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Conjunctival myxoma – atypical presentation of a rare tumour: case report and review of literature

机译:结膜粘液瘤-罕见肿瘤的非典型表现:病例报告和文献复习

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Background Conjunctival myxomas are rare, benign, connective tissue tumours that classically present as slow-growing, painless, well-circumscribed masses (Arch Ophthalmol 124:735-8, 2006; Case Rep Ophthalmol 3:145-50, 2012). There have been 29 cases reported in the literature (Arch Ophthalmol 124:735-8, 2006; Malays J Med Sci 20(1):92-4, 2013; Case Rep Ophthalmol 3:145-50, 2012; Middle East Afr J Ophthalmol 19(3):353-3, 2012). We present a case with atypical features, and emphasize the importance of excisional biopsies for diagnosing indeterminate conjunctival lesions. Case presentation A 32?year old Korean woman presented with a 5?mm × 7?mm × 3?mm pedunculated firm cystic lesion on the inferior palpebral conjunctiva of her right lower eyelid. The lesion had rapidly enlarged over the course of a week. She gave a history of uncomplicated bilateral epiblepharon correction performed in Korea three months prior. There were no systemic features, or family history of genetic conditions. The lesion was excised under local anaesthesia and reported to be a conjunctival myxoma. The clinical and histopathological features of this lesion were consistent with previous reports on conjunctival myxoma (Arch Ophthalmol 124:735-8, 2006; Arch Ophthalmol 101:1416-20, 1983; Case Rep Ophthalmol 3:145-50, 2012; Am J Ophthalmol 102(1):80-84, 1986). The unusual features of this case were, the rapid growth of the lesion - with the previously documented mean time before presentation being 34?months (range 3?months - 24?years) (Arch Ophthalmol 124:735-8, 2006; Case Rep Ophthalmol 3:145-50, 2012); the location of the lesion in the inferior palpebral conjunctiva - 93?% of previously reported cases had occurred in the bulbar conjunctiva (Arch Ophthalmol 124:735-8, 2006; Case Rep Ophthalmol 3:145-50, 2012); and its occurrence in association with recent eyelid surgery - which has never been reported. Conclusion This case of conjunctival myxoma adds to the small number of documented cases, by demonstrating an atypical presentation. Conjunctival myxomas can occur in association with the Carney Complex, which is an autosomal dominant syndrome associated with benign tumours, spotty mucocutaneous pigmentation, and endocrine overactivity (Ophthalmic Surg Lasers Imaging 39(6):514-6, 2008). Ophthalmic manifestations of the Carney Complex have been found to precede vascular embolic events secondary to cardiac myxoma, thus early diagnosis of conjunctival myxoma can prevent potentially devastating consequences (Ophthalmic Surg Lasers Imaging 39(6):514-6, 2008). The different presentations of this rare tumour emphasise the importance of excisional biopsies in diagnosing indeterminate conjunctival lesions; and its association with cardiac myxoma, highlights the need for cardiac investigations in all patients who present with conjunctival myxoma (J Ophthalmol (1);1-5, 2014; Ophthalmic Surg Lasers Imaging 39(6):514-6, 2008).
机译:背景结膜粘液瘤是罕见的,良性的,结缔组织肿瘤,通常表现为生长缓慢,无痛,边界清楚的肿块(Arch Ophthalmol 124:735-8,2006; Case Rep Ophthalmol 3:145-50,2012)。文献中已报道29例(Arch Ophthalmol 124:735-8,2006; Malays J Med Sci 20(1):92-4,2013; Case Rep Ophthalmol 3:145-50,2012; Middle East Afr J Ophthalmol 19(3):353-3,2012)。我们提出了一个具有非典型特征的病例,并强调了切除活检对诊断不确定的结膜病变的重要性。病例介绍一名32岁的韩国妇女在她的右下眼睑下睑结膜出现5毫米×7毫米×3毫米有蒂的硬性囊性病变。病变在一周内迅速扩大。她提供了三个月前在韩国进行的简单双侧上睑肌矫正的历史。没有系统性特征或遗传病的家族史。该病灶在局部麻醉下切除,据报道是结膜粘液瘤。该病变的临床和组织病理学特征与先前关于结膜粘液瘤的报道一致(Arch Ophthalmol 124:735-8,2006; Arch Ophthalmol 101:1416-20,1983; Case Rep Ophthalmol 3:145-50,2012; Am J Ophthalmol 102(1):80-84,1986)。该病例的不寻常特征是病变的快速增长-先前记录的平均出诊时间为34个月(范围3个月-24年来)(Arch Ophthalmol 124:735-8,2006; Case Rep邻苯二酚3:145-50,2012);病变位于睑下结膜的位置-先前报道的病例中93%发生在延髓结膜中(Arch Ophthalmol 124:735-8,2006; Case Rep Ophthalmol 3:145-50,2012);其发生与最近的眼睑手术有关-从未报道过。结论该结膜粘液瘤病例表现出非典型性表现,增加了少数文献记载的病例。结膜粘液瘤可与卡尼复合体联合发生,该复合体是与良性肿瘤,斑点状粘膜皮肤色素沉着和内分泌过度活跃有关的常染色体显性综合症(眼科手术激光成像39(6):514-6,2008)。已经发现卡尼复合体的眼部表现在继发于心脏粘液瘤的血管栓塞事件之前,因此,结膜粘液瘤的早期诊断可以防止潜在的毁灭性后果(Ophthalmic Surg Lasers Imaging 39(6):514-6,2008)。这种罕见肿瘤的不同表现形式强调了切除活检在诊断不确定的结膜病变中的重要性。并且它与心脏粘液瘤的关联性凸显了所有结膜粘液瘤患者都需要进行心脏检查(J Ophthalmol(1); 1-5,2014; Ophthalmic Surg Lasers Imaging 39(6):514-6,2008)。

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