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多发性眼眶神经纤维瘤,与冯雷克林霍增氏病无关。

Multiple orbital neurofibromas unassociated with von Recklinghausen's disease.

作者信息

Shields J A, Shields C L, Lieb W E, Eagle R C

机构信息

Ocular Oncology Service, Wills Eye Hospital, Jefferson Medical College, Thomas Jefferson University, Philadelphia, Pa 19107.

出版信息

Arch Ophthalmol. 1990 Jan;108(1):80-3. doi: 10.1001/archopht.1990.01070030086034.

Abstract

A 58-year-old man with a 10-year history of right periorbital pain treated with multiple analgesics presented with slowly progressive exophthalmos of the right eye. Orbital imaging studies disclosed three separate well-defined tumors, located in the temporal fossa, the intraconal space, and within the floor of the orbit. All three tumors were removed intact and proved on histopathologic evaluation to be localized neurofibromas. The patient had complete relief of the chronic pain following removal of the tumors. We emphasize that the unusual occurrence of multiple circumscribed orbital tumors should suggest the diagnosis of neurofibroma even in the absence of von Recklinghausen's neurofibromatosis.

摘要

一名58岁男性,有10年右眶周疼痛病史,曾使用多种镇痛药治疗,现出现右眼缓慢进行性眼球突出。眼眶影像学检查发现三个界限清楚的独立肿瘤,分别位于颞窝、肌锥内间隙和眶底。所有三个肿瘤均完整切除,组织病理学评估证实为局限性神经纤维瘤。肿瘤切除后,患者的慢性疼痛完全缓解。我们强调,即使没有冯·雷克林豪森神经纤维瘤病,眼眶出现多个边界清楚的肿瘤这一不寻常情况也应提示神经纤维瘤的诊断。

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