) cases. The NAB2-STAT6 fusion variant types were significantly associated with their primary site (P < 0.001). In addition, a TERT promoter mutation was detected in 7/73 (10%) cases, and it showed a significant association with malignant SFTs (P = 0.003). To identify molecular changes during malignant progression, we selected an index patient to obtain parallel tissue samples from the primary and metastatic tumors. In the metastatic tissue, 10 unique molecular alterations, including those in TP53 and APAF1, were detected. In vitro functional experiments showed that APAF1 depletion increased the tumor potency of cells expressing NAB2-STAT6 fusion protein under treatment with staurosporine. We found that TP53 immunopositivity (P = 0.006) and loss of APAF1 immunoreactivity (P < 0.001) were significantly associated with malignant SFTs. Our study suggests that dysfunction of TP53 and APAF1 leads to impaired apoptotic function, and eventually contributes toward malignant SFT transformation. KEY MESSAGES: We firstly found that the TERT promoter mutation was strongly associated with malignant SFTs (P = 0.003) and the representative 1b (NAB2ex4-STAT6ex2) or 2a (NAB2ex6-STAT6ex16) fusion variants similarly contribute to tumorigenicity. We also found that TP53 immunopositivity (P = 0.006) and loss of APAF1 immunoreactivity (P < 0.001) were significantly associated with malignant SFTs. Our study suggests that dysfunction of TP53 and APAF1 leads to impaired apoptotic function, and eventually contributes toward malignant SFT transformation.

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Ex situ hepatectomy and liver autotransplantation for a treating giant solitary fibrous tumor: A case report.

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