Zhang Rui, Huang Xiaoming, Huo Yandi, Xie Rui, Du Anshi, Wu Tong, Sun Fengyuan
Department of Orbital Disease and Oculoplastic Surgery, Aier Eye Hospital (East of Chengdu), AIER Eye Hospital Group, Chengdu, Sichuan, China.
Department of Orbital Disease and Oculoplastic Surgery, Sichuan Eye Hospital, AIER Eye Hospital Group, Chengdu, Sichuan, China.
Medicine (Baltimore). 2025 Aug 8;104(32):e43348. doi: 10.1097/MD.0000000000043348.
Orbital ameloblastoma is a rare benign tumor with metastatic potential, typically exhibiting follicular or plexiform histopathological patterns. Ameloblastoma commonly occurs in the jaws and rarely metastasizes; when it does, the lungs and lymph nodes are the most frequent secondary sites. In the world's reported literature, there are 32 cases of maxillary ameloblastoma metastasizing to the orbit, with only 4 cases of mandibular ameloblastoma metastasizing to the orbit. The mystery lies in its high recurrence rate and aggressive malignant potential despite being classified as a benign tumor, posing a serious threat to the ocular health and quality of life of patients.
This article reports an unusual case of a female patient who was initially diagnosed with mandibular ameloblastoma 17 years ago. Despite undergoing treatment, the tumor recurred and unusually metastasized to the orbit, resulting in a massive lesion that compressed the tissues surrounding the eyeball. She complained of gradual vision loss in her right eye, redness of the eye, and incomplete eyelid closure.
Magnetic resonance imaging (MRI) scans of the orbit indicated a lesion measuring approximately 5.8 cm5.1 cm5.7 cm (centimeter, cm). The clinical diagnosis is recurrent ameloblastoma of the right orbital, with the histopathological subtype being the basal cell type.
The patient underwent 2 successful partial excisions of the orbital tumor, effectively relieving the compression on the eyeball caused by the tumor.
Currently, the 22-month follow-up after the second surgery has shown satisfactory results, with the patient's visual function being preserved.
The patient exhibited significant facial disfigurement at the time of presentation to the ophthalmology department, attributable to the large size of the orbital mass. Inadequate awareness of this uncommon pathology may result in misdiagnosis as basal cell carcinoma or other malignant orbital neoplasms. Despite the preservation of visual function, limited understanding of the disease could lead to overly aggressive surgical management. Therefore, this case is reported to provide insights into the diagnosis, management, and prognosis, serving as a reference for clinicians encountering similar presentations.
眼眶成釉细胞瘤是一种罕见的具有转移潜能的良性肿瘤，通常表现为滤泡状或丛状组织病理学模式。成釉细胞瘤常见于颌骨，很少发生转移；一旦发生转移，肺和淋巴结是最常见的继发部位。在世界范围内已报道的文献中，有32例上颌成釉细胞瘤转移至眼眶，仅有4例下颌成釉细胞瘤转移至眼眶。其奥秘在于，尽管被归类为良性肿瘤，但其复发率高且具有侵袭性的恶性潜能，对患者的眼部健康和生活质量构成严重威胁。
本文报告了一例不寻常的病例，一名女性患者17年前最初被诊断为下颌成釉细胞瘤。尽管接受了治疗，但肿瘤复发并异常转移至眼眶，导致巨大病变压迫眼球周围组织。她主诉右眼视力逐渐下降、眼睛发红和眼睑闭合不全。
眼眶磁共振成像（MRI）扫描显示一个大小约为5.8 cm×5.1 cm×5.7 cm（厘米，cm）的病变。临床诊断为右眼眶复发性成釉细胞瘤，组织病理学亚型为基底细胞型。
患者成功接受了2次眼眶肿瘤部分切除术，有效缓解了肿瘤对眼球的压迫。
目前，第二次手术后22个月的随访显示结果令人满意，患者的视觉功能得以保留。
患者到眼科就诊时面部出现明显畸形，这归因于眼眶肿块较大。对这种罕见病理情况认识不足可能导致误诊为基底细胞癌或其他眼眶恶性肿瘤。尽管保留了视觉功能，但对该疾病的了解有限可能导致手术治疗过度激进。因此，报告此病例以提供有关诊断、治疗和预后的见解，为遇到类似表现的临床医生提供参考。
