Primary Orbital Tumors Research Articles

Clinicodemographic profile of orbital exenteration in a tertiary eye care center - A 20-year experience.

This study aimed to determine the clinical indications for orbital exenteration, demographic profile of these patients, and clinicopathologic correlations in the current times and to compare these results with previous published data. It was a retrospective study. All exenterations performed at a tertiary eye care center over a period of 20 years (from January 2001 to June 2020) were retrospectively evaluated. Patient records were reviewed to obtain demographic data, presenting symptoms and their duration, laterality, and clinical and histopathologic diagnosis. A total of 352 cases (males:females = 222:130) who underwent exenteration were identified. Patients age ranged from 11 months to 87 years (mean: 43.86 years, median: 50 years). The most common indication for exenteration was found to be eyelid malignancy in 54.36%, followed by retinoblastoma in 18.75% and primary orbital tumors in 14.49%. Out of 156 cases of eyelid malignancies requiring exenteration, squamous cell carcinoma (SCC) was the most common histologic subtype ( n = 94, 60.26%), followed by sebaceous gland carcinoma ( n = 40, 25.64%) and basal cell carcinoma ( n = 20, 12.82%). The most common primary orbital tumors requiring exenteration were adenocystic carcinoma of the lacrimal gland in adults and rhabdomyosarcoma in the pediatric age group. Benign conditions requiring exenteration included fulminant fungal orbital infections and lymphangioma among others. The number of exenterations performed have significantly increased in terms of absolute numbers. However, the ratio of exenteration to other tumor-related surgeries, mainly excision biopsy, has reduced compared to that reported from a previous study. The most common indication in our study remains eyelid malignancy followed by intraocular malignancy. However, SCC has emerged as the most common tumor histologic subtype requiring exenterations.

Diagnostic and Therapeutic Management of Primary Orbital Neuroendocrine Tumors (NETs): Systematic Literature Review and Clinical Case Presentation.

The ocular involvement of neuroendocrine neoplasms (NENs) is uncommon and mainly represented by metastases from gastrointestinal and lung neuroendocrine tumors. Primary orbital NENs are even less common and their diagnostic and therapeutic management is a challenge. A systematic review of the literature was conducted from 1966 to September 2023 on PubMed to identify articles on orbital NENs and to summarize their clinical-pathological features, diagnosis and therapeutic management. Furthermore, we presented a case of a locally advanced retro-orbital primary neuroendocrine tumor that was referred to the certified Center of Excellence of Sant'Andrea Hospital, La Sapienza University of Rome, Italy. The final analysis included 63 records on orbital NENs and 11 records focused on primary orbital NENs. The localization was mostly unilateral and in the right orbit; proptosis or exophthalmos represented the initial symptoms. The diagnostic work-up and therapeutic management was discussed and a diagnostic algorithm for the suspicion of primary orbital NENs was proposed. A multidisciplinary approach is required for the management of primary orbital NENs, emphasizing the importance of early referral to dedicated centers for prompt differential diagnosis, tailored treatment, and an improved quality of life and survival.

Precision Surgery for Orbital Cavernous Hemangiomas: The Role of Three-Dimensional Printing in Individualized Resection-An Educational Experience.

Orbital cavernous hemangiomas are the most common primary orbital tumors in adults, providing challenges for optimal surgical approach planning within an anatomically complex area with close proximity to vital neurovascular structures. The authors present an individualized lateral mini-orbitozygomatic approach for the resection of an orbital cavernous haemangioma based on a preoperative 3-dimensional-printed model. This individualized approach enabled the surgeons to achieve optimal exposure while maintaining safety during the resection of the lesion, but also to respect the patient's physiognomy and hairline. In addition, the model was used for patient informed consent, helping the patient understand the procedure. Although adding additional effort to preoperative planning, 3-dimensional model-based approaches can offer great benefits when it comes to customizing surgical approaches, especially for anatomically challenging resections.

Orbital resection by intranasal technique (ORBIT): A new classification system for reporting endoscopically resectable primary benign orbital tumors.

The Cavernous Hemangioma Exclusively Endonasal Resection (CHEER) staging system has become the gold standard for outcomes reporting in endoscopic orbital surgery for orbital cavernous hemangiomas (OCHs). A recent systematic review demonstrated similar outcomes between OCHs and other primary benign orbital tumors (PBOTs). Therefore, we hypothesized that a simplified and more comprehensive classification system could be developed to predict surgical outcomes of other PBOTs. Patient and tumor characteristics as well as surgical outcomes from 11 international centers were recorded. All tumors were retrospectively assigned an Orbital Resection by Intranasal Technique (ORBIT) class and stratified based on surgical approach as either exclusively endoscopic or combined (endoscopic and open). Outcomes based on approach were compared using chi-squared or Fisher's exact tests. The Cochrane-Armitage test for trend was used to analyze outcomes by class. Findings from 110 PBOTs from 110 patients (age 49.0±15.0 years, 51.9% female) were included in the analysis. Higher ORBIT class was associated with a lower likelihood of gross total resection (GTR). GTR was more likely to be achieved when an exclusively endoscopic approach was utilized (p<0.05). Tumors resected using a combined approach tended to be larger, to present with diplopia, and to have an immediate postoperative cranial nerve palsy (p<0.05). Endoscopic treatment of PBOTs is an effective approach, with favorable short-term and long-term postoperative outcomes as well as low rate of adverse events. The ORBIT classification system is an anatomic-based framework that effectively facilitates high-quality outcomes reporting for all PBOTs.

Risk and Lag-time for Development of Lacrimal Gland Carcinoma Expleomorphic Adenoma: Observations and Conjectural Study.

To estimate the proportion of de novo lacrimal gland pleomorphic adenomas (PAs) and carcinomas expleomorphic adenomas (CEPAs), together with age at presentation and first symptom. Conjectural models of tumor growth are considered. Retrospective review of patients with orbital lobe PA or CEPA. The presenting age was examined for conformation to a Gaussian distribution and the cumulative distribution function derived for both tumor types. The risk of CEPA with age was estimated by logistic regression. About one-sixth (27/172; 16%) of these primary orbital lobe tumors were CEPAs, with 145 PAs (76/145 male; 52%) and 27 CEPAs (12/27 male; 44%). The mean presenting age for PAs was 48.3 years (median 47.7; range 11-84 years) and 57.7 years for CEPAs (median 61.2, range 27-91 years) ( p = 0.0062), and the standard deviations for each group are almost identical (16.3 for PAs, 15.9 for CEPAs; p = 0.92). Five (3.4%) PAs and 1 (3.7%) CEPA were asymptomatic: otherwise, the median symptom duration was 24 months for both PAs and CEPAs ( U test: p = 0.65). The odds of CEPA rises significantly with age, increasing 1.04-fold annually ( p = 0.0079). The almost identical measures of dispersion for the presenting ages of PA and CEPA suggests that, once malignant transformation occurs, there might be a relatively constant period before it is evident. CEPAs present about a decade after PAs, this unexpectedly later presentation for the malignancy possibly being explained by a gradual replacement of the PA by the newly arising carcinoma within the preceding benign tumor.

Clinico-epidemiological analysis of 1000 cases of orbital tumors.

To clarify the incidence, demography and clinical features of orbital tumors diagnosed in a single institute in Japan. Retrospective, observational case series. Patients with primary orbital tumors including tumor-like lesions diagnosed clinically or histopathologically at Tokyo Medical University Hospital between 1995 and 2019 were analyzed. Incidence of all orbital tumors, demographic profile and clinical features of major benign and malignant tumors were reviewed retrospectively. Totally 1000 cases of primary orbital tumor were diagnosed clinically or histopathologically during the study period. Benign tumors accounted for 72% and malignant tumors 28%. 55% of benign tumors and 99% of malignant tumors were proven histopathologically. The most common benign orbital tumor was idiopathic orbital inflammation (27%), followed by IgG4-related ophthalmic disease (17%), cavernous venous malformation (13%) and pleomorphic adenoma (9%). The most common malignant tumor was lymphoma (70%), followed by adenoid cystic carcinoma (7%) and solitary fibrous tumor (5%). Epidemiology of orbital tumors has changed by the improvement of imaging techniques, establishment of novel clinical and histopathological criteria, and changes in population age structure associated with the aging society. Currently, lymphoproliferative diseases including lymphoma and IgG4-related ophthalmic diseases form the major orbital tumors in Japan.

Optimising surgical approaches to the orbit — a retrospective analysis of a series of forty orbital tumours

Background: This study aimed to analyse the influence of location, pathology, and approach on the extent of resection of orbital tumours. Material and methods: We have conducted a retrospective analysis of the clinico-radiological features of all orbital tumours operated between January 2012 and December 2019. Frontotemporal craniotomy with orbitotomy was performed for almost all lesions, except those limited to the lateral compartment (lateral orbitotomy), superior compartment (mini-craniotomy), and inferior medial compartment (transconjunctival/endoscopy). Statistical Analysis System (SAS) version 9.4 was used for analysis, and a p-value &lt; 0.05 was considered to be statistically significant. The study was approved by the Institutional Review Board (3/10/2020). Results: Our study group comprised forty patients with a mean age of 40.89 years ± 19 years. Of the forty tumours, 21 (52.5%) cases were primary tumours of the orbit, and 19 (47.5%) were secondary tumours. The majority, 27 (67.5%), of the tumours were extraconal in location. The two most common presenting symptoms were proptosis (36/40; 90%) and visual blurring (30/40; 75%). The majority of the patients could be successfully operated through a fronto-tempero-orbital approach with or without removal of zygoma (28/40; 70%). Meningioma was the most common histology (45%), followed by schwannoma and lacrimal gland tumours (10%). Primary orbital tumours (15/21; 71.6%) and intraconal tumours (9/13; 69.23%)were associated with greater rates of gross total removal (GTR). GTR in sphenoorbital meningiomas was poor (2/17; 11.8%). Conclusion: Intraconal primary benign orbital tumours have a better chance of total gross removal. A frontotempero- orbital approach with or without removal of zygoma provides adequate exposure for the majority of the tumours.

A giant dermoid cyst of the orbit present in adulthood — a case report

Dermoid cysts are common orbital developmental tumors in childhood. They account for 3 to 9% of primary orbital tumors. They are considered to be congenital, but not all of them are diagnosed at birth. Dermoid cysts are benign choristomas that arise from sequestration of ectoderm along the lines of embryonic fusion of mesodermal processes destined to be bone. In most cases, the lesion is close to the zygomatic-frontal suture leading to a superotemporal subcutaneous mass in the eyebrow or eyelid. The intraorbital location is relatively rare and more prone to complications, particularly compressive. We report the case of a 41-year-old man with a large intraorbital tumor. The imaging aspects were evocative of an extraconal dermoid cyst. The tumor was resected through a transconjunctival route. Histopathologic examination confirmed the diagnosis of dermoid cyst.

Exclusively endoscopic endonasal resection of benign orbital tumors: a systematic review and meta-analysis.

The Cavernous Hemangioma Exclusively Endonasal Resection (CHEER) classification system was developed to standardize prospective outcome analysis following orbital cavernous hemangioma (OCH) resection. The goal of this study was to retroactively apply the CHEER system to all prior existing reports of endoscopic resection of primary benign orbital tumors (BOTs) to: (1) compare patient presentations, perioperative characteristics, and outcomes between OCH and other BOTs; and (2) determine whether the CHEER categorization regime could be expanded to other BOTs. A systematic review of studies reporting exclusively endoscopic resections of OCH and other BOTs (eg, solitary fibrous tumor, schwannoma, and meningioma) was performed. Patient, tumor characteristics, and operative outcomes were recorded. All tumors with adequate reporting were retrospectively assigned a CHEER stage. Outcomes were compared using chi-square or Fisher's exact tests. Ninety-three studies met inclusion criteria, and sufficient data were available in 36 studies, comprising 105 tumors (n = 87 OCHs; n = 18 other BOTs). Baseline patient and tumor characteristics, as well as intraoperative and short-term postoperative outcomes were not significantly different between OCHs and other BOTs. Long-term outcomes (eg, visual deficits, diplopia, eye position, and recurrence) also did not differ when controlling for CHEER stage. This review represents the largest collection of outcomes data following exclusively endoscopic endonasal resection of BOTs. Short-term and long-term outcomes appear similar between OCHs and other BOTs. These results suggest that exclusively endoscopic resection of orbital tumors may be effective in a range of benign pathologies. Furthermore, these results support a broader application of the CHEER system to other benign primary orbital tumors.

Postoperative severe visual impairment: surgical outcome of 165 patients with orbital tumours in the muscle cone.

This study aimed to evaluate the risk factors of postoperative severe vision impairment (PSVI) for a primary orbital tumour in the muscle cone. A retrospective analysis of the patients who underwent orbitotomy for primary intraconal tumours at the Tianjin Medical University Eye Hospital from January 2010 to December 2015. A total of 165 cases of orbitotomy for primary orbital tumours in the muscle cone were included in the study. Postoperatively, 12 cases with vision acuity ≤20/400 or ≥4 rows of vision decline and without any corrected effect were analysed as PSVI, including no light perception (NLP) for 3 cases. The multivariate logistic regression indicated that the tumour in orbital apex (P = 0.048, OR = 4.912, 95% CI: 1.011-23.866), severe optic nerve displacement (P = 0.030, OR = 6.007, 95% CI: 1.184-30.473) and intraoperative tight adhesion (P = 0.003, OR = 12.031, 95% CI: 2.282-63.441) were the independent risk factors for PSVI. The incidence of PSVI for the intraconal tumour was 7.3%, and the incidence of NLP was 1.8%. The tumour in orbital apex, severe optic nerve displacement and intraoperative tight adhesion were independent risk factors for PSVI.

Gene Fusions in Ocular Adnexal Tumors

To highlight the increasing importance of gene fusions in the diagnosis, prognosis, and therapy of ocular adnexal tumors. Perspective. A focused review of gene fusions, their pathogenic mechanism, and gene fusion detection methods in lacrimal gland and primary orbital and ocular adnexal soft tissue tumors; reappraisal of diagnostic, prognostic, and therapeutic approach to ocular adnexal tumors in light of emerging molecular genetic data. The widespread implementation of fluorescence insitu hybridization and next-generation sequencing methods in pathology practice has led to identification of recurrent gene rearrangements and fusions in a variety of tumors. As a result, molecular genetic methods have become the gold standard for diagnosis of tumors with overlapping histology and immunophenotype, such as small round blue cell tumors. Identification of canonic gene fusions has led to development of sensitive and specific immunohistochemical markers, such as STAT6 in solitary fibrous tumor. In addition to diagnostic accuracy, gene fusions have prognostic implications, such as unfavorable prognosis of PAX3-FOXO1 fusion in alveolar rhabdomyosarcoma. Finally, recognition of gene fusions as a driving mechanism in neoplasia has led to development of U.S. Food and Drug Administration-approved targeted therapies, such as TRK inhibitors for NTRK fusion-positive cancers. The discovery of recurrent gene fusions in various tumors, including those involving ocular adnexa, has led to a deeper insight into the molecular mechanisms of these neoplasms, revolutionizing our approach to their diagnosis, prognostication, and therapy.

The Lateral Orbitotomy Approach for Intraorbital Lesions.

The lateral orbitotomy approach (LOA) was first described by Kronlein in 1888 and has since been subject to many modifications and variations. When considering orbital approaches, the location of the pathology is often more important in decision making than the type of pathology. The LOA is best suited for access to intraconal and extraconal lesions lateral to the optic nerve. Pathologies treated via the LOA include primary orbital tumors, extraorbital tumors with local extension into the orbit, and distantly metastatic lesions to the orbit. These all often initially manifest with vision loss, oculomotor deficits, or proptosis. The expertise of a multidisciplinary team is needed to execute safe and effective treatment. Collaboration between many specialties may be required, including ophthalmology, neurosurgery, otolaryngology, plastic surgery, oncology, and anesthesiology. The modern technique involves either a lateral canthotomy or eyelid crease incision with removal of the lateral orbital wall. It affords many advantages over a pterional craniotomy, primarily a lower approach morbidity and superior cosmetic outcomes. Reconstruction is fairly simple and the rate of complications-vision loss and extraocular muscle palsy-are low and infrequently permanent. Deep orbital apex location and intracranial extension have traditionally been considered limitations of this approach. However, with increased surgeon comfort, modern technique, and the adoption of endoscopy, these limits have expanded to even include primarily intracranial pathologies. This review details the LOA, including the general technique, its indications and limitations, reconstruction considerations, complications, and recent data from case series. The focus is on microscopic access to intraorbital lesions.

A Case Report on Intraorbital Extraconal Dermoid Cyst

Orbital dermoid cyst represents 3 to 4% of all primary orbital tumors. The intraorbital location is relatively rare and it commonly occurs at childhood. We reported the case of a 4-year-old male child with an intraorbital dermoid cyst, who presented with progressive left proptosis, which had noticed 6 months earlier. Visual acuity, ocular motility and fundoscopic examination were normal. Magnetic resonance Imaging (MRI) revealed an extraconal orbital mass situated on superior &amp; mildly posterior aspect of orbit and which was iso-to-hypointense in T1, hyperintense in T2, restriction of diffusion in DWI, hyperintense signal on ADC (Shine through) and no enhancement after giving contrast. The tumor was completely excised via a left fronto-orbital access. Histopathologically, the tumor was confirmed as dermoid cyst.

Orbital exenteration and reconstruction in a tertiary UK institution: a 5-year experience

ABSTRACT Purpose Orbital exenteration is a radical oncological surgery that is usually indicated for advanced primary orbital tumors or invasion from local malignancy. We report a 5-year series from a tertiary head and neck center with particular focus on our ablative and reconstructive approach. Methods We performed a clinicopathological review of patients referred to Guy’s and St Thomas’ NHS Foundation Trust Head and Neck multidisciplinary team for management input of an orbital malignancy during the period of 2013 to 2018. Cases involving local invasion from sinonasal malignancy were excluded. The reconstructive approach, perioperative complications, disease-free and overall survival were analyzed. Results 27 patients were identified and of those treated surgically, a radical extended orbital exenteration was required in almost half (44.4%), with squamous cell carcinoma being the most common pathology (55.6%). A concurrent neck dissection and parotidectomy were commonly performed with confirmed or suspected regional disease, or in the presence of high-risk pathological features. This approach resulted in favourable 2-year overall survival in these advanced stage cases of 84.6% and disease-free survival of 73.2%, with 92% achieving a negative surgical margin. The majority of treated patients required a free flap reconstruction, especially when an extended exenteration defect or adjuvant treatment was anticipated. The anterolateral thigh flap was the most commonly used donor site, and we present our algorithm for reconstruction of these defects. Conclusions A multidisciplinary approach to advanced orbital malignancy with a comprehensive approach to surgical resection and reconstruction results in favorable oncological outcomes and addresses functional and cosmetic patient rehabilitation.

Cystic appearance - a new feature of solid fibrous tumours in the lacrimal gland: a case report with literature review

BackgroundSolitary fibrous tumours (SFTs) rarely occur in the orbit, especially in the lacrimal area. These tumours are mostly solid. Cystic changes have been documented, but they remain very rare. Only three cases of primary orbital solitary fibrous tumours with cystic changes have been reported in the literature, but no cases have been reported to occur in the lacrimal gland. Solitary fibrous tumours generally follow a benign course and are treated definitively with surgical excision. Data from the literature suggest that the cystic nature of SFT presents a risk of recurrence and could be a harbinger of malignancy.Case presentationA 42-year-old woman was admitted to the endocrinology department for right unilateral exophthalmia and epiphora in the last 8 months. An ophthalmological evaluation showed exophthalmia only in the right eye (22 mm) and normal visual acuity, visual field and extraocular movements. Investigations revealed normal thyroid function. Orbital magnetic resonance imaging detected a 4 × 2,2 × 2,7 cm septate pseudocystic mass in the right lacrimal gland. Given her lacrimal gland tumour diagnosis, the patient was submitted for neurosurgical intervention with total ablation of the tumoural mass and complete right dacryoadenectomy. Although the intraoperative extemporaneous examination results were suggestive of a haemangiopericytoma, histological and immunocytochemical examination showed an extrapleural SFT. The postoperative clinical evolution was favourable, with remission of the exophthalmia. Fifteen months after surgery, no signs of recurrence were noticed.ConclusionsWe report the first case of an SFT with cystic changes in the lacrimal gland. Although the presence of cavitary lesions alone does not necessarily indicate aggressive behaviour, cystic changes pose a risk of recurrence and may suggest malignant transformation over time. As a result, our case requires long-term follow-up due to recurrence and malignant potential.

Craniofacial Approaches to Pediatric Orbital Tumors.

Primary pediatric orbital tumors requiring surgery are uncommon and often require multidisciplinary management. Commonly used surgical approaches to the orbit include transconjunctival, transcutaneous (eyelid), transcranial, or extracranial osteotomies. This paper reviews a 10-year experience of cases that required a transcranial or extracranial surgical approach at the Birmingham Children's Hospital. A total of 9 patients were identified between the years 2008 to 2017. Pathologies included rhabdomyosarcoma, juvenile ossifying fibroma, optic nerve glioma, and retinoblastoma. Surgical approaches to the orbit included supraorbital bar osteotomy (transcranial) or lateral orbitotomy (extracranial). Surgical team members included neurosurgery, craniofacial surgery, and ophthalmology. This study aims to review the role of surgery in management as well as the specific indications for performing transcranial or extracranial osteotomies. It also highlights the excellent access achieved with the use of these osteotomies in certain cases, especially when compared with transconjunctival or transcutaneous approaches.

Orbital Tumors: Report of 70 Surgically Treated Cases

The orbital cavity is an anatomically complex region and an area of interest of many specialists, each of whom is familiar with specific surgical approaches. We retrospectively reviewed clinical and outcome data of 70 patients who underwent surgery for resection of an orbital tumor with a mean follow-up of 111.9± 79.6 months. The clinical outcome was reported and the role of sex, age, histology, tumor location, tumor size, and surgical approach on the extent of tumor removal was assessed. Total removal was achieved in 74.3%, subtotal removal in 20%, and partial removal in 5.7% of patients. A fronto-orbital craniotomy was used in 57.1% of cases, frontal approach in 17.1%, fronto-orbit-zygomatic approach in 10%, and endoscopic endonasal approach in 11.4%. Complications included visual acuity decrease (4.3%), cerebrospinal fluid leak (4.3%), nerve palsy (10%; supra-orbital nerve 4.3%; frontal branches of facial nerve 2.9%, third cranial nerve 2.9%), and enophthalmos (1.4%). Lateral orbitotomy, combined fronto-orbital and maxillotomy, and trans-eyelid approaches were used in the remaining cases. The fronto-orbital, frontal, and lateral orbitotomy approaches were associated with greater rates of total resection as compared with the fronto-orbit-zygomatic approach, which was used in difficult cases in which the tumor involved several regions. We recommend, 1) the endoscopic endonasal approach for primary orbital tumors located in the medial or inferior orbital walls without extra-orbital extension; 2) the trans-eyelid approach for tumors of the upper and upper-lateral quadrants extraconally located, and 3) the fronto-orbital approach for intraconally located tumors involving more than one quadrant.

Clinical Presentation and Management of 10 Consecutive Cases of Primary Orbital Tumors of Neurological Origin Presenting to a Regional Institute of Ophthalmology

Introduction: Proptosis is a common presentation to our outpatient department. The eyes are as much a visual structure as they are an aesthetic part of the face. We designed a study to document the clinical presentation and management of ten consecutive cases of primary benign neural orbital tumors presenting to a regional institute of ophthalmology. Aim: The aim of the study is to document the clinical presentation and management of ten consecutive cases of primary benign neural orbital tumors presenting to a regional institute of Ophthalmology. Methodology: Ten consecutive cases of primary benign neural orbital tumors were enrolled. They were subjected to a thorough clinical examination and imaging. Thereafter they were managed by observation, surgery or radiotherapy as per the case. Results: There were five cases of schwannoma which responded well to surgery with good visual outcome. The two cases of optic nerve glioma one was subjected to observation due to slow growth and good vision. The second case was managed surgically due to proptosis and poor vision. Two cases of meningioma were subjected to surgery due to proptosis with poor vision. One case was observed and subjected to radiotherapy due to good vision and slow growth. Discussion: We report the clinical presentation and management of primary orbital tumors of neurological origin from our part of the world. The current management scenario from our perspective is in concordance with that reported by other workers from different parts of the globe. Conclusion: Primary benign neural tumors of the orbit can be managed by surgery or observation as per the case. Our unique perspective on these tumors is invaluable to the world oncology literature.

Orbital tumors: A prospective study of 95 cases

Background: A wide variety of tumors and pseudotumors can involve the orbit. Although the incidence of orbital tumors is relatively low, the diagnosis and the treatment strategy for such tumors are of great concern to neurosurgeons and ophthalmologists. Objectives: The main aim of this study is to determine the distribution of patient age, sex, pathology, origin, and location of orbital tumors for optimum management because the delay in the diagnosis and treatment is the most preventable cause of morbidity and mortality. Materials and Methods: In this study, 95 cases of orbital tumors are studied prospectively during 10 years' period in the Hilla Teaching Hospital in Babylon-Iraq from 2008 to 2018. The parameters dealt with in this study included; the distribution of patient age and sex, clinical manifestations, investigations, pathology, origin of tumors and tumor location in the orbit, management, surgical procedures, and mortality in 95 patients (aged from 6 months to 75 years, the mean age is 29.8). Results: In this study, the highest age group incidence of orbital tumors is in the first decade of life which constitutes (24.2%), the second peak is in the fourth decade of life which constitutes (17.9%). 42 (44.2%) of the patients are males and 53 (55.8%) of the patients are females. Among the 95 cases, 76 (80%) are primary orbital tumors, 15 (15.8%) are secondary orbital tumors (tumors that originating from contiguous regions), and 4 (4.2%) are metastatic orbital tumors. Orbital tumors location; 83 (87.4%) are extraconal and 12 (12.6%) are intraconal. The two most common orbital tumors are dermoid cyst (27.4%) and pseudotumor (16.8%). Overall mortality is seven patients (7.4%). Conclusion: Orbital tumors are a relatively rare and challenging group of tumors. The age of onset, state of vision, tumor location in the orbit and other radiological findings provide the most important information for the diagnosis of orbital tumor prior biopsy or surgical resection and to make decision about other further treatment modality. For any patient complain from visual loss, proptosis, and impaired ocular motility, it is better to undergo magnetic resonance imaging and computed tomography-scan imaging to detect or exclude intraorbital or intracranial diseases.

Expanding the limits of endoscopic intraorbital tumor resection using 3-dimensional reconstruction

IntroductionEndoscopic orbital surgery is a nascent field and new tools are required to assist with surgical planning and to ascertain the limits of the tumor resectability. ObjectiveWe purpose to utilize three-dimensional radiographic reconstruction to define the theoretical lateral limit of endoscopic resectability of primary orbital tumors and to apply these boundary conditions to surgical cases. MethodsA three-dimensional orbital model was rendered in 4 representative patients presenting with primary orbital tumors using OsiriX open source imaging software. A 2-Dimensional plane was propagated between the contralateral nare and a line tangential to the long axis of the optic nerve reflecting the trajectory of a trans-septal approach. Any tumor volume falling medial to the optic nerve and/or within the space inferior to this plane of resectability was considered theoretically resectable regardless of how far it extended lateral to the optic nerve as nerve retraction would be unnecessary. Actual tumor volumes were then superimposed over this plan and correlated with surgical outcomes. ResultsAmong the 4 lesions analyzed, two were fully medial to the optic nerve, one extended lateral to the optic nerve but remained inferior to the plane of resectability, and one extended both lateral to the optic nerve and superior to the plane of resectability. As predicted by the three-dimensional modeling, a complete resection was achieved in all lesions except one that transgressed the plane of resectability. No new diplopia or vision loss was observed in any patient. ConclusionThree-dimensional reconstruction enhances preoperative planning for endoscopic orbital surgery. Tumors that extend lateral to the optic nerve may still be candidates for a purely endoscopic resection as long as they do not extend above the plane of resectability described herein.