Recurrent orbital space-occupying lesions:a clinicopathologic study of 253 cases

Objective：To analyze the clinical features,histopathologic classification and frequencies of various types of recurrent orbital space-occupying lesions.Methods：A retrospective study was carried out in 253 consecutive patients with recurrent orbital spaceoccupying lesions treated by surgical excision in the Institute of Orbital Diseases,the General Hospital of the Armed Police Force from January 2009 to December 2010.Results：The patients included 123 males and 130 females aged 2 to 78 years（mean,36.2 years）,and the last recurrence interval after operation ranged from 1 month to 40 years（median,4.75 years）.Of all the cases,159（62.8%）,65（25.7%）,20（7.9%）,8（3.2%） and 1（0.4%） had previously experienced once,twice,three,four and six times of surgeries,respectively.Among them,29（11.5%） cases had recurred 3 times or over,and 37（14.6%） cases got recurrence in 10 or more years postoperatively.Most of the patients with local recurrence presented with various clinical manifestations,while 31（12.3%） cases were symptom-free.Two hundred and thirty-one（91.3%） cases underwent surgical removal of the recurrent orbital lesions,and another 22（8.7%） cases had to receive the exenteration of orbit.Categories of these recurrent orbital lesions after operation were as follows：lacrimal gland tumors,65（25.7%） cases;vasogenic diseases,54（21.3%） cases;neurogenic tumors,42（16.6%） cases;secondary tumors,24（9.5%） cases;orbital inflammation,21（8.3%） cases;myogenic tumors,14（5.5%） cases;fibrous and adipose tumors,12（4.7%） cases;lympho-hematopoietic tumors,7（2.8%） cases;bone or cartilage tumors,7（2.8%） cases;orbital cysts,6（2.4%） cases;and indefinitely differentiated tumor,1（0.4%） case.The 10 top histopathologic diagnoses were lacrimal gland pleomorphic adenoma,hemangiolymphangioma,lacrimal gland adenoid cystic carcinoma,meningioma,inflammatory pseudotumor,neurofibroma,sebaceous gland carcinoma,vascular malformation,rhabdomyosarcoma and hemangioma.Conclusions：The variety of recurrent orbital lesions after operation includes mainly of tumors except for vascular malformation and orbital inflammatory lesions.The lacrimal gland epithelial tumor is most prone to relapse after resection,and early and longer-term postoperative follow-up is needed.

Magnetic Resonance Imaging and DWI Features of Orbital Rhabdomyosarcoma

Purpose: To describe the magnetic resonance imaging(MRI)features of orbital rhabdomyosarcoma(RMS).Methods: Thirty-nine patients with histopathologically confirmed orbital RMS were retrospectively reviewed. All patients underwent orbital conventional MRI, including axial, sagittal,and coronal T1-weighted, T2-weighted, and postcontrast T1-weighted sequences. The location, shape, margin, and MRI signal of the 39 lesions were reviewed. DWI in 15 patients and susceptibility weighted imaging(SWI) in 2 patients were also analyzed.Results: Orbital MRI was available in 39 patients and revealed a soft tissue mass in the orbital region in all cases. Of the 39 patients, the primary tumor sites were limited to the orbital proper in 31 cases, while 28 cases had extraocular muscle invasion and 8 cases had extraorbital invasion. All lesions were unilateral. Thirty-three cases were well-defined soft tissue masses and 6 cases appeared as less well-defined softtissue masses. Thirty-four cases showed homogeneous isointense or slightly hypointense signals on T1-weighted imaging(T1WI) and hyperintense signal on T2-weighted imaging(T2WI) compared with extraocular muscles. Five cases had heterogeneous signals with focal areas of increased signal on T1WI or decreased signal on T2WI, including 1 case with hypointense signal on SWI. The mean apparent diffusion coefficient(ADC) value of the viable part of tumors was(0.925±0.09)×10-3mm2/ s. All cases showed moderate to marked enhancement after contrast administration.Conclusion: Several MRI features-including homogeneous isointense or slightly hypointense signal on T1WI and slightly hyperintense signal on T2WI, relative low ADC values, and moderate to marked enhancement, extraocular muscles invasion, and extraorbital extensionare helpful in the diagnosis of orbital RMS.

Adhesion of cavernous hemangioma in the orbit revealed by CT and MRI:analysis of 97 cases

AIM: To assess features of cavernous hemangioma (CH) in the orbit revealed by CT and MRI and summarize prediction of preoperative CT and MRI for the adhesion degree of CH in the orbit. METHODS: A total of 97 patients with pathologically confirmed CH in the orbit were examined with axial and coronal CT scan, and axial, coronal, sagittal, and enhanced fat suppression MRI scan. CT and MRI findings and intraoperative adhesion degrees were retrospectively analyzed. RESULTS: There were 47 patients with slight adhesion, for whom CT and MRI showed round masses with well defined margins in the extraocular muscles; 14 patients with mild adhesion, for whom CT and MRI revealed irregular masses with unclear boundary between CH and the optic nerve in coronal images, and emissary veins in the posterior region of masses in contrast-enhanced images; 36 patients with severe adhesion, for whom CT and MRI exhibited an irregular or ovoid mass filling the orbital apex, or showed distorted and even spiky margins in the posterior region of masses in contrast enhanced images at the presence of a transparent triangle between the mass and the orbital apex. CONCLUSION: Preoperative CT and MRI aid in accurate diagnosis, selection of the surgical approach, and assessment of the adhesion degree and surgical risks for CH.

Clinical features and linkage analysis for a Chinese family with autosomal dominant central areolar choroidal dystrophy

Background A Chinese family with autosomal dominant central areolar choroidal dystrophy （CACD） was identified. The purpose of this study was to collect the clinical findings from the family and to identify the genetic entity by linkage analysis.Methods Forty-three individuals from 3 generations of the family underwent ophthalmologic examinations, including best-corrected visual acuity, examination of the anterior segments, and inspection of the ocular fundus after pharmacologic mydriasis. Affected family members further underwent color vision test, color fundus photography, fluorescein angiography, automated perimetry, and electroretinography. The family was followed up for 30 months. Peripheral venous blood or buccal swabs were collected from each family member and genomic DNA was extracted. Linkage analysis was performed for candidate genes or loci using microsatellite markers.Results Seven family members in 3 continuous generations were diagnosed as having autosomal dominant CACD. The family showed progressive development of the disease, affecting both male and female. Age of onset of visual disturbances varied between 11 and 50 years. Phenotypic variability among affected individuals was apparent and ranged from relatively normal-appearing fundus with mild parafoveal pigment mottling to geographic atrophy of the macula. Fluorescein angiography showed hyperfluorescent parafoveal changes in early stage or well-demarcated area of chorioretinal atrophy with enhanced visibility of the residual underlying choroidal vessels in the late stage. Peripheral retina and visual fields were normal in affected individuals. Electroretinogram showed normal or mild reduction in the photopic amplitude. Eight candidate genes （STGD4, RCD1, peripherin/RDS, GUCA 1A, RIMS1, UNC119, GUCY2D, and AIPL1） and two genetic loci （4p15.2-16.3, and 17p13） were excluded to be responsible for the disease by linkage analysis.Conclusions The clinical findings Of this Chinese family with CACD shared similarities with previously reported families of other ethnicities. Linkage analysis excluded the known genes and genetic loci, indicating genetic heterogeneity of the disease.