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骨孤立性纤维性肿瘤的临床、组织学及分子特征:单机构回顾性研究

Clinical, Histological, and Molecular Features of Solitary Fibrous Tumor of Bone: A Single Institution Retrospective Review.

作者信息

Bianchi Giuseppe, Lana Debora, Gambarotti Marco, Ferrari Cristina, Sbaraglia Marta, Pedrini Elena, Pazzaglia Laura, Sangiorgi Luca, Bartolotti Isabella, Dei Tos Angelo Paolo, Scotlandi Katia, Righi Alberto

机构信息

Department of Orthopedic Oncology, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy.

Department of Pathology, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy.

出版信息

Cancers (Basel). 2021 May 19;13(10):2470. doi: 10.3390/cancers13102470.

Abstract

Primary solitary fibrous tumor (SFT) of the bone is extremely rare, with only few cases reported in the literature. We retrieved all cases of primary SFT of the bone treated at our institution and we assessed the morphology and the immunohistochemical and molecular features to investigate the clinical outcome of primary SFT of the bone and any clinical relevance of clinical and histological criteria of aggressiveness currently adopted for the soft tissues counterpart. Morphologically, 15 cases evidenced high cellularity, cytologic atypia, and foci of necrosis and were associated with more than 4 mitotic figures/10 HPF. Immunohistochemical analysis showed an expression of CD34 and of STAT6 immunopositivity in 95% and in 100% of cases, respectively. The presence of chimeric transcripts was found in 10 out of 12 cases in which RT-PCR analysis was feasible, whereas promoter mutations analysis was feasible in 16 cases and only a C-to-T substitution in a heterozygous state was found in one DNA sample for the C228T genetic variant. variants were assessed in 12 cases: 11 (91.6%) cases showed a variation, while in one case, no alteration was found. Disease-specific survival was 64% at 5 years and 49% at 10 years. Statistical analysis showed no correlation between survival and all the clinicopathological and molecular parameters evaluated. In conclusion, at difference to SFT of soft tissues, aggressive behavior of primary SFT of the bone seems to be independent from mitotic count or any other clinicopathological and molecular features.

摘要

骨原发性孤立性纤维性肿瘤(SFT)极为罕见,文献中仅有少数病例报道。我们检索了在本机构接受治疗的所有骨原发性SFT病例,并评估了其形态学、免疫组化和分子特征,以研究骨原发性SFT的临床结局以及目前用于软组织对应物的侵袭性临床和组织学标准的任何临床相关性。形态学上,15例表现为细胞密度高、细胞异型性和坏死灶,且每10个高倍视野有超过4个有丝分裂象。免疫组化分析显示,分别有95%和100%的病例表达CD34和STAT6免疫阳性。在12例可行逆转录聚合酶链反应(RT-PCR)分析的病例中,有10例发现嵌合转录本;在16例可行启动子突变分析的病例中,仅在1份DNA样本中发现C228T基因变异的杂合状态下有一个C到T的替换。在12例病例中评估了变异情况:11例(91.6%)有变异,而1例未发现改变。5年疾病特异性生存率为64%,10年为49%。统计分析显示生存率与所有评估的临床病理和分子参数之间无相关性。总之,与软组织SFT不同,骨原发性SFT的侵袭性行为似乎与有丝分裂计数或任何其他临床病理和分子特征无关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/311d/8158730/a87bc5c1fc76/cancers-13-02470-g001.jpg

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