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  • Prior studies describe FDG PET scans


    Prior studies describe FDG-PET scans as best suitable, i.e. FDG positive, in tumours exceeding Ki67 index of 10 and 20% [27]. The hitherto unseen high portion of positive FDG-PET scans in patients with TC and AC found in this study challenges this conception and should be taken into account in future imaging guidelines for these types of tumours. New imaging modalities using radiolabeled somatostatin analogues such as the 68Ga-DOTATOC are gaining impact in the imaging of BP-NETs due to high resolution and sensitivity [28,29]. However, the FDG-PET scan still has great value as first-line clinical imaging in visualizing location and activity of tumours and metastasis. Some studies suggest that SUVmax can be used as predictor of death and recurrence though this needs to be further confirmed before final conclusions can be made [30]. Due to Danish national guidelines concerning guaranty of treatment after a certain GSK1838705A after diagnosis, only 12% of our patients had somatostatin imaging done before treatment. However, all patients with TC and AC had a postoperative 68Ga-DOTATOC scan performed to verify that no residual disease was present. Survival analysis showed a correlation between tumour type and overall survival. Patients with TC had the best prognosis with 5-year survival rates of 87% compared to 63% for the AC group and 22% for the LCNECs. These results correlate well with analysis of overall survival from prior studies [31,32]. Survival analysis showed a significant difference in survival rates between patients who underwent surgery and patients who did not. Survival rates in the operated group mimic those found in previous studies and in the literature with 5-year survival rates of 97% in patients with TC and 78% in patients with AC [30] compared with findings in this paper with 5-year survival in operated TC and AC patients of 93% and 87%, respectively. It could be argued that stage explains the observed difference in OS between operated and non-operated patients. However, when performing multivariate analysis, taking into account N and M stage, operation still remained an independent factor for OS in the current study. Although only a small proportion of patients with TC had N2 stage disease in the current cohort, we were still able to show a difference in survival between operated and non-operated patients, indicating that Endonucleases should be consided to operate TC patients with N2 stage disease if otherwise acceptable from a clinical point of view.
    Conflict of interest
    Funding Danish Cancer Society, Denmark.
    Introduction SMARCA4 is one of the core catalytic subunits of the SWI/SNF complex and its inactivation has been reported in several aggressive tumors with high-grade undifferentiated rhabdoid morphology including small cell carcinoma of the ovary, hypercalcemic type (SCCOHT) [1]. Recent genetic analysis revealed a group of undifferentiated thoracic malignancies with SMARCA4 inactivation presenting as compressive tumors often involving the mediastinum, with or without lung involvement, and occurring in relatively young patients and displaying aggressive behavior with poor prognosis [2]. These tumors were reported to be distinct from SMARCA4-mutated lung carcinomas and to have a closer molecular relationship with malignant rhabdoid tumors and SCCOHT. Because of these clinicopathological and molecular differences, these SMARCA4-inactivated thoracic tumors were termed ‘SMARCA-deficient thoracic sarcoma (SMARCA4-DTS)’ [2]. Previous reports of SMARCA4-DTS were mostly based on retrospective histopathological reviews with exhaustive immunohistochemical work-ups and next-generation sequencing; accordingly, the detailed clinical course of SMARCA4-DTS, including sensitivity to chemotherapy and radiotherapy, remains unclear [2,3]. Here, we report the clinical courses and molecular profiles of two cases of SMARCA4-DTS. Both patients suffered from severe skeletal related events (SREs) which required emergent surgical and radiological therapies. Severe SREs could thus be a new significant clinical feature of SMARCA4-DTS.