Background: Primary pleural and pulmonary synovial sarcomas (PPSSs) are very rare and aggressive neoplasms that affect adults. The oncologic characteristics, treatment, and prognosis for PPSSs are not well defined because of a paucity of data. Dysregulation of Wnt/β-catenin and EGFR pathways leads to tumorigenesis with poor prognosis. We investigated the involvement of Wnt/β-catenin and EGFR signaling in PPSSs. Methods: This retrospective study identified seven PPSS cases at the Department of Thoracic Surgery, SS. Annunziata Hospital in Chieti from 2002 to 2015. Paraffin tumour tissues were evaluated for protein and gene expression by IHC and RT-qPCR. Results: The retrospective study for PPSS reveals by IHC 5 biphasic and 2 monophasic phenotypes with minimal focal epithelial component. Mesenchymal component shown vimentin expression in all PPSSs while CkAE1/AE3, EMA, E-cadherin and β-catenin were absent in the two monophasic PPSS. SYT–SSX1 fusion transcripts were found in 3 out of 7 PPSSs. Using one of the pleural normal tissues with a lower Ct value as a calibrator, RT-qPCR showed very strong expression in LEF1 trascripts in all tumors. APC increased in one pulmonary PPSS whereas the other PPSS showed a normal range. EGFR was overexpressed in all samples. Interestingly, gene expression indicate a normal range of ERB-b2 and ERB-b3 expression for one pleural monophasic sample, while ERB-b4 was over expressed. Conclusions: Preliminary results confirmed the involvement of Wnt/β-catenin and EGFR pathways in PPSSs and highlight unanswered questions, the solutions of which will be imperative in the rational exploration of these pathways in future molecular treatment strategies.
Primary pleuro-pulmonary synovial sarcoma: a single-center 13-year experience
DI RUSSO, SIMONE;DE LUCA, GRAZIANO;MOSCATELLO, CARMELO;DI MARCANTONIO, Maria Carmela;MINCIONE, Gabriella;MARCHETTI, Antonio;BATTISTA, Pasquale;MUCILLI, Felice
2016-01-01
Abstract
Background: Primary pleural and pulmonary synovial sarcomas (PPSSs) are very rare and aggressive neoplasms that affect adults. The oncologic characteristics, treatment, and prognosis for PPSSs are not well defined because of a paucity of data. Dysregulation of Wnt/β-catenin and EGFR pathways leads to tumorigenesis with poor prognosis. We investigated the involvement of Wnt/β-catenin and EGFR signaling in PPSSs. Methods: This retrospective study identified seven PPSS cases at the Department of Thoracic Surgery, SS. Annunziata Hospital in Chieti from 2002 to 2015. Paraffin tumour tissues were evaluated for protein and gene expression by IHC and RT-qPCR. Results: The retrospective study for PPSS reveals by IHC 5 biphasic and 2 monophasic phenotypes with minimal focal epithelial component. Mesenchymal component shown vimentin expression in all PPSSs while CkAE1/AE3, EMA, E-cadherin and β-catenin were absent in the two monophasic PPSS. SYT–SSX1 fusion transcripts were found in 3 out of 7 PPSSs. Using one of the pleural normal tissues with a lower Ct value as a calibrator, RT-qPCR showed very strong expression in LEF1 trascripts in all tumors. APC increased in one pulmonary PPSS whereas the other PPSS showed a normal range. EGFR was overexpressed in all samples. Interestingly, gene expression indicate a normal range of ERB-b2 and ERB-b3 expression for one pleural monophasic sample, while ERB-b4 was over expressed. Conclusions: Preliminary results confirmed the involvement of Wnt/β-catenin and EGFR pathways in PPSSs and highlight unanswered questions, the solutions of which will be imperative in the rational exploration of these pathways in future molecular treatment strategies.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
