Guo Sitong, Wang Songze, Chen Chunli, He Xiaojin, Yang Bentao, Huang Zhiqin, Ma Jianmin, Shi Jitong, Jiang Libin, Chen Fred Kuanfu
Department of Ophthalmology, Beijing Friendship Hospital, Capital Medical University, Beijing, 100050, People's Republic of China.
Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University; Beijing Ophthalmology and Visual Sciences Key Laboratory, Beijing, 100730, People's Republic of China.
J Inflamm Res. 2024 Dec 14;17:11029-11039. doi: 10.2147/JIR.S485499. eCollection 2024.
Orbital inflammatory myofibroblastic tumors (IMTs) are a rare tumor with intermediate biological potential. We analyzed a series of orbital IMTs to determine their unique features.
Records from patients with pathologically confirmed IMT at Beijing Tongren Hospital, Capital Medical University, between January 2004 and August 2022, were reviewed for their ocular presentation and treatment outcomes.
Thirteen cases of primary orbital IMT with positive anaplastic lymphoma kinase on fluorescent in situ hybridization were included. These consists of five men and eight women with an age of onset ranged from 2 to 54 years. Nearly half of the primary orbital IMTs displayed local invasion into the maxillary sinus, ethmoid sinus, and pterygopalatine fossa. On magnetic resonance imaging, orbital IMT usually appeared as an ill-defined, oval, irregular, or diffuse mass with heterogeneous signals. Nearly half (46.2%) of these lesions caused bone destruction, and 31% developed recurrence, which was more likely to occur in lesions involving multiple tissues (50%). All cases of recurrence occurred within five months after the first surgery. No patient developed distant metastases.
Orbital IMT is rare and prone to local invasion and recurrence. The histology and behavior of orbital IMT requires further investigation.
眼眶炎性肌纤维母细胞瘤（IMTs）是一种具有中等生物学潜能的罕见肿瘤。我们分析了一系列眼眶IMTs以确定其独特特征。
回顾了2004年1月至2022年8月期间首都医科大学附属北京同仁医院经病理确诊的IMT患者的记录，以了解其眼部表现和治疗结果。
纳入13例荧光原位杂交检测间变性淋巴瘤激酶呈阳性的原发性眼眶IMT。其中男性5例，女性8例，发病年龄为2至54岁。近一半的原发性眼眶IMT表现为局部侵犯上颌窦、筛窦和翼腭窝。在磁共振成像上，眼眶IMT通常表现为边界不清的椭圆形、不规则形或弥漫性肿块，信号不均匀。这些病变中近一半（46.2%）导致骨质破坏，31%发生复发，复发更易发生在累及多个组织的病变中（50%）。所有复发病例均在首次手术后5个月内发生。无患者发生远处转移。
眼眶IMT罕见，易于局部侵犯和复发。眼眶IMT的组织学和行为需要进一步研究。
