Abstract：
OBJECTIVE: Malignant triton tumours (MTTs) are rare but aggressive subtypes of malignant peripheral nerve sheath tumours (MPNSTs) with a high recurrence rate and 5-year survival of 14%. Systematic imaging data on MTTs are scarce and mainly based on single case reports. Therefore, we aimed to identify typical CT and MRI features to improve early diagnosis rates of this uncommon entity.
METHODS: A systematic review on literature published until December 2022 on imaging characteristics of MTTs was performed. Based on that, we conducted a retrospective, monocentric analysis of patients with histopathologically proven MTTs from our department. Explorative data analysis was performed.
RESULTS: Initially, 29 studies on 34 patients (31.42 ± 22.6 years, 12 female) were evaluated: Literature described primary MTTs as huge, lobulated tumours (108 ± 99.3 mm) with central necrosis (56% [19/34]), low T1w (81% [17/21]), high T2w signal (90% [19/21]) and inhomogeneous enhancement on MRI (54% [7/13]). Analysis of 16 patients (48.9 ± 13.8 years; 9 female) from our institution revealed comparable results: primary MTTs showed large, lobulated masses (118 mm ± 64.9) with necrotic areas (92% [11/12]). MRI revealed low T1w (100% [7/7]), high T2w signal (100% [7/7]) and inhomogeneous enhancement (86% [6/7]). Local recurrences and soft-tissue metastases mimicked these features, while nonsoft-tissue metastases appeared unspecific.
CONCLUSIONS: MTTs show characteristic features on CT and MRI. However, these do not allow a reliable differentiation between MTTs and other MPNSTs based on imaging alone. Therefore, additional histopathological analysis is required.
CONCLUSIONS: This largest published systematic analysis on MTT imaging revealed typical but unspecific imaging features that do not allow a reliable, imaging-based differentiation between MTTs and other MPNSTs. Hence, additional histopathological analysis remains essential.
METHODS: A systematic review on literature published until December 2022 on imaging characteristics of MTTs was performed. Based on that, we conducted a retrospective, monocentric analysis of patients with histopathologically proven MTTs from our department. Explorative data analysis was performed.
RESULTS: Initially, 29 studies on 34 patients (31.42 ± 22.6 years, 12 female) were evaluated: Literature described primary MTTs as huge, lobulated tumours (108 ± 99.3 mm) with central necrosis (56% [19/34]), low T1w (81% [17/21]), high T2w signal (90% [19/21]) and inhomogeneous enhancement on MRI (54% [7/13]). Analysis of 16 patients (48.9 ± 13.8 years; 9 female) from our institution revealed comparable results: primary MTTs showed large, lobulated masses (118 mm ± 64.9) with necrotic areas (92% [11/12]). MRI revealed low T1w (100% [7/7]), high T2w signal (100% [7/7]) and inhomogeneous enhancement (86% [6/7]). Local recurrences and soft-tissue metastases mimicked these features, while nonsoft-tissue metastases appeared unspecific.
CONCLUSIONS: MTTs show characteristic features on CT and MRI. However, these do not allow a reliable differentiation between MTTs and other MPNSTs based on imaging alone. Therefore, additional histopathological analysis is required.
CONCLUSIONS: This largest published systematic analysis on MTT imaging revealed typical but unspecific imaging features that do not allow a reliable, imaging-based differentiation between MTTs and other MPNSTs. Hence, additional histopathological analysis remains essential.
摘要：
目的：恶性triton肿瘤（MTT）是罕见但侵袭性的恶性外周神经鞘瘤（MPNSTs）亚型，复发率高，5年生存率为14％。MTT的系统成像数据很少，主要基于单例报告。因此,我们旨在识别典型的CT和MRI特征,以提高这种罕见实体的早期诊断率.
方法：对2022年12月之前发表的关于MTT影像学特征的文献进行了系统评价。基于此,我们做了一个回顾,来自我们部门的经组织病理学证实的MTT患者的单中心分析。进行探索性数据分析。
结果：最初，对34例患者进行29项研究（31.42±22.6年，12名女性）进行了评估：文献描述了主要的MTT巨大，小叶性肿瘤（108±99.3mm）伴中央坏死（56%[19/34]），低T1w（81%[17/21]），高T2w信号(90%[19/21])和MRI不均匀增强(54%[7/13])。对我们机构的16例患者（48.9±13.8岁；9名女性）的分析显示出可比的结果：原发性MTT显示大，分叶肿块（118mm±64.9），坏死面积（92％[11/12]）。MRI显示低T1w(100%[7/7]),高T2w信号(100%[7/7])和不均匀增强(86%[6/7])。局部复发和软组织转移模仿了这些特征，而非软组织转移似乎没有特异性。
结论：MTT在CT和MRI上表现出特征性特征。然而,这些不允许MTT和其他MPNST之间的可靠区分仅基于成像.因此,需要额外的组织病理学分析。
结论：这项已发表的关于MTT成像的最大系统分析揭示了典型但非特异性的成像特征，基于成像的MTT和其他MPNST之间的区别。因此，额外的组织病理学分析仍然至关重要。
方法：对2022年12月之前发表的关于MTT影像学特征的文献进行了系统评价。基于此,我们做了一个回顾,来自我们部门的经组织病理学证实的MTT患者的单中心分析。进行探索性数据分析。
结果：最初，对34例患者进行29项研究（31.42±22.6年，12名女性）进行了评估：文献描述了主要的MTT巨大，小叶性肿瘤（108±99.3mm）伴中央坏死（56%[19/34]），低T1w（81%[17/21]），高T2w信号(90%[19/21])和MRI不均匀增强(54%[7/13])。对我们机构的16例患者（48.9±13.8岁；9名女性）的分析显示出可比的结果：原发性MTT显示大，分叶肿块（118mm±64.9），坏死面积（92％[11/12]）。MRI显示低T1w(100%[7/7]),高T2w信号(100%[7/7])和不均匀增强(86%[6/7])。局部复发和软组织转移模仿了这些特征，而非软组织转移似乎没有特异性。
结论：MTT在CT和MRI上表现出特征性特征。然而,这些不允许MTT和其他MPNST之间的可靠区分仅基于成像.因此,需要额外的组织病理学分析。
结论：这项已发表的关于MTT成像的最大系统分析揭示了典型但非特异性的成像特征，基于成像的MTT和其他MPNST之间的区别。因此，额外的组织病理学分析仍然至关重要。
