Cryoprobe-Assisted Excision of Cavernous Hemangioma of the Orbit: A Clinical Case Report.

This case report details the management of orbital cavernous hemangioma in a middle-aged female. The treatment involved a medial orbitotomy combined with an innovative application of a cryoprobe. This approach underscores the benefits of open orbitotomy, particularly when combined with cryoextraction. This combination provides improved control over bleeding and results in a substantial reduction in surgical time, offering valuable insights in the context of the contemporary trend towards endoscopic surgeries.

Imaging of the skull base and orbital tumors.

The skull base and orbit have complicated anatomical structures where various tumors can occur. The tumor may present with neurological symptoms; however, its diagnosis is clinically difficult owing to accessibility issues. Therefore, diagnostic imaging is crucial in assessing tumors in the skull base and orbit and guiding subsequent management. Notably, some tumors have a predilection for a specific site of origin, and identifying the site of origin on imaging can help narrow the differential diagnosis. At the skull base, chordomas typically occur in the clivus, chondrosarcomas in the paramedian areas, paragangliomas in the jugular foramen, neurogenic tumors, and perineural spread in the neural foramen. Among orbital tumors, cavernous hemangiomas usually occur in the intraconal space, and pleomorphic adenomas and adenoid cystic carcinomas occur in the lacrimal glands. Some skull base and orbital tumors exhibit distinctive imaging features. Chordomas and chondrosarcomas of the skull base show high signal intensities on T2-weighted images, with chondrosarcomas often displaying cartilaginous calcifications. Paragangliomas are characterized by their hypervascular nature. In the orbit, cavernous hemangiomas and pleomorphic adenomas present unique dynamic patterns. Immunoglobulin G4-related disease forms lesions along the nerves. Identifying the tumor origin and its imaging characteristics can help narrow the differential diagnosis of skull base and orbital tumors.

Post-operative outcomes of primary benign orbital tumor resection are independent of tumor size and morphology.

KEY POINTS: Using 3D modeling, we studied the influence of orbital tumor morphology on post-operative outcomes. Orbital tumor volume did not influence postoperative complication rates. Less spherical tumors may be correlated with a more extensive surgical approach.

Bilateral Lacrimal Gland Lymphoma - Case Presentation.

Lacrimal gland lymphomas are rare orbital tumors, constituting a minor fraction of all orbital and ocular adnexal malignancies. This case study presents an 83-year-old male with bilateral lacrimal gland tumors, more prominent in the left orbit, causing decreased visual acuity, red eye, excessive tearing, and diplopia. Initial ophthalmological evaluations and imaging suggested bilateral lacrimal gland lymphoma, confirmed by histopathology as diffuse large B-cell non-Hodgkin lymphoma of the MALT type. Due to the significant tumor size and risk of visual function loss, surgical intervention was performed, followed by corticosteroid therapy. Postoperatively, a marked improvement in symptoms and a reduction in tumor size were observed. This case underscores the importance of comprehensive diagnostic approaches, including clinical, imaging, and histopathological evaluations, highlighting the need for a multidisciplinary approach in managing rare orbital tumors like lacrimal gland lymphoma. The patient's postoperative and follow-up care included oncological management to monitor and ensure long-term disease control and patient well-being. Abbreviations: RE = right eye, LE = left eye, CT = Computer tomography, MRI = Magnetic Resonance Imaging, TOD = intraocular pressure of right eye, TOS = intraocular pressure of left eye, US = ultrasound.

Spheno-Orbital Meningiomas: The Rationale behind the Decision-Making Process of Treatment Strategy.

Surgery stands as the primary treatment for spheno-orbital meningiomas, following a symptoms-oriented approach. We discussed the decision-making process behind surgical strategies through a review of medical records from 80 patients who underwent surgical resection at the University of Naples Federico II. Different surgical approaches were employed based on the tumor's location relative to the optic nerve's long axis, categorized into lateral (type I), medial (type II), and diffuse (type III). We examined clinical, neuroradiological, surgical, pathological, and outcome factors. Proptosis emerged as the most frequent symptom (97%), followed by visual impairment (59%) and ocular motility issues (35%). Type I represented 20%, type II 43%, and type III 17%. Growth primarily affected the optic canal (74%), superior orbital fissure (65%), anterior clinoid (60%), and orbital apex (59%). The resection outcomes varied, with Simpson grades I and II achieved in all type I cases, 67.5% of type II, and 18% of type III. Recurrence rates were highest in type II (41.8%) and type III (59%). Improvement was notable in proptosis (68%) and visual function (51%, predominantly type I). Surgery for spheno-orbital meningiomas should be tailored to each patient, considering individual characteristics and tumor features to improve quality of life by addressing primary symptoms like proptosis and visual deficits.

Optic Nerve Schwannoma: A Report of a Rare Case From India and Literature Review.

Optic nerve schwannoma is a very rarely occurring tumor described in the literature. It is due to the fact that the optic nerve is myelinated by oligodendrocytes. Schwannomas are tumors of the peripheral nervous system, hence optic nerve schwannoma is a rare phenomenon. A 34-year-old patient presented in the outpatient department with complaints of gradual painless protrusion of the left eye (LE) for the past one year. There was no history of diminution of vision. On examination, vision in both eyes was 6/6, anterior segment examination in both eyes was normal, and pupils were central, circular, and reacting to light. Intraocular pressure was measured on a noncontact tonometer and was within normal range. Both eyes' optic disc, fundus, and visual fields were normal. On inspection, axial proptosis was noted in the LE. Proptosis measurement (on Hertel exophthalmometer) in the right eye was 17 mm and in the left eye was 21 mm. MRI of the orbit without contrast was done and showed a well-defined, soft tissue lesion of the optic nerve in the intraconal compartment of the left orbit. Surgical excision of the tumor was done by lateral orbitotomy approach and the tumor was removed in total. Histopathological examination of the mass revealed a benign spindle cell neoplasm suggestive of schwannoma. Postoperatively, proptosis was resolved, 17 mm both in the right and left eye (on Hertel exophthalmometer), and vision in LE remained unchanged (6/6). Postoperatively, intraocular pressure (on noncontact tonometer) was within normal range, and the optic disc, fundus, and visual fields were normal.

The value of cryoablation in orbital surgery.

Aim of this article is to give an overview of the technical background and the advantages of modern devices for different applications of cryoablation in cranio-orbital neurosurgery.The treatment of orbital lesions is complicated by the complex and potentially inapparent anatomy due to retro-orbital fat. With the help of cryoprobes different well-defined lesions such as cavernous venous malformations can be safely and effectively removed thanks to the cryoadhesive effect. Their use has been described in several different approaches including traditional lateral or transcranial orbitotomy but also anterior transconjunctival as well as transnasal endoscopic approaches. Recently, single-use devices were introduced that allow the use of cryosurgery also without the need for large investment or service costs.

Do Minimally Invasive Approaches to Pediatric Orbital Tumors Provide an Advantage on Outcome and Efficiency?

OBJECTIVE: The present study evaluated whether minimally invasive approaches to orbital lesions could improve surgical, clinical, and aesthetic outcomes compared with more invasive ones. This is the first study specifically addressing this topic in children. METHODS: Children consecutively operated on from January 2010 to January 2020 were analyzed. Thirty patients matched the inclusion criteria and were divided into group A: 14 cases treated with traditional surgical approaches; and group B: 16 cases managed by minimally invasive approaches. RESULTS: There were no significant differences between the 2 groups in terms of demographic data and extent of tumor resection. Mean surgical time for the approach (40 minutes vs. 70 minutes, P < 0.0001), surgical complication such as periorbital edema (37% vs. 78%, P = 0.02) and dural tear (0 vs. 21%, P = 0.05), and procedures cost (P < 0.0001) were significantly reduced in group B. Regarding clinical outcomes, group B showed a significant reduction both in terms of postoperative pain (mean score based on visual pain scale was 2.9 vs. 4.1 P = 0.003) and mean hospitalization time (4.5 days vs. 5.5 days, P = 0.0004). The cosmetic outcome according to the Sloan classification was significantly better in group B as well (81% vs. 36% class I patients, P = 0.005). CONCLUSIONS: The use of mini-invasive approaches to orbital tumor has clear advantages in terms of surgical, clinical, and cosmetic outcomes in comparable patients; therefore, they should be preferred whenever feasible. Craniotomic approaches remain necessary for very large tumors.

Orbital Intradiploic Epidermoid Cyst: A Case Report of a Rare Entity.

Cranial epidermoid cysts are relatively rare. More frequently reported in middle-aged men with a wide variety of signs and symptoms such as headache, seizures, cerebellar and cranial nerve deficits/visual disturbance. The approach for surgical removal of the cyst depends on its size and location. In addition, a multidisciplinary team must be involved due to the common occurrence of misdiagnosis. We present the unusual age of presentation for intradiploic epidermoid cysts. A 14-year-old boy is complaining of a 2-month history of painless progressive swelling of the right eyebrow. Magnetic resonance imaging revealed an intradiploic cystic mass within the right frontal bone. The cystic mass was removed, and histological examination confirmed the diagnosis of an epidermoid cyst. This case illustrated the potential of developing intradiploic epidermoid cysts in pediatrics.

Endoscopic Endonasal Approach to Intraconal Orbital Tumors: Outcomes and Lessons Learned.

BACKGROUND: Lesions involving the intraconal space of the orbit are rare and challenging to manage. Operative techniques and outcomes for the endoscopic endonasal approach (EEA) to tumors in the medial intraconal space (MIS) remain poorly characterized. OBJECTIVE: We present our experience with a wide range of isolated intraconal pathology managed via an EEA. METHODS: A retrospective review of all cases (2014-2021) performed by a single skull base team in which the EEA was employed for the management of an intraconal orbital lesion. RESULTS: Twenty patients (13 men, 7 women) with a mean age of 59 years (range, 40-89 years) were included. All lesions were isolated to the MIS, pathology addressed included: cavernous hemangioma (6), schwannoma (4), lymphoma (4), inflammatory pseudotumor (2), chronic invasive fungal sinusitis (2), and metastatic disease (2). Either a biopsy (10/20) or a complete resection (10/20) was performed. In all cases, the MIS was accessed via an endonasal corridor between the medial and inferior rectus muscles. Retraction and safe, intra-orbital dissection of the lesion was performed using a two-surgeon, multi-handed technique. Gross total resection of benign lesions was achieved in 90% (9/10) of cases; a pathologic diagnosis was achieved in 100% (10/10) of biopsy cases. No orbital reconstruction was required. Visual acuity returned to normal in 80% (8/10) of planned resection cases and postoperative diplopia resolved by 3 months in 90%. Mean follow-up was 15 months. CONCLUSION: This study demonstrates that the EEA is safe and effective for accessing lesions in the MIS. This technique affords very favorable outcomes with minimal postoperative morbidity. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:47-55, 2024.

Radiotherapy of Orbital and Ocular Adnexa Lymphoma: Literature Review and University of Catania Experience.

Orbital and ocular adnexa lymphomas are rare neoplasms confined to the orbital region. The prognosis is generally favorable, with a high proportion of localized disease, indolent clinical course, prolonged disease-free intervals, and low lymphoma-related mortality rate. We report our experience on eleven patients with confirmed histological diagnosis of lymphoma stage IE-IIE, treated between 2010 and 2021 with radiotherapy alone or in association with chemotherapy or immunotherapy. Eight patients were treated with primary radiotherapy only, while three received previous systemic treatments. Six patients were treated with Proton beam therapy (PBT), and five with external beam radiotherapy (EBRT). The five-year local control rate was 98%; only one patient developed an out-of-field recurrence. We also conducted a comprehensive literature review using electronic databases (PubMed, EMBASE, and Cochrane Library). Articles were selected based on their pertinence to treatment of the ocular and adnexal lymphoma focusing on radiotherapy techniques (electron beam radiotherapy, photon beam radiotherapy, or proton beam radiotherapy), treatment total dose, fractionation schedule, early and late radio-induced toxicities, and patient's clinical outcome. Radiotherapy is an effective treatment option for orbital lymphoma, especially as standard treatment in the early stage of orbital lymphoma, with excellent local control rate and low rates of toxicity.

Orbital Exenteration in Recurrence Cancer: 5 Years Experience.

INTRODUCTION: The purpose of this study was to assess the overall survival (OS) and disease-free survival (DFS) of patients who underwent orbital exenteration for periorbital, conjunctival, and primary intraorbital carcinomas. Additionally, we assessed the outcomes of anterior retrograde temporalis muscle flap restoration. METHODS: For all patients who had orbital exenteration in the previous five years, a non-comparative retrospective assessment of their medical records, histology, and radiographic imaging was carried out. We investigated the relationships between the various qualitative factors using Cramer's V Kaplan-Meier (KM) analysis. For each of the patient's categorical factors that were of relevance, estimates of the survival distribution were displayed, and log-rank tests were used to determine whether the survival distributions were equal. RESULTS: This study looks at 19 participants. The sample is made up of 13 men (68%) and 6 women (32%). The degree of relationship (Cramer's V index) between lymph node metastases (N) and the existence of distant metastases (M) is high, at 64%, and is statistically significant because the p-value is 0.0034 < 0.005. Lymph node metastases had a statistically significant impact on overall survival (p = 0.04 < 0.05). Thirteen of the nineteen patients tested had no palsy (68%). There was no one presenting a CSF leak. CONCLUSION: Our findings show how crucial it is to identify any lymph node involvement that orbital neoplasms may have. In patients who have received many treatments, sentinel lymph node biopsy (SLNB) may be used to determine the stage and spread of the cancer. To determine whether additional tumor characteristics may be explored, more expertise in the SLNB field for patients with orbital cancer who have received many treatments may be helpful. To prevent additional scarring and to be comparable to previous techniques for facial nerve lesions, the anterior retrograde approach and the transorbital procedure for temporal muscle flap in-setting are both effective methods.

Targeted therapy and immunotherapy for orbital and periorbital tumors: a major review.

Traditionally, for patients who are poor candidates for surgery and/or radiotherapy, palliative chemotherapy is often offered but with significant toxic side effects. However, recent advancements in our understanding of tumor biology and molecular genetics have brought new understanding to the molecular pathways of certain tumors and cancers. This has ushered in a new era of precision medicine specific to a tumor or cancer treatment pathway (targeted therapy) or directed to host-tumor responses (immunotherapy). This article will focus on recent updates in the application of available targeted and immunotherapy for managing orbital and periorbital tumors and tumor-like conditions, which include cutaneous basal cell carcinoma, cutaneous squamous cell carcinoma, cutaneous melanoma, Merkel cell carcinoma, sebaceous gland carcinoma, solitary fibrous tumor, dermatofibrosarcoma protuberans, orbital meningioma, neurofibromatosis, Langerhans cell histiocytosis, ocular adnexal lymphoma, orbital lymphatic malformation, and adenoid cystic carcinoma.

Primary optic nerve sheath schwannoma: A case report and literature review.

Schwannomas are benign tumors of the peripheral nervous system that arise from Schwann cells. Intracranial schwannomas most commonly arise from the vestibulocochlear nerve, followed by the fifth nerve. However, optic nerve sheath schwannomas are very rare due to the lack of Schwann cells on it. A few exceptional cases of optic nerve sheath schwannoma have been described in the literature. In this article, we report a rare case of primary optic nerve sheath schwannoma in a 48-year-old woman who presented with progressive visual loss and left-sided proptosis. Magnetic resonance imaging revealed a well-demarcated intraconal orbital mass with cystic appearance and rim enhancement. The diagnosis of optic nerve schwannoma was proposed and confirmed histologically after surgical resection.

Orbital Cavernous Venous Malformation in a 35-Year-Old Man: A Case Report.

In this article we present a case of a 35-year-old patient with a massive 43 × 35 × 34 mm cavernous venous malformation of the left orbit. The orbital lesion was initially observed in 2008 and remained untreated to 2021 due to the patient's refusal to consent to the surgical procedure; which caused the tumor to grow to monstrous dimensions.

Endoscopic Transorbital Approach for Spheno-Orbital Tumors: Case Series and Systematic Review of Literature.

BACKGROUND: Transorbital neuroendoscopic surgery (TONES) comprises a group of approaches with indications expanding from orbital tumors to more complex skull base lesions. We analyzed the role of the endoscopic transorbital approach (eTOA) for spheno-orbital tumors, reporting the results of our clinical series and of a systematic review of the literature. MATERIALS AND METHODS: All patients operated on from 2016 to 2022 at our institution for a spheno-orbital tumor through an eTOA were included in a clinical series, and a systematic review of the literature was performed. RESULTS: Our series consisted of 22 patients (16 females, mean age 57 ± 13 years). Gross tumor removal was achieved in 8 patients (36.4%) after the eTOA and in 11 (50.0%) after a multistaged strategy combining the eTOA with the endoscopic endonasal approach. Complications included 1 chronic subdural hematoma and 1 permanent extrinsic ocular muscle deficit. Patients were discharged after 2.4 ± 1.3 days. The most common histotype was meningioma (86.4%). Proptosis improved in all cases, visual deficit in 66.6%, and diplopia in 76.9%. These results were confirmed by the review of the 127 cases reported in the literature. CONCLUSIONS: Despite its recent introduction, a significant number of spheno-orbital lesions treated with an eTOA are being reported. Its main advantages are favorable patient outcome and optimal cosmetic results, with minimal morbidity and quick recovery. This approach can be combined with other surgical routes or adjuvant therapies for complex tumors. However, it is a technically demanding procedure, requiring specific skills in endoscopic surgery, that should be reserved to dedicated centers.

Monocular visual loss in a disseminated colorectal malignancy-A case report of a rare skull base metastasis.

Neoplasms of the orbit may be primary, secondary (infiltration from the adjacent structures), or metastatic (from distant structures). It can be divided into 3 histologic categories: benign, benign but locally aggressive, and malignant. Primary and secondary orbital tumors, including intra-orbital and optic nerve tumors are uncommon observations in daily medical practice. Orbital tumors represent approximately 0.1% of all tumors and approximately 18% of all orbital diseases. We report a case of a 42 year old male patient with colorectal malignancy with basal skull metastasis involving the orbital apex and involving the optic nerve causing visual loss.

Spontaneous regression of an orbital Langerhans cell histiocytosis after biopsy: A case report. Spontaneous regression of an orbital Langerhans cell histiocytosis.

Langerhans histiocytosis or Langerhans cell histiocytosis (LCH) is a rare benign pathology representing less than 1% of orbital tumors. It can cause either localized or generalized lesions, leading to the destruction of hard and soft tissues. Eosinophilic granuloma is the most benign form and the predominant clinical presentation of LCH. We report a case of eosinophilic granuloma with orbital involvement in an 18-year-old male patient. Orbital radiotherapy was initially planned, but finally it was not performed due to a spontaneous regression of the lesion after the incisional biopsy. The presented case supports an expectant attitude given the possibility of a spontaneous regression after the biopsy, especially in small lesions. However, long-term follow-up is essential given the risk of recurrence.

Extraocular Muscle Biopsy: Surgical Technique and Indications.

Extraocular muscle biopsy is a technique used for the histological diagnosis of several orbital diseases; it could bring valuable assistance to the diagnostic process. The purpose of this article is to review the indications, surgical technique, and complications of this biopsy. Extraocular muscle biopsy is frequently used in tumoral pathology, however, in inflammatory diseases, it is only used when facing a poor reaction to the treatment or in case of chronicity. The surgical procedure must be precise and least traumatic, in order to avoid intra- and post-operative complications, but also to preserve the integrity and function of the muscle. This procedure remains non-standardized and not devoid of complications, hence the need for a careful evaluation of the benefit/risk ratio before considering it.

Correlation between Frozen Section and Permanent Histopathologic Diagnoses in Adult Orbital Lesions.

Introduction: When indicated, intraoperative use of frozen sections may assist in determining the surgical course or appropriate processing of surgical specimens. Knowing the accuracy of a preliminary frozen section diagnosis is important. The purpose of this study is to determine the rate of correlation between frozen and permanent histopathologic diagnoses of adult orbital lesions, analyze characteristics of discordant cases, and examine the effects of discordance on surgical decision-making. Methods: A 15-year retrospective chart review was conducted at a tertiary care center of all adult patients with orbital lesions for which frozen section and corresponding permanent section tissue diagnoses were obtained. Results: Sixty-five orbital surgeries were performed with a total of 89 frozen sections sampled. In 63 surgeries (96.9%), at least 1 frozen section diagnosis matched the final permanent section diagnosis. Overall, frozen section diagnosis corresponded with permanent section diagnosis in 81 of 89 (91.0%) specimens. Of the 8 (9.0%) specimens from 5 unique patients that did not correlate, the final diagnoses on permanent sections were amyloidosis (5), margin-positive infiltrating breast carcinoma (2), and lymphoid hyperplasia (1). The discrepancy between frozen and permanent sections did not alter care in any patient. Conclusion: Frozen section diagnoses correlate with permanent histopathologic tissue diagnosis in adult orbital biopsies in greater than 90% of cases. Among non-correlated specimens, amyloidosis was the most common diagnosis. Although rare, orbital amyloid disorders may be considered in the differential diagnosis of cases of orbital biopsies with nonspecific findings on a frozen section.