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Pediatric primary pleural synovial sarcoma: A unique case report with brief review of literature

机译:小儿原发性胸膜滑膜肉瘤:独特病例报告并简要回顾文献

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Synovial sarcoma (SS) primary to the pleura is an extremely rare tumor. So far, only nine cases have been reported in pediatric patients. However, none of these patients was found to have a conglomeration of tumors. Here, we report a case of 16-year-old female with monophasic SS and synchronous occurrence of left paraspinal ganglioneuroma and a right paraovarian cystadenoma. A next-generation sequencing genetic panel revealed a novel variant of unknown significance in the MET gene. The occurrence of multiple different tumors in a young patient with a novel genetic variant in a known oncogene (MET) may suggest a possibility of a hitherto unknown cancer predisposition syndrome. We also present a brief review of primary pleural SS reported in pediatric patients.
机译:原发于胸膜的滑膜肉瘤(SS)是一种极为罕见的肿瘤。到目前为止,小儿患者仅报道了9例。然而,这些患者均未发现肿瘤聚集。在这里,我们报道一例16岁女性单相性SS,并同时发生左椎旁神经节神经瘤和右卵巢旁囊性腺瘤。下一代测序遗传学小组揭示了MET基因中一个未知意义的新型变体。在已知致癌基因(MET)中具有新遗传变异的年轻患者中,多种不同肿瘤的发生可能提示了迄今未知的癌症易感综合症。我们还简要介绍了小儿患者报告的原发性胸膜SS。

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