Clinical and CT Histological Diagnosis of Orbital Tumors

Summary: The clinical manifestation and characteristics of CT image of 117 cases of orbital tumors in our hospital were investigated. The hemangioma had the highest incidence, and the less common tumors were, in sequence of incidence, pseudotumor, dermoid cysts, neurilemmoma, polymorphous adenoma and meningioma. The sensitivity in diagnosis of orbital tumor by CT was 93. 3 %. The coincidence of CT histological diagnosis with pathology were 83. 3 %, 82. 6% and 71. 4 % for dermoid cysts, hemangioma, and pseudotumor respectively, but the general coincidence of CT histological diagnosis with pathology was only 67. 8 %. When CT was combined with ultrasound, cytological examination and clinical manifestations, the accuracy of histological diagnosis could be improved to 83.3%.

Rare neonatal malignant primary orbital tumors: Three case reports

BACKGROUND Aggressive malignant primary orbital tumors are extremely rare in newborns.The current cases further clarify the clinical features of malignant primary orbital tumors in neonates.CASE SUMMARY At the time of presentation at the Seventh Center of People’s Liberation Army General(PLAG)Hospital,the children were 1-,2-and 5-mo-old,respectively,and included 2 boys and 1 girl.All three cases had unilateral proptosis at birth,and underwent mass excision and histopathologic examination.A peripheral primary neuroectodermal tumor,an aggressive infantile fibromatosis and an embryonic rhabdomyosarcoma were diagnosed,respectively.The first case underwent routine chemotherapy following surgery but died within three months due to worsening condition as the tumor spread throughout the body.The other two children were treated by surgery,and at the follow-up visits 6 mo and 1 year after surgery,respectively,the wound was completed healed,and they had normal growth and development without radiotherapy or chemotherapy.A review of highly uncommon orbital tumors in newborns is also provided.CONCLUSION Malignant primary tumors should be considered in the presence of unilateral proptosis in newborns.

Updates in Orbital Tumors

Orbital anatomy, the clinical features of orbital tumors, the recent development of the diagnosis and management of orbital tumors were described. The incidence of orbital tumors in Dr. Cipto Mangunkusumo Hospital in the past years were introduced. The principle of management of orbital tumors and their prognosis were discussed.

Global incidence and prevalence of malignant orbital tumors

Purpose:Aims to provide an overview of the contemporary epidemiology of malignant orbital tumors by analyzing population-based incidence patterns across various regions worldwide.Methods:In this article,we retrieved orbital malignancy data from the MEDLINE database and analyzed the incidence and prevalence of orbital malignancies worldwide.We performed the literature search by searching on the Mesh terms for malignant orbital tumors("orbital","tumor","lymphoma","malignant","cancer","incidence",and"epidemiology").All included studies were published between 1993 and 2023 and were written in English.Results:Ocular or ophthalmic lymphoma most frequently occurred in the orbit,with a prevalence ranging from 47%to 54%.The incidence of malignant orbital tumors was increasing in the USA 2.0 per million(1981–1993),Netherlands(0.86(1981–1985)to 2.49(2001–2005)per million)and South Korea(0.3–0.8 per million(1999–2016)),respectively.Ophthalmic lymphoma which includes orbit lymphoma was increasing in Canada(0.17–1.47 per million(1992–2010)),Denmark(0.86 per million(1981–1985)to 2.49 per million(2001–2005)),respectively.Conclusions:The predominant primary malignant orbital tumor in adults was lymphoma.Ocular or ophthalmic lymphoma most frequently occured in the orbit.The limited data available suggested an increasing trend in the incidence of malignant orbital tumors in each country included,which were mainly attributed to the increase in lymphoma.Generally,incidence rates were found to increase with advancing age,with no difference between males and females.

Incidence of orbital,conjunctival and lacrimal gland malignant tumors in USA from Surveillance,Epidemiology and End Results,1973-2009

AIM： To determine the types and incidence of tumors affecting the orbit, conjunctiva and lacrimal glands and to study the trend line of these tumors in the United States from 1973 to 2009.METHODS： We used the publicly available Surveillance, Epidemiology and End Results （SEER） database registries to determine the incidence rates. Age was adjusted to the 2000 US Standard Population. Patients were stratified according to age group, gender, race and histological grouping of tumor lesions. Three age groups were defined： 0-19, 20-49 and ≥50y. Annual percentage changes were calculated to examine trends.RESULTS： The overall age adjusted incidence rate was 3.39 （95%CI： 3.27-3.52） per million person-years. The tumors were more prevalent in age group ≥50 counting 9.51 （95%CI： 9.11-9.92） per million person-years. Most of the soft tissue sarcomas occurred in the young age with incidence rate of 0.35 （95%CI： 0.28-0.42） per million person-years. Lymphomas were the dominant subtype in the adult population with incidence rate of 5.74 （95%CI： 5.43-6.06） per million person-years. Incidence rates were higher in males than females with an overall rate ratio of 1.31 （95%CI： 1.21-1.41） mainly caused by the increase in carcinoma subtypes. White race had a higher tumor incidence with a rate ratio of 1.47（95%CI： 1.25-1.73） driven by the higher incidence of most histological subtypes. Orbital tumors showed a higher incidence rate followed by conjunctival and lacrimal gland tumors with incidence rates of 1.59, 1.37 and 0.43 per million person-years respectively. The trend line of overall incidence of tumors showed a significant increase （APC=3.11, 95%CI： 2.61-3.61） mainly due to increase of lymphomas. This increase was higher than the increase of lymphomas at other sites.CONCLUSION： Orbital, conjunctival and lacrimal gland malignant tumors differ among children and adults. Over the years there has been a noticeable increase in incidence rates of orbital and lacrimal gland tumors mainly caused by an increase in lymphomas and an apparent increase due to advances in diagnostic techniques. ICD-O-3 topographical coding should be improved to consider the different orbital bones and ocular structures.

Does reconstruction affect outcomes following exclusively endoscopic endonasal resection of benign orbital tumors: A systematic review with meta-analysis

Objective:As exclusively endoscopic endonasal resection of benign orbital tumors has become more widespread,high-quality outcomes data are lacking regarding the decision of when and how to reconstruct the medial orbital wall following resection.The goal of this study was to systematically review pertinent literature to assess clinical outcomes relative to orbital reconstruction practices.Methods:Data Sources:PubMed,EMBASE,Web of Science.A systematic review of studies reporting exclusively endoscopic endonasal resections of benign orbital tumors was conducted.Articles not reporting orbital reconstruction details were excluded.Patient and tumor characteristics,operative details,and outcomes were recorded.Variables were compared usingχ^(2),Fisher’s exact,and independentt tests.Results:Of 60 patients included from 24 studies,34(56.7%)underwent orbital reconstruction following resection.The most common types of reconstruction were pedicled flaps(n=15,44.1%)and free mucosal grafts(n=11,32.4%).Rigid reconstruction was uncommon(n=3,8.8%).Performance of orbital reconstruction was associated with preoperative vision compromise(P<0.01).The tendency to forego orbital reconstruction was associated with preoperative proptosis(P<0.001),larger tumor size(P=0.001),and operative exposure of orbital fat(P<0.001)and extraocular muscle(P=0.035).There were no statistically significant differences between the reconstruction and nonreconstruction groups in terms of short-or long-term outcomes when considering all patients.In patients with intraconal tumors,however,there was a higher rate of short-term postoperative diplopia when reconstruction was foregone(P=0.041).This potential benefit of reconstruction did not persist:At an average of two years postoperatively,all patients for whom reconstruction was foregone either had improved or unchanged diplopia.Conclusion:Most outcomes assessed did not appear affected by orbital reconstruction status.This general equivalence may suggest that orbital reconstruction is not a necessity in these cases or that the decision to reconstruct was well-selected by surgeons in the reported cases included in this systematic review.

Orbital liposarcoma: a retrospective, single-center study of thirteen patients

AIM:To explore the clinical and pathological characteristics of thirteen patients with orbital liposarcoma.METHODS:The clinical history data of thirteen patients diagnosed as orbital liposarcoma at Beijing Tongren Hospital,from 2006 to 2021 were collected and analyzed.The data includes age,gender,affected orbital side,course of disease,status of disease(primary or recurrent),clinical manifestations,preoperative visual acuity,operative treatment,the relations between liposarcoma and surrounding tissue,longest diameter of liposarcoma,histological subtype,immunohistochemical indicators,follow-up treatment and prognosis.RESULTS:The initial symptoms are diverse.Proptosis is the most frequent chief complaint and the others included vision loss,epiphora,diplopia,and eyelid palpable mass.Results of imaging examination[computed tomography(CT)or magnetic resonance imaging(MRI)]showed orbital mass.In terms of treatment,10 patients received tumor resection,and the mean longest diameter of the tumor was 3.39±1.36 cm.The other 3 patients had optic nerve invaded,so they received orbital exenteration.Pathological examination results confirmed the diagnose of liposarcoma for 13 patients.Six patients displayed as myxoid type,and three patients in each type of dedifferentiated and welldifferentiated type.One patient was verified as pleomorphic,which was a rare type of liposarcoma.All of the patients showed Vimentin positive,and most showed CD34 and S-100 positive.Besides,four patients showed smooth muscle actin positive.All thirteen patients were alive.CONCLUSION:Orbital liposarcoma is a rare disease and it has no specific clinical manifestation.The diagnosis of liposarcoma should be considered when proptosis and orbital mass occurred in orbit.It is recommended to perform pathological examination to achieve early detection and early treatment.

Adjuvant therapy for orbital non-rhabdomyosarcoma soft tissue sarcoma:comparison of long-term outcome between radiotherapy and chemotherapy

AIM:To illustrate clinicopathological features of orbital non-rhabdomyosarcoma soft tissue sarcoma(NRSTS),and to compare the treatment outcome between postoperative radiotherapy(RT) and chemotherapy in a retrospective analysis nearly 20y.METHODS:A retrospective cohort study of 56 patients with orbital NRSTS were reviewed,34 of whom received postoperative RT,and 22 received postoperative chemotherapy.The clinicopathological features,local recurrence,metastases,and survival data were recorded.Survival analysis was performed using the Kaplan-Meier method.RESULTS:During follow-up(111.8mo,ranged 8-233mo) for 56 patients,19 patients of them developed local recurrence,and 7 patients developed distant metastases.Fifteen patients died during follow-up period.Overall survival rates considering the whole study group was 78.57% at 5y,and 72.16% at 10y after the initial diagnosis.Compared with chemotherapy,RT was associated with lower risk of local recurrence [hazard ratio for RT vs chemotherapy,0.263,95% confidence interval(CI),0.095-0.728,P=0.0015];with lower risk of distant metastasis(hazard ratio for RT vs chemotherapy,0.073,95%CI,0.015-0.364,P=0.0014);and with lower risk of death from disease(hazard ratio for RT vs chemotherapy,0.066,95%CI,0.022-0.200,P<0.0001).The 5-year survival rate in RT group was 97.06% compared to 50% in chemotherapy group.CONCLUSION:In patients with orbital NRSTS,postoperative RT provides better control of local recurrence,distant metastasis,and death from disease than chemotherapy.RT is the more preferrable adjuvant therapy compared to chemotherapy possibly.

Whole Body Positron Emission Tomography/Computed Tomography （PET/CT） in the Evaluation of Ophthalmic Tumors

The clinical value of whole body positron emission tomography/computed tomography （PET/CT） as an imaging tool in diagnosis of ophthalmic tumors was investigated. The retrospective observational case series were performed on the patients with suspected ophthalmic tumors who underwent whole body PET/CT. The golden standard of diagnosis was the final pathological diagnosis or the results of long-term follow-up for patients without surgery/ biopsy. PET/CT findings were compared with the golden standard. The sensitivity, specificity, accuracy an^t positive likelihood ratio of PET/CT in the detection of ophthalmic tumors were calculated. The clinical application of PET/CT in different types of ophthalmic tumors was evaluated. The results showed that 30 patients （18 males and 12 females） with a mean age of 43.0 years （range 4-63 years） were collected. The mean sizes of orbital tumors and intraocular tumors were 26.8 mmxl7.8 mm and 11.2 minx6.1 mm, respectively. The overall sensitivity, specificity, accuracy and positive likelihood ratio of whole body PET/CT in ophthalmic tumors were 76.5%, 71.4%, 75.0% and 2.67, and were 62.5%, 100% and 70.0% in intraocular tumors, and those were 100%, 60.0% and 84.6% in orbital tumors, respectively. PET/CT findings were applied to help make appropriate treatment options in 27 out of 30 patients （90.0%）, and 12 （40.0%） patients changed the treatment strategy. False negative results in 4 cases and false positive results in 2 cases were observed in this series. It was suggested that PET/CT was an effective imaging modality in detecting, diagnosing and developing therapeutic schedule for patients with ophthalmic tumors. It was more sensitive and accurate for detecting orbital tumors than for detecting intraocular tumors.

Primary orbital monophasic synovial sarcoma with calcification:A case report

BACKGROUND Synovial sarcoma is a malignant mesenchymal neoplasm with variable epithelial differentiation.Most synovial sarcoma cases are reported in young adults and can arise in any body site.Notably,primary orbital synovial sarcoma is rare.CASE SUMMARY An 8-year-old east Asian girl with 1-month history of gradual painless proptosis and lacrimation of the right eye was admitted.The patient presented with painless proptosis,downward eyeball displacement,and upward movement disorders.According to clinical manifestations,imaging examinations and postoperative immunohistochemical examinations,the diagnosis was monophasic synovial sarcoma with calcification.The patient underwent anterior orbitotomy procedure for removal of the right orbital mass under general anesthesia.The diagnosis of monophasic synovial sarcoma with calcification was confirmed finally through histological and immunohistochemical exam.The follow-up period was 6 mo,and no recurrence was observed during this period.CONCLUSION Primary orbital monophasic synovial sarcoma with calcification is a rare sarcoma,and clinical manifestations and imaging results are not specific.The tumor may present similar features as a benign tumor.Comprehensive analysis of clinical,radiological,and pathological findings is critically important for making the right diagnosis.Conventional treatment approach for synovial sarcoma is surgical resection with adjuvant or neoadjuvant radiotherapy,which is highly effective for localized tumors.

Commentary on"Primary orbital monophasic synovial sarcoma with calcification:A case report"

The present letter to the editor is related to the study titled“Primary orbital monophasic synovial sarcoma with calcification:A case report”.Orbital synovial sarcoma is one of the rare intraorbital masses seen in adult and pediatric populations.Some case reports in the literature revealed that synovial sarcoma may contain calcifications.Therefore,it is important to make differential diagnosis among calcified orbital masses in childhood.

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.

Orbital and sinus rhabdomyosarcoma with concurrent central retinal artery occlusion:A case report

BACKGROUND Both rhabdomyosarcoma(RMS)and central retinal artery occlusion(CRAO)are rare medical diseases,and their simultaneous occurrence in the same patient is extraordinarily uncommon.This study presents a comprehensive overview of the clinical manifestations,diagnostic imaging results,and therapeutic interventions of a patient with both conditions.CASE SUMMARY In this report,we present a 30-year-old male who presented with significant protrusion,pain and vision loss and was diagnosed with RMS in the orbit and sinus with CRAO.Following resection of the sinus and orbital mass and enucleation of the right eye,the patient experienced symptom improvement.CONCLUSION This article provides an in-depth analysis of the patient’s clinical manifestations,the tumor’s anatomical origin,and the etiology of CRAO.The concurrent manifestation of both RMS and CRAO is exceedingly uncommon in clinical practice.

Presentation of massive orbital hidrocystoma at birth:case report and review of the literature

Background:Hidrocystoma,or sudoriferous cyst,is an eyelid tumor originating from apocrine or eccrine sweat glands.Its presence in the orbit is relatively rare.Case presentation:A full-term female child with severe right eye extrusion was referred to our department two hours after birth.We performed cyst aspiration under ultrasonic guidance;15 cc straw-colored fluid was obtained and proptosis resolved significantly.Orbital magnetic resonance imaging(MRI)showed a large unilocular retrobulbar mass with hypo-intensity in T1 and hyper-intensity in T2.The case underwent further daily ocular assessment until day 5;when proptosis began to worsen again.On day 6,under general anesthesia,we performed aspiration and then the cyst was completely removed with an intact wall through a trans-conjunctival incision.The diameter of the aspirated cyst was about 4 cm.In pathologic consultation,a cystic cavity lined by a layer of sweat duct like epithelium with apical snouts consistent with the diagnosis of apocrine hidrocystoma was reported.Conclusion:To date,in the literature,six other cases of orbital hidrocystoma have been reported in childhood with protean clinical pictures;none of which presented at birth.Herein,we introduce the first case report at birth and also provide a review on the literature.Our report strongly argues against the well reputed theory of traumatic origin for orbital hidrocystoma;it has been postulated that this tumor may be the result of sweat gland cells implantation through the orbit.We thereby suggest the possible presence of choristomatous ectopic sweat gland cells in the orbit during embryogenesis.

Aneurysmal Bone Cyst of the Orbit

Aneurysmal bone cyst （ABC） is an uncommon benign lesion with a reported incidence rate of 0.14 cases for every 1,000,000 people.ABC can occur in any part of the skeletal system but is mainly detected in the long bones.Orbit involvement is rare （〈1% of all ABCs）.In this article,we presented two female patients aged 49 and 33 years old respectively who suffered from ABC.After successful surgical removal of the cyst via frontal craniotomy and simple curettage,the patients recovered well and have been in good health throughout the 2.5-4.5 years of follow-up.