Hofsli Mikael, Gampenrieder Nico, Heegaard Steffen
Department of Ophthalmology, Naestved Hospital, Naestved, Denmark.
Department of Ophthalmology, Odense University Hospital, Odense, Denmark.
Case Rep Ophthalmol. 2017 Jan 31;8(1):67-72. doi: 10.1159/000456067. eCollection 2017 Jan-Apr.
To present a rare case of a 2-year-old girl with neurofibromatosis type 1 (NF1) who presented with ptosis of the right upper eyelid along with a tumor in the eyelid.
A magnetic resonance imaging scan of the orbit revealed a solid tumor located extraconally at the site of the right lacrimal gland. A transcranial orbitotomy was performed.
Histopathological examination demonstrated expanded nerve branches/fascicles cut in various planes in between normal lacrimal gland acini. These findings were consistent with a plexiform neurofibroma presumably deriving from the lacrimal nerve and/or a supraorbital nerve branch.
This is the first case of a plexiform neurofibroma involving the lacrimal gland ever described and the tumor shows similarities with neurofibroma in other salivary glands with a high recurrence rate. Plexiform neurofibromas are frequently seen in patients with NF1 and rarely undergo malignant transformation.
报告1例罕见的1型神经纤维瘤病（NF1）2岁女童，表现为右上睑下垂伴眼睑肿物。
眼眶磁共振成像扫描显示，在右泪腺部位的眶锥外有一实性肿物。实施了经颅眶切开术。
组织病理学检查显示，在正常泪腺腺泡之间可见不同平面切断的增粗神经分支/束。这些表现符合推测起源于泪腺神经和/或眶上神经分支的丛状神经纤维瘤。
这是首例报道的累及泪腺的丛状神经纤维瘤，该肿瘤与其他涎腺的神经纤维瘤相似，复发率高。丛状神经纤维瘤常见于NF1患者，很少发生恶变。
