Kim Jenna May, Hall Laura Bressler, Elia Maxwell, Ehrlich Michael S, Materin Miguel A, Sinard John H
Yale New Haven Hospital, New Haven, Connecticut, USA.
Ocul Oncol Pathol. 2017 Nov;3(4):304-309. doi: 10.1159/000464466. Epub 2017 Apr 21.
We report a case of acutely presenting mesectodermal leiomyoma of the ciliary body in a 29-year-old female who reported waking up with swollen eyelids of the right eye and light-perception vision. The affected eye had elevated intraocular pressure, a flat anterior chamber, and a pale, round mass arising from the nasal ciliary body, invading the angle and protruding into the visual axis posterior to the lens. Within days, the visual acuity decreased to no light perception. The eye was enucleated.
The enucleated eye harbored a tumor arising from the ciliary body, measuring 18 mm in the greatest dimension. Spindled cells with fibrillary cytoplasmic processes suggested a neural origin though negative for S-100, Melan-A, and HMB-45. The cells stained strongly positive for smooth muscle actin and vimentin, leading to the diagnosis of mesectodermal leiomyoma of the ciliary body.
We review the literature to expand upon the clinical findings, diagnostic methods, and histopathologic and immunohistochemistry characteristics of mesectodermal leiomyoma.
Leiomyoma must be in the differential diagnosis for ciliary body mass, especially in women of reproductive age. Diagnosis relies on histopathology and immunohistochemistry. The mechanism of acute symptom onset may be multifactorial. This case emphasizes the possibility of acute presentation of a rare, benign intraocular tumor.
我们报告一例29岁女性急性出现的睫状体中胚叶平滑肌瘤病例，该患者晨起时右眼眼睑肿胀，仅有光感视力。患眼眼压升高，前房变平，鼻侧睫状体有一苍白圆形肿物，侵犯房角并突入晶状体后方的视轴。数天内，视力下降至无光感。遂行眼球摘除术。
摘除的眼球有一个起源于睫状体的肿瘤，最大径为18mm。梭形细胞有纤维状胞质突起，提示神经起源，但S-100、Melan-A和HMB-45染色阴性。细胞平滑肌肌动蛋白和波形蛋白染色强阳性，诊断为睫状体中胚叶平滑肌瘤。
我们回顾文献，以详述中胚叶平滑肌瘤的临床发现、诊断方法以及组织病理学和免疫组化特征。
平滑肌瘤必须列入睫状体肿物的鉴别诊断，尤其是在育龄女性中。诊断依靠组织病理学和免疫组化。急性症状发作的机制可能是多因素的。本病例强调了一种罕见的良性眼内肿瘤急性表现的可能性。
