AUTHOR=Li Zhiwen , Pu Xiaohong , He Lu , Fu Yao , Li Lin , Xu Yuemei , Guan Wenyan , Fan Xiangshan 

TITLE=Malignant Gastrointestinal Neuroectodermal Tumor in the Right Heart: A Report of an Extremely Rare Case Presenting With a Cardiac Mass

JOURNAL=Frontiers in Cardiovascular Medicine

VOLUME=Volume 8 - 2021

YEAR=2021

URL=https://www.frontiersin.org/journals/cardiovascular-medicine/articles/10.3389/fcvm.2021.702215

DOI=10.3389/fcvm.2021.702215

ISSN=2297-055X

ABSTRACT=Malignant gastrointestinal neuroectodermal tumor (GNET) is an extremely rare soft tissue sarcoma, and has been designated as a new entity recently. At present, GNET virtually exclusively occurs in gastrointestinal tract. Here, we report a case of extra-gastrointestinal GNET that arose in the right heart. A 62-year-old male complained of chest distress and breathing difficulty while lying down at night for over one month at admission. Radiological findings revealed an occupying lesion involving the right atrium and right ventricle without any abdominal abnormalities. The patient then underwent the surgical resection. Microscopically, neoplastic cells proliferated in the pattern of nests and sheets with fibrous separation. Focal areas with cellular dyscohesion imparted a vague pseudopapillary pattern. These tumor cells were small to medium-sized with fine chromatin and predominantly pale eosinophilic cytoplasm. Nuclei were typically round to oval with somewhat irregular contours and contained small nucleoli. Mitotic figures were easily found. Immunohistochemically, the neoplastic cells were positive for S100 and SOX-10 but negative for HMB-45, A103 and CD99. EWSR1-AFTF1 rearrangement was detected by fluorescence in situ hybridization and further confirmed by whole transcriptome sequence analysis. The patient had pulmonary metastasis eight months later and soon died of the disease. The overall survival of the patient was 20 months. In summary, we reported an extremely rare case of cardiac GNET indicating that the location of GNET should not be confined to GI tract as initially defined. Due to the lack of specific effective treatment and the occurrence of early metastasis, cardiac GNET conferred a poor prognosis. More clinical and experimental studies are warranted to better manage this disease in future.