Clinicopathologic features and BRAF mutation status of tracheal glomus tumors-Characterization of 4 cases and the distinction from low-grade neuroendocrine tumors
单位：[a]Departments of Pathology, China-Japan Friendship Hospital, Beijing, China[b]Departments of Pathology, Peking Union Medical College Hospital, Beijing, China[c]Departments of Radiology, China-Japan Friendship Hospital, Beijing, China[d]Departments of Thoracic Surgery, China-Japan Friendship Hospital, Beijing, China[e]Departments of Respiratory and Critical Care Medicine, China-Japan Friendship Hospital, Beijing, China
Background: Glomus tumors are uncommon and mostly benign mesenchymal neoplasms of the perivascular family. To date, only a few cases of glomus tumors occurring in the trachea have been reported. Tracheal glomus tumors simulated low-grade neuroendocrine tumors on clinical and histomorphological examination, so the differential diagnosis between these two entities is very necessary. The latest studies showed that BRAF mutation may be associated with a malignant phenotype of glomus tumors. Methods: We investigated the clinical, histopathologic, immunohistochemical, and BRAF V600E mutation status of four cases of tracheal glomus tumors. Results: The cases showed a female predilection (male:female, 1:3) with a median age of 35.5. All of the cases had the typical morphological characteristics of glomus tumors, such as uniform round tumor cells with nest-like distribution surrounding thin-walled vessels; two of them met the malignant diagnostic criteria based on the 5th edition of WHO classification, including marked nuclear atypia and any level of mitotic activity. Immunohistochemistry showed diffusely positive for vimentin (4/4), alpha-SMA (4/4) and collagen IV (4/4), variably reactive for synaptophysin (3/4) and SSTR2 (2/2), and negative for AE1/AE3 (0/4) and chromogranin A (0/4). Three tested cases harbored no BRAF V600E mutation. Three follow-up cases were alive and free of disease with an average follow-up of 89.3 months. Conclusions: Tracheal glomus tumors are rare mesenchymal tumors that have overlapping morphologic and immunohistochemical features with neuroendocrine neoplasms. Our cases highlight the importance of careful histomorphological examination and comprehensive immunohistochemical study in reaching a correct diagnosis of glomus tumors of the trachea. Other than BRAF mutation, malignant glomus tumors may have a complex mutational profile.
Jie Lin,Jie Luo,Huang Chen,et al.Clinicopathologic features and BRAF mutation status of tracheal glomus tumors-Characterization of 4 cases and the distinction from low-grade neuroendocrine tumors[J].ANNALS of DIAGNOSTIC PATHOLOGY.2021,55:doi:10.1016/j.anndiagpath.2021.151797.
APA：
Jie Lin,Jie Luo,Huang Chen,Zhiyuan Li,Yanfen Shi...&Dingrong Zhong.(2021).Clinicopathologic features and BRAF mutation status of tracheal glomus tumors-Characterization of 4 cases and the distinction from low-grade neuroendocrine tumors.ANNALS of DIAGNOSTIC PATHOLOGY,55,
MLA：
Jie Lin,et al."Clinicopathologic features and BRAF mutation status of tracheal glomus tumors-Characterization of 4 cases and the distinction from low-grade neuroendocrine tumors".ANNALS of DIAGNOSTIC PATHOLOGY 55.(2021)
