Surgical therapy and next-generation sequencing-based genetic alteration analysis of malignant solitary fibrous tumor of the pleura

Abstract

BackgroundSolitary fibrous tumor of the pleura (SFTP) is a rare pleural neoplasm arising from mesenchymal cells, accounting for <5% of pleural neoplasms. Approximately 10% of cases of SFTP demonstrate malignant potential, leading to local recurrence after radical surgery and subsequent metastasis.MethodsA large malignant-like mass was found in the left thoracic cavity of a 61-year-old woman. Following radical resection of the mass, the patient was diagnosed with malignant SFTP by histologic and immunohistochemical analyses. In addition, a next-generation sequencing-based mutation test was used to reveal the mutational profile of the tumor. The genetic alteration panel was analyzed with reference to public data on the ClinVar and COSMIC databases, after which the public SFTP data were analyzed for frequency of altered genes. Finally, through overlay of the abovementioned two sets, the genetic alteration accounting for SFTP initiation was anticipated to be identified.ResultsIn the mutation panel of our malignant SFTP group, kinase insert domain receptor (KDR) and fms-related tyrosine kinase 1 (FLT1) scored high in pathogenesis but had only a medium frequency; the NAB2–STAT6 fusion appeared to be the dominant genetic alteration in public SFTP samples.ConclusionThe high frequency of NAB2–STAT6 fusion indicates its prominent role in SFTP, while somatic mutations such as FLT1-R593W and KDR-V297I may also contribute to the malignant angiogenic phenotype. The present study affirmed the heterogeneity of SFTP, and more sophisticated classification methods will be needed to explore its underlying mechanisms.SummaryWe believe that improvement in the prognosis of SFTP relies on early diagnosis, margin-free resection, and long-term follow-up. Through genetic analysis, it appears that both NAB2–STAT6 fusion and somatic mutations such as FLT1-R593W and KDR-V297I contribute to SFTP development.

Case Report: A Review of the Literature on Spinal Intradural Hemangiopericytoma With Spinal Cord Infiltration and a Case Report

“…Total surgical resection is the preferred treatment for HPC ( 12 , 13 ), which is consistent with the conclusion of our analysis. However, massive bleeding during surgery becomes a barrier to total removal.…”

Pleural Solitary Fibrous Tumors—A Retrospective Study on 45 Patients

“…NAB2-R593W fusion plays a major role in malign PSFT. Meanwhile, FLT1-R593W and KDR-V2971I somatic mutations are proof of the existence of a malignant angiogenetic phenotype [10].…”

“…PSFTs are found within the pleura in 57.7% of cases, with the rest (42.3%) being localized extrapleural [8,10]. The World Health Organization (2015) reviewed the classification of pleural tumors from a pathological view and placed them in three categories: mesothelial tumors, mesenchymal tumors, and lympho-proliferative disorder.…”

Solitary fibrous tumor of the pleura with marked cystic degeneration: a case report

“…The check-valve mechanism may have caused cystic degeneration during the growth of the tumor. On the other hand, it has been reported that genetic alterations analyzed by next-generation sequencing could be involved in malignant transformation in SFTP [ 10 ], and genetic alterations may also be involved in cystic degeneration. To the best of our knowledge, this is the first report of SFTP with cystic degeneration.…”