Congenital orbital teratoma: a rare case with intracranial extension.

INTRODUCTION: Teratoma is the most common congenital tumor, but the orbital location is rare. It is composed of tissues from ectoderm, mesoderm, and endoderm. CLINICAL PRESENTATION: Congenital orbital teratoma commonly presents as unilateral proptosis, with rapid growth, leading to exposure keratopathy. DIAGNOSIS: Prenatal ultrasound may detect the orbital mass, computed tomography (CT) scans, and magnetic resonance (MR) imaging are better in demonstrating multilocular cystic and solid mass, without bone erosion. Laboratory tests should include alfa-fetoprotein (AFP) and B-human chorionic gonadotropin (B-HCG), and histopathologically, it contains all three germ cell layers components. The management is surgical removal of the lesion, the mature teratoma has a benign behavior, and the immature has a poor prognostic. We describe a rare case of congenital orbital teratoma with intracranial extension of the lesion, in which was treated with orbital exenteration. After surgery, AFP levels decreased, the middle face displacement has improved and development milestones were appropriate.

Pediatric orbital lesions: neoplastic extraocular soft-tissue lesions.

Pediatric neoplastic extraocular soft-tissue lesions in the orbit are uncommon. Early multimodality imaging work-up and recognition of the key imaging features of these lesions allow narrowing of the differential diagnoses in order to direct timely management. In this paper, the authors present a multimodality approach to the imaging work-up of these lesions and highlight the use of ocular ultrasound as a first imaging modality where appropriate. We will discuss vascular neoplasms (congenital hemangioma, infantile hemangioma), optic nerve lesions (meningioma, optic nerve glioma), and other neoplastic lesions (plexiform neurofibroma, teratoma, chloroma, rhabdomyosarcoma, infantile fibrosarcoma, schwannoma).

Conservative or surgical management of orbital schwannomas: a population-based case series.

INTRODUCTION: Orbital schwannomas (OS) are rare occurrences with no more than 500 cases reported in the literature. The tumor's potential to compromise the delicate neuro-ophthalmic structures within the orbit prompts surgical removal. Tumor removal is performed by ophthalmologists, often requiring a multidisciplinary surgical approach. The literature contains a very limited number of cases managed non-surgically. However, the inherent risks of orbital surgery warrant a comparison of the outcomes of conservative and surgical management strategies. AIMS: To review the national Swedish experience with the management of orbital schwannomas. METHODS: The study center is the primary Swedish referral center for the multidisciplinary management of orbital tumors, including schwannomas. During the period of 2005 to 2021, 16 patients with an OS diagnosis were managed at the center. RESULTS: Four patients initially underwent surgery where gross total resection (GTR) was achieved in three (75%) and subtotal resection (STR) in one (25%) case. The remaining 12 patients, who had a low risk of neuro-ophthalmic impairment, were managed conservatively with radiological and clinical examinations at regular intervals. After an average follow-up of 17 months, surgery was performed in three of these cases (25%). No recurrences or tumor growths were detected on radiological follow-ups (mean 50 months), and all patients experienced postoperative improvement at clinical follow-up (mean 65 months). The remainder of the conservatively treated patients (n=9) experienced no clinical progression (mean 30 months). A slight radiological tumor progression was detected in one patient after 17 months. CONCLUSION: There were no differences in long-term outcome between patients who had been managed with early surgery and those operated later after an initially conservative management. Conservatively treated patients had minimal to no symptoms and remained clinically stable throughout the follow-up period. Based on these findings, conservative management may successfully be adopted in cases with mild symptoms, no signs of compressive optic neuropathy and low risk of neuro-ophthalmic impairment. Conversion to surgical management is indicated upon clinical deterioration or tumor growth. Based on the findings of this study a decision tree for the management of orbital schwannomas is suggested.

Radiologic Features of Well-circumscribed Orbital Tumors With Histopathologic Correlation: A Multi-center Study.

PURPOSE: To delineate specific imaging characteristics of solitary fibrous tumors, schwannomas, cavernous venous malformations, and well-circumscribed orbital lymphoma. METHODS: Patients undergoing excisional biopsy of solitary fibrous tumor, schwannomas, cavernous venous malformations, or well-circumscribed orbital lymphoma with preoperative MRIs available for review were identified at 3 academic centers in the United States and Australia. An exploratory statistical analysis was performed to identify important radiologic features, which were subsequently included in a random forest model. Histopathologic correlates were evaluated in representative cases. RESULTS: A total of 91 cases were included with a mean age of 52.9 ± 17.2 years. Nearly all solitary fibrous tumors were located in the anterior or mid orbit (87.5%) and they more commonly demonstrated intralesional heterogeneity on T2-weighted imaging (45.5%) ( p < 0.01). Compared with the other tumors, schwannomas tended to be intraconal (66.7%) and were often in the mid or posterior orbit (83.4%) ( p < 0.01). Cavernous venous malformations characteristically demonstrated progressive contrast enhancement (93.9%; p < 0.01). Most lesions in all 4 groups were hypointense on T1-weighted imaging (80%-100%; p = 0.14) and only well-circumscribed orbital lymphoma tended to also be hypointense on T2 (81.8%) ( p < 0.01). Finally, cases of lymphoma had significantly lower apparent diffusion coefficient ratios (0.9 ± 0.2) ( p < 0.001), while the other 3 groups were not significantly different from one another (cavernous venous malformations: 1.8 ± 0.4; schwannomas: 1.8 ± 0.5; and solitary fibrous tumor: 1.6 ± 0.6) ( p = 0.739). CONCLUSIONS: Key features that aid in the differentiation of these 4 tumors from one another include T2 intensity and homogeneity, early contrast-enhancement pattern, and ADC ratio.

Correlation Between Radiologic and Histopathologic Features of Orbital Epidermoid and Dermoid Cysts.

PURPOSE: Epidermoid cysts (EC) and dermoid cysts (DC) typically appear as well-circumscribed lesions on CT. This study aimed to clarify the radiologic and histopathologic characteristics of orbital EC and DC and to determine the correlations between them. METHODS: The medical records of 69 patients who underwent surgery for orbital DC or EC at Samsung Medical Center between January 2001 and August 2016 were retrospectively reviewed. The size and location of the cysts, rim enhancement, homogeneity of contents, presence of hemorrhagic or calcific components, radiodensity of contents, and extent of bony remodeling were evaluated using CT. Additionally, the cyst lining and contents were examined histopathologically. RESULTS: Among patients with orbital cysts, EC and DC were diagnosed in 10 (14.5%) and 59 (85.5%) patients, respectively. Further, 50.0% of EC and 79.7% of DC were located in the superotemporal quadrant of the orbit. On orbital CT, the average radiodensity of EC and DC was 18.9 ± 56.2 and -67.9 ± 63.3 HU, respectively. The cystic contents were more frequently homogeneous than heterogeneous in both EC and DC; however, the radiodensity of cysts differed significantly, which may be attributed to sebaceous gland activity. Focal bony notching, bone remodeling under pressure, and bony changes from dumbbell-shaped cysts were observed more frequently in DC than in EC. CONCLUSIONS: Radiological and histopathological features are correlated in orbital EC and DC. Therefore, orbital EC and DC can be preoperatively differentiated using CT, based on the average radiodensity and bony remodeling.

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.

Double trouble: orbital rhabdomyoma with trichinellosis.

Rhabdomyoma of the orbit is a rare tumor with very few cases reported in the literature. We herein describe a 5-year-old boy who presented to us with a deviation of his left eye. Magnetic Resonance Imaging (MRI) showed a well-defined homogeneous intraconal mass in the superomedial aspect compressing the optic nerve. An excision biopsy was performed and the diagnosis of rhabdomyoma was confirmed on histopathology and immunohistochemistry with a coincidental finding of Trichinella spiralis larvae within the excised specimen. We report this phenomenon in two rare diseases with a predilection for striated muscle occurring simultaneously in a single patient.

A signature of structural MRI features at 3 Tesla allows an accurate characterization of orbital cavernous venous malformation.

OBJECTIVES: To differentiate OCVM from other orbital lesions using structural MRI. METHODS: This IRB-approved a historical-prospective cohort single-center analysis of a prospective cohort that included consecutive adult patients presenting with an orbital lesion undergoing a 3T MRI before surgery from December 2015 to May 2021. Two readers blinded to all data read all MRIs assessing structural MRI characteristics. A univariate analysis followed by a stepwise multivariate analysis identified structural MRI features showing the highest sensitivity and specificity when diagnosing OCVM. RESULTS: One hundred ninety-one patients with 30/191 (16%) OCVM and 161/191 (84%) other orbital lesions were included. OCVM were significantly more likely to present with a higher signal intensity than that of the cortex on T2WI: 26/29 (89.7%) versus 28/160 (17.5%), p < 0.001, or with a chemical shift artifact (CSA): 26/29 (89.7%) versus 16/155 (10.3%), p < 0.001, or to present with a single starting point of enhancement, as compared to other orbital lesions: 18/29 (62.1%) versus 4/159 (2.5%), p = 0.001. The step-wise analysis identified 2 signatures increasing performances. Signature 1 combined a higher signal intensity than that of the cortex on T2WI and a CSA. Signature 2 included these two features and the presence of a single starting point of enhancement. Sensitivity, specificity, and accuracy were 0.83, 0.94, and 0.92 for signature 1 and 0.97, 0.93, and 0.93 for signature 2, respectively. CONCLUSION: Structural MRI yields high sensitivity and specificity when diagnosing OCVM. KEY POINTS: • Structural MRI shows high sensitivity and specificity when diagnosing orbital cavernous venous malformation. • We identified two signatures combining structural MRI features which might be used easily in routine clinical practice. • The combination of higher signal intensity of the lesion as compared to the cortex on T2WI and of a chemical shift artifact yields a sensitivity and specificity of 0.83 and 0.94 for the diagnosis of orbital cavernous venous malformation, respectively.

Brain Tumors Affecting the Orbit Globe and Orbit Tumors Affecting the Brain.

Brain tumors affecting the orbit and orbital tumors affecting the brain are a heterogeneous group of lesions, with histological features, behaviors, diagnostic criteria, and treatments varying from each other. Dermoid cyst and cavernous hemangiomas are considered the most frequent benign lesions, while non-Hodgkin lymphoma is the most common malignant tumor in this region. Sharing the same anatomical region, clinical manifestations of orbital lesions may be often common to different types of lesions. Imaging studies are useful in the differential diagnosis of orbital lesions and the planning of their management. Lesions can be classified into ocular or extra-ocular ones: the latter can be further differentiated into extraconal or intraconal, based on the relationship with the extraocular muscles. Surgical therapy is the treatment of choice for most orbital lesions; however, based on the degree of removal, their histology and extension, other treatments, such as chemotherapy and radiotherapy, are indicated for the management of orbital lesions. In selected cases, chemotherapy and radiotherapy are the primary treatments. This chapter aimed to discuss the orbital anatomy, the clinical manifestations, the clinical testing and the imaging studies for orbital lesions, and the principal pathological entities affecting the orbit together with the principles of orbital surgery.

How I do it: micro-neurosurgical resection of spheno-orbital meningioma with customized orbit reconstruction technique.

BACKGROUND: Spheno-orbital meningioma (SOM) is a rare intracranial pathology with intraosseous hypertrophy and intraorbital extension. METHOD: We described a middle-aged female with SOM who was managed in a micro-neurosurgical manner. The titanium implant was customized and applied to rebuild the orbital wall to prevent postoperative enophthalmus. CONCLUSION: Despite technical demands, favorable cosmetic, and clinical outcomes without complications can be achieved by meticulous surgical technique following radical resection. CLINICAL TRIAL REGISTRATION: NA.

Superior divisional third nerve palsy due to breast cancer metastasis to the orbital apex.

PURPOSE: To report a case of breast cancer with metastasis to the orbital apex with manifestation of superior divisional third nerve palsy. CASE DESCRIPTION: A 40-year-old female, a known case of breast cancer diagnosed 3 years ago, referred to our neuro-ophthalmology clinic with chief complaint of ptosis and diplopia. In ophthalmic examination, visual acuities of both eyes were 20/20 and there was no significant finding in the anterior and posterior segment ophthalmic examination. There was left eye ptosis with MRD1 of 2 mm. In ocular movement examination, there was limitation of motion in supraduction (-2 to -3) of the left eye. Orbital MRI with contrast was requested for the patient which demonstrated a lesion in the orbital apex of the left eye. At the end, the patient was diagnosed as having superior divisional third nerve palsy. Orbital radiotherapy was recommended for the patient. CONCLUSION: In a patient with breast cancer who presents with diplopia, metastasis to the orbital apex with manifestation of divisional third nerve palsy should be kept in mind, and appropriate orbital imaging should be considered.

Primary optic nerve sheath schwannoma: a case report.

BACKGROUND: The optic nerve is an unusual site of schwannoma as it lacks Schwannoma cells on it. We report a primary optic sheath schwannoma and to review the literature. CASE REPORT: A 29-year old female presented with progressive painless non-pulsatile proptosis. Ocular examination revealed only axial proptosis. Imaging showed a well-defined intraconal mass abutting optic nerve. A left frontal craniotomy with orbitotomy and tumor excision was done. The tumor was well encapsulated, posteriorly attached to optic nerve without any plane, probable site of origin. The postoperative duration was uneventful without any complications. The histopathology examination confirms the diagnosis of schwannoma. CONCLUSIONS: We suggest to consider orbital optic nerve schwannoma in differential diagnosis of orbital tumors despite its exceedingly rare occurrence.

Clinical Characteristics and Prognostic Analysis of Osteolytic and Hyperostosis Sphenoid Orbital Meningiomas: A Single-Center Experience.

Reactive hyperostosis of spheno-orbital meningiomas (SOMs) often occurred in the sphenoid wing, while osteolytic SOMs (O-SOMs) were rarely discussed. This study preliminarily evaluated the clinical characteristics of O-SOMs and analyzed prognostic factors affecting the recurrence of SOMs. We retrospectively analyzed the medical records of consecutive patients who underwent surgery for a SOM between 2015 and 2020. According to the bone changes of sphenoid wing, SOMs were divided into O-SOMs and hyperostosis SOMs (H-SOMs). A total of 31 procedures were performed in 28 patients. All cases were treated by pterional-orbital approach. It was confirmed that 8 cases were O-SOMs and the other 20 cases were H-SOMs. Total tumor resection was performed in 21 cases. There were 19 cases with Ki 67 ≥3%. The patients were followed up for 3 to 87 months. Proptosis improved in all patients. All O-SOMs had no visual deterioration, while 4 H-SOMs cases had visual deterioration. There was no significant difference in clinical outcomes between the two types of SOM. The recurrence of SOM was related to the degree of resection, but not to the type of bone lesions, invasion of cavernous sinus and Ki 67.

Maligne Neoplasien der Orbita.

Malignant masses of the orbit include a large variety of neoplasms of epithelial or mesenchymal origin. The treatment of orbital malignancies is an essential interdisciplinary field of medicine that integrates ENT medicine, facial surgery, plastic surgery, neurosurgery, oncology and radiology.The main symptom of malignant orbital masses is the exophthalmos. A symptom that can help to differentiate a benign from a malignant orbital mass can be the pain. The main diagnostic tool is the MRI including new sequences like DWI and DCE.After presenting symptoms and diagnostic strategies of malignant orbital masses, this article starts with the description of malignant epithelial neoplasms of the lacrimal gland. Furthermore, it describes new insights in orbital lymphomas, followed by the discussion of semimalignant orbital masses. Last but not least the text deals with malignant neoplasms of the skin that can grow secondarily in the orbit. Finally, the manuscript discusses orbital metastases.

Primary orbital yolk sac tumor presenting as fungating mass.

Primary yolk sac tumor of the orbit is a rare entity. Orbital involvement is usually seen in young children and proptosis is the commonest presentation. Aggressive orbital involvement and presentation as a fungating mass is rarely seen. We report a case of primary orbital yolk sac tumor with an aggressive presentation that responded well to systemic chemotherapy.

Rare periorbital, pseudocystic metastasis of squamous cell carcinoma of the bladder: Case report and review of the literature.

We present the case of a man in his fifties with a history of bladder carcinoma who presented with a large periorbital cystic lesion that was found to be a metastasis. Bladder carcinomas are a very rare cause of peri-/orbital metastasis. The primary tumor in this case predominately showed squamous cell differentiation and small areas of adenoid differentiation. To our knowledge only one previous case of orbital metastasis from squamous cell carcinoma of the bladder has been reported. Cyst formation in bladder cancer metastasis has not been reported and is very rare for orbital metastases in general. The pathogenesis of metastatic cyst development is not fully understood and may vary from case to case. A biopsy of an atypical cyst is indicated.

Persistent orbital inflammation following complete excision of deep dermoid cysts.

Piecemeal excision of dermoid cysts carries the risk of implanting epithelial fragments into orbital fat, which is well recognized to continue secreting oily debris, inciting chronic, often granulomatous inflammation. The authors present the clinical and histological details for two patients with persistent lipogranulomatous inflammation for years after piecemeal excision of deep orbital dermoid cysts, in the absence of any residual epithelium. The importance of copious saline lavage - to 'float-out" and reduce microscopic lipid droplets - is also emphasised.

Childhood proliferative fasciitis of the orbit mimicking sarcoma: a case report.

Proliferative fasciitis (PF) is a benign fibroblastic reaction with histological and clinical characteristics that overlap with those of malignant soft tissue tumors; thus, it is referred to as a pseudosarcomatous reaction. It continues to be an important cause of diagnostic error and overtreatment. The childhood PF subtype has some distinct histological and immunohistochemical characteristics that make differential diagnosis with malignant tumors even harder, especially with sarcoma. These proliferations generally occur in the lower limbs, and the periorbital region is a rare location of appearance. Here, we describe a case of childhood subtype PF in a 16-year-old girl located in the periorbital area. To the best of our knowledge, this is the first reported case of childhood subtype PF in the periorbital area, and the third case if PF subtypes are not taken into account.

Computed tomography and magnetic resonance imaging of an unusual intraconal orbital osteoma.

We report the case of a 64-year-old male patient with a 5 month history of proptosis, motility limitation and vision loss in OD. Visual acuity (VA) was 20/200 in OD and 20/20 in OS. CT showed a large, round, intraconal lesion, with bony density and no apparent connection to adjacent orbital walls. MRI showed a T1-weighted hypointense lesion surrounded by a contrast enhancing capsule. The orbital tumor was excised through a lateral orbitotomy revealing a nodular, round, osseous structure. Histological examination disclosed well-formed lamellar bone trabeculae, with no necrosis or mitosis figures. Immunohistochemical staining was negative for MDM2 and CDK4. After 3 years, there was no evidence of tumor recurrence and VA had improved to 20/30. Intraconal osteomas with no clear attachment to orbital walls are extremely rare. We are aware of a few reported cases in the lid, hand, thigh, tongue, pterygopalatine fossa and brain. To the authors' knowledge, this is the first report in English literature of an orbital intraconal osteoma without any visible relation to the orbital walls.

Acute periocular haemorrhage associated with severe proptosis as a presenting sign of orbital tumours.

Periocular haemorrhage without a preceding history of trauma, and in the absence of vascular malformations is rare. While acute proptosis is well described in the setting of orbital pathology, accompanying periocular haemorrhage has been rarely reported. We describe three cases with these concomitant presenting signs in orbital malignancies - metastatic small cell bladder carcinoma, haemangiopericytoma (solitary fibrous tumour) and myeloma. Clinical photographs and radiology are presented with review of the current literature. All cases had an associated rapid onset of severe proptosis and co-existing periocular bruising on the same side. The presence of ecchymosis of the eyelids in addition to proptosis without a history of trauma warrants thorough investigation to rule out underlying potential sight and life threatening illness.