Haihui Jiang1,2, Kaiyuan Yang1,2, Xiaohui Ren1,2, Yong Cui1,2, Mingxiao Li1,2, Yifei Lei3, Song Lin1,2. 1. Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, China. 2. National Clinical Research Center for Neurological Diseases, Center of Brain Tumor, Beijing Institute for Brain Disorders and Beijing Key Laboratory of Brain Tumor, Beijing, China. 3. Qingdao University, Qingdao, China.
Abstract
BACKGROUND: Diffuse midline glioma (DMG), H3 K27M-mutant occurs in both adult and pediatric populations. The characteristics of the two DMG groups were systematically explored in this study. METHODS: One-hundred-sixteen patients diagnosed with H3 K27M-mutant DMG in Beijing Tiantan Hospital from May 2016 to December 2018 were included. Patients were classified into adult group (n=57, 49.1%) and pediatric group (n=59, 50.9%). The clinical, radiological and molecular features were compared between the groups. Univariate and multivariate analyses were performed to identify prognostic factors. RESULTS: Compared with the adult group, pediatric patients had a younger age (8.9±4.1 vs. 35.1±11.8 years, P<0.001), a lower preoperative Karnofsky performance scale score (62.9±15.5 vs. 72.1±16.5, P=0.004), a lower rate of total resection (5.7% vs. 26.8%, P=0.009), a larger tumor size (4.4±0.9 vs. 3.9±1.5 cm, P=0.045), a higher Ki-67 index (63.0% vs. 37.8%, P=0.047), and higher rates of postoperative cranial nerve palsy (61.0% vs. 36.8%, P=0.009) and ataxia (45.8% vs. 26.3%, P=0.029). Adult DMG was predominantly located in the thalamus, while the predilection site for pediatric DMG was brainstem (P<0.001). Kaplan-Meier plot showed that the median survival of adult and pediatric DMG was 16.0 (9.7-22.3) months and 10.0 (8.3-11.7) months, respectively, which imparted a significant difference (P=0.008). Age at diagnosis, radiotherapy, motor deficit and hydrocephalus were confirmed as independent prognostic factors according to the multivariate analysis (P<0.05). CONCLUSION: Compared with adult patients, children with H3 K27M-mutant DMG confer distinct clinical, radiological and molecular characteristics, and have a dismal prognosis. Radiotherapy is an independent factor associated with prolonged survival.
BACKGROUND: Diffuse midline glioma (DMG), H3 K27M-mutant occurs in both adult and pediatric populations. The characteristics of the two DMG groups were systematically explored in this study. METHODS: One-hundred-sixteen patients diagnosed with H3 K27M-mutant DMG in Beijing Tiantan Hospital from May 2016 to December 2018 were included. Patients were classified into adult group (n=57, 49.1%) and pediatric group (n=59, 50.9%). The clinical, radiological and molecular features were compared between the groups. Univariate and multivariate analyses were performed to identify prognostic factors. RESULTS: Compared with the adult group, pediatric patients had a younger age (8.9±4.1 vs. 35.1±11.8 years, P<0.001), a lower preoperative Karnofsky performance scale score (62.9±15.5 vs. 72.1±16.5, P=0.004), a lower rate of total resection (5.7% vs. 26.8%, P=0.009), a larger tumor size (4.4±0.9 vs. 3.9±1.5 cm, P=0.045), a higher Ki-67 index (63.0% vs. 37.8%, P=0.047), and higher rates of postoperative cranial nerve palsy (61.0% vs. 36.8%, P=0.009) and ataxia (45.8% vs. 26.3%, P=0.029). Adult DMG was predominantly located in the thalamus, while the predilection site for pediatric DMG was brainstem (P<0.001). Kaplan-Meier plot showed that the median survival of adult and pediatric DMG was 16.0 (9.7-22.3) months and 10.0 (8.3-11.7) months, respectively, which imparted a significant difference (P=0.008). Age at diagnosis, radiotherapy, motor deficit and hydrocephalus were confirmed as independent prognostic factors according to the multivariate analysis (P<0.05). CONCLUSION: Compared with adult patients, children with H3 K27M-mutant DMG confer distinct clinical, radiological and molecular characteristics, and have a dismal prognosis. Radiotherapy is an independent factor associated with prolonged survival.
Authors: John Lynes; Alvina A Acquaye; Hannah Sur; Anthony Nwankwo; Victoria Sanchez; Elizabeth Vera; Tianxia Wu; Brett Theeler; Terri S Armstrong; Mark R Gilbert; Edjah K Nduom Journal: J Neurooncol Date: 2020-07-23 Impact factor: 4.130