Gamma knife radiosurgery for orbital cavernous hemangioma: a systematic review and single-arm meta-analysis.

PURPOSE: Gamma knife radiosurgery (GKRS) for orbital cavernous hemangioma (OCH) has emerged as a promising method due to its significant clinical improvement and low incidence of complications. This study aimed to evaluate the safety and efficacy of GKRS for the treatment of OCH. METHODS: In accordance with the PRISMA framework, we searched PubMed, Cochrane Central, and Embase for studies reporting outcomes of GKRS for OCH. Studies reporting complications, visual improvement, proptosis, tumor reduction rate, and tumor progression rate for OCH following GKRS were included. RESULTS: Six studies, out of 1856 search results, with 100 patients were included. Among them, only 5 minor complications were related to GKRS, including 3 with orbital pain and 2 with periorbital chemosis. Thus, the complication rate was 13% (95% CI, 7-25%). Visual acuity and visual field improvement rates after GKRS were 80% (95% CI, 63-96%) and 71% (95% CI, 47-95%) respectively. Proptosis improved in 94% of cases (95% CI, 83-100%). The tumor reduction rate was 77% after GKRS (95% CI, 69-85%). CONCLUSION: GKRS for OCH appears to be a safe technique, as evidenced by the rate of clinical improvement and radiological improvement. However, studies are limited by an absence of a control group. Additional studies are needed to evaluate the relative efficacy of GKRS as compared with alternative surgical modalities for OCH.

A Case of Blastic Plasmacytoid Dendritic Cell Neoplasm with Orbital Tumor as the Initial Symptom.

BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy arising from precursor dendritic cells. It is a rare and challenging clinical presentation. For decades, there has been no treatment course for managing BPDCN and its overall prognosis is poor. METHODS AND RESULTS: We report a 27-year-old man who was admitted to the hospital due to an orbital tumor as the first symptom. Progressive enlargement of the orbital tumor was accompanied by multiple purple circular nodules on the body trunk. Pathological confirmation of BPDCN after resection of the orbital mass. Bone marrow smear and flow cytometry on examination indicate AML-M5. Performance of chemotherapy and peripheral blood autologous stem cell transplantation. CONCLUSIONS: The clinical manifestations of blastic plasmacytoid dendritic cell neoplasms are diverse. The diagnosis of BPDCN can be difficult due to overlapping morphologic, immunophenotypic, and clinical features of other hematologic AML. Relapsed and refractory BPDCN remains an elusive therapeutic challenge. The future of new targeted therapeutic drugs is expected.

23-year review of spheno-orbital meningioma: clinical, radiological, and pathological insights from 100 cases.

BACKGROUND: Spheno-orbital meningioma (SOM) represents a unique variant of sphenoid wing meningiomas, distinguished by its propensity for bone infiltration and cranio-orbital involvement. SOM exhibits a considerable incidence of misdiagnosis and recurrence. PURPOSES: To elucidate the clinical, radiological, and pathological characteristics of SOM. METHODS: Review of electronic medical records, histopathology, radiological images and follow-up information of 100 SOM patients. RESULTS: Of the 100 patients (28 males, 72 females) with SOM, mean age was 46.8 ± 12.6 years and prevalent symptoms were proptosis (99%). All the CT scans showed hyperostosis with 89.3% of the hyperostosis having an irregular edge. In MRI scans, dural tail sign was observed across all patients and the cranio-orbital tumors often penetrated temporal muscle (74.1%), extraocular muscle (74.1%) and lacrimal gland (63%). All the 100 patients underwent surgical intervention, and among them, 62 individuals received postoperative radiotherapy. Grade I resections had a lower recurrence rate(16.7%), which further decreased with the addition of radiotherapy(13.9%). In contrast, all patients with grade II or higher grade resections without radiotherapy experienced recurrence, indicating a higher risk associated with less complete tumor removal. The pathological examination revealed that intraorbital sections exhibited comparable tumor density to intraorbital SOM tumors, along with increased fibrous density but decreased vascular distribution. CONCLUSIONS: Radiological characteristics of SOM included cranio-orbital tumors, hyperostosis of the sphenoid wing with an irregular edge, and dural tail sign. Combination of gross total resection and adjuvant radiotherapy was recommended to minimize recurrence rate. Intracranial SOM tumors tended to be softer and more bleed-prone than intraorbital sections, necessitating surgical precision.

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.

Assessing the Usefulness of Ultrasonography for the Diagnosis and Evaluation of Intra-Orbital Lesions in Pediatric Patients: A Retrospective Analysis.

OBJECTIVES: To assess the usefulness of ultrasonography in the diagnosis and evaluation of extraocular intra-orbital lesions in pediatric patients. METHODS: Twenty-three pediatric patients with intra-orbital lesions who underwent both ultrasound and computed tomography/magnetic resonance imaging (CT/MRI) were included. The following parameters were evaluated using ultrasound: 1) lesion detection rate (presence or absence of lesions), 2) lesion characteristics, 3) lesion location (extraconal or intraconal), and 4) the lesion longest linear dimensions, and these were compared using Fisher's exact test and Mann-Whitney U test. RESULTS: Two lesions could not be detected using ultrasound; in the other 21 cases, the lesion characteristics diagnosed by ultrasound were correct. Diagnostic accuracy of detection and characteristics assessment using ultrasound were 91.3% and 91.3%, respectively. The lesion location was not significantly different between the two groups (intraconal/extraconal in those detected using ultrasound versus those in the absence on ultrasound = 7/14 versus 0/2, P > .999); however, in two cases that were not detected on ultrasound, the lesions were located at extraconal. Lesions that were small in longest linear dimensions on CT/MRI were not detected using ultrasound (the longest linear dimensions in lesions detected using ultrasound versus that in the absence of ultrasound: 29.5 ± 8.2 [range, 13-46] versus 10 and 11 mm, P = .043). CONCLUSIONS: Ultrasonography proved to be useful for visualizing and evaluating intra-orbital lesions except for lesions that were relatively small in size. Therefore, although ultrasound could not detect lesions located behind bone and bone invasion, it could be used for diagnosing and selecting treatment strategies for intra-orbital lesions.

Endoscopic resection of primary sinonasal mucosal melanoma with orbital invasion: How I do it.

Primary sinonasal mucosal melanoma is a rare aggressive malignancy. In this video, a case of a 68-year-old female who presented with diplopia for 2 weeks is described. The present video reports the endoscopic endonasal surgical excision of a primary sinonasal mucosal melanoma. The video contains patient's medical history, preoperative radiological evaluations and step-by-step description of surgical steps of the procedure with the utilization of computer-assisted navigation system.

Hybrid Schwannoma-Perineurioma of the Orbit.

A 52-year-old woman presented with a 6-month history of progressive right proptosis associated with intermittent right retrobulbar and facial pain. MRI revealed a heterogeneously enhancing, well-circumscribed, ovoid, soft tissue mass in the intraconal space near the right orbital apex displacing the optic nerve medially. Excisional biopsy established the diagnosis of a schwannoma-perineurioma hybrid peripheral nerve sheath tumor (HPNST). This case represents only the second reported occurrence, to our knowledge, of an orbital schwannoma-perineurioma HPNST.

A Rapidly Expanding Hemorrhagic BRAF-Mutant Orbital Atypical Glomus Tumor.

A healthy 32-year-old woman presented with the acute onset of left sided eye pain, upper eyelid fullness, and binocular diplopia during light weightlifting. Examination elevated intraocular pressure, proptosis, upper eyelid ptosis, and motility deficits. CT demonstrated a well-circumscribed, homogeneous-appearing extraconal mass in the superior left orbit. The patient underwent an urgent orbitotomy with the excision of a hemorrhagic mass. Histopathology showed a glomus tumor with atypical features and hemorrhagic infarction, best classified as having uncertain malignant potential. A B-Raf proto-oncogene V600E mutation was detected with immunohistochemistry, which suggests a more aggressive tumor behavior yet presents an opportunity for targeted primary or adjunctive therapy. This is the first reported case of a B-Raf proto-oncogene-mutant atypical glomus tumor arising in the orbit.

Orbital Liposarcoma Progression From a Well-Differentiated to a Dedifferentiated Type in a Young Patient.

A young male patient presented with unilateral proptosis of the left eye. Laboratory tests and an initial computed tomography scan of the orbit were compatible with euthyroid orbitopathy. He subsequently underwent decompression surgery for unfavorable cosmesis and corneal exposure symptoms with good results, but returned 1 year later for recurrence of proptosis. There was a superomedial fatty density extraconal mass on computed tomography scan. A biopsy done a year later confirmed the diagnosis of well-differentiated liposarcoma from the previously noted mass and a dedifferentiated component in the newly appeared mass in the intraconal space. Orbital exenteration with radial forearm free flap was performed followed by orbital radiotherapy. No disease recurrence or metastasis was seen on follow-ups for 7 years since undergoing treatment.

Melanoma Arising Beneath the Lateral Rectus Muscle in a Teenager With Ocular Melanocytosis: Possible Origin From Intrascleral Melanocytes.

Ocular melanocytosis is a well-established risk factor for choroidal melanomas but, despite its reported associations in the literature, it is infrequently discussed in relation to orbital melanomas. The authors describe a teenage patient with ocular melanocytosis who presented with an asymptomatic ipsilateral right orbital mass associated with the lateral rectus muscle. An exploratory orbitotomy revealed a lesion lightly adherent to the underlying sclera. Histopathology demonstrated a markedly atypical epithelioid melanocytic proliferation, bound by a thin rim of superficial sclera, implying an origin from intrascleral melanocytes, likely within an emissary canal. Next-generation sequencing identified GNAQ and NF1 mutations. The histopathology and molecular genetics designated the lesion as having a uveal melanoma-like profile, suggesting that it may behave as a choroidal melanoma. This case underscores the importance of the association between ocular melanocytosis and orbital melanoma and provides additional evidence for primary orbital melanoma etiopathogenesis.

Sinonasal Chondrosarcoma With Unusual Orbital Invasion.

A case is presented of a 43-year-old male with a chronic history of progressive nasal obstruction and epiphora. MRI confirmed a heterogeneous mass involving the middle and superior turbinates with T2 hyperintense and calcified components, with extension into the inferomedial orbit. Tissue biopsy revealed a grade 2 chondrosarcoma of the conventional subtype. Endonasal wide local resection of the lesion was performed with clear margins. The patient had no functional sequelae and will undergo routine surveillance.

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.

Clinical characteristics and treatment outcomes of secondary ocular adnexal lymphoma.

BACKGROUND: To describe the clinical characteristics and treatment outcomes of secondary ocular adnexal lymphoma (OAL), and emphasise the impact of timely biopsy and systemic evaluation on the diagnosis and treatment. METHODS: The data of patients with secondary OAL in our hospital from January 2010 to June 2021 were retrospectively reviewed. RESULTS: A total of 54 patients (30 men and 24 women) were included in the study. The mean age at presentation was 60 years (median 62 years; range 37-83 years). The main symptoms included ocular mass (74%), periorbital swelling (43%), and proptosis (39%). The main histopathological types were mucosa-associated lymphoid tissue lymphoma (30%), diffuse large B-cell lymphoma (28%), and natural killer/T-cell lymphoma (18%). In particular, three patients exhibited different pathological types of ocular lymphoma compared to previously diagnosed systemic lymphoma. The most common site of ocular and systemic involvement was the orbit (85%) and lymph nodes (56%), respectively, and 25 (46%) patients had occult extraocular lesions. Additionally, the 5-year overall survival rate of the entire cohort was 56%, and significant differences were observed between the three main pathological types and stages of the disease (all p < 0.001). CONCLUSIONS: Secondary OAL is a rare disease with a poor prognosis. That the disease is associated with a more aggressive pathological type is well established, indolent lymphoma is not uncommon. Furthermore, OAL and previously diagnosed systemic lymphoma may be pathologically distinct in some patients. Therefore, we recommend a prompt excision biopsy and a thorough systemic evaluation of patients with suspected OAL.

Multiple CD34-Negative Orbital Solitary Fibrous Tumors in a Pediatric Patient.

A 12-year-old Japanese male presented with a 2-month history of headache that was later on associated with diplopia, painless proptosis of the OS, and left ophthalmoplegia. Initial examination revealed a 7-mm OS protrusion, which worsened to 9 mm in less than a month. Preoperative visual acuity worsened from 1.0 to 0.2 with the development of left afferent pupillary defect. Left ocular motility was severely restricted in all directions. Magnetic resonance imaging showed two well-defined lesions adjacent to one another in the left orbit. The patient underwent surgical excision of the left orbital masses. Histopathology findings were consistent with solitary fibrous tumor of the orbit. Immunohistochemistry findings revealed CD34-negative but signal transducer and activator of transcription 6-positive for both specimens. The patient was monitored postoperatively and there was no recurrence of the tumor even after 6 months.

Orbital Dermoid Cysts in Monozygotic Twins.

Two healthy 12-year-old monozygotic twin sisters presented with strikingly similar, painless orbital masses along their frontozygomatic suture line that had been slowly enlarging since birth. The masses were clinically consistent with orbital dermoid cysts and the patients underwent excision of their lesions, with the diagnosis confirmed by histological analysis. There are prior case reports of both nasal and ovarian dermoid cysts in twins, however, no prior case of orbital dermoid cysts in twins have been described. These dermoid cysts are generally thought to be a sporadic disorder of embryogenesis, yet the authors' case suggests genetics may play a role in the underlying etiology of dermoid cysts.

Thirty Years of Experience With Ocular Adnexal T-Cell Lymphoma.

PURPOSE: To evaluate the disease characteristics and survival of patients with ocular adnexal T-cell lymphoma. METHODS: A retrospective, observational study of patients with a histopathological diagnosis of T-cell lymphoma of the ocular adnexa seen between 1992 and 2022. Demographic data, clinical presentation, imaging, histology, immunohistochemistry, treatment, and outcomes were reviewed. RESULTS: Fifteen patients were included in the study with the mean age at diagnosis of 50 years old (range 7-85). The most common presenting symptoms were ulcerated eyelid skin lesions (40%) followed by eyelid swelling (13.3%), and lacrimal passageways obstruction (13.3%). The anaplastic large cell lymphoma (33%) and primary cutaneous T-cell lymphoma (33%) were the most diagnosed peripheral T-cell lymphoma subtypes, followed by the nasal type natural killer/T-cell lymphoma (27%) and peripheral T-cell lymphoma not otherwise specified (7%). The most prevalent stage of disease progression was stage I and stage IV (Ann Arbor classification) with seven (47%) patients each. Eight (53%) patients succumbed to the disease process of which three (37.5%) deceased in the first six months and six (75%) within the first year of diagnosis. We identified a strong statistical association between stage and disease-related death ( p = 0.003). CONCLUSIONS: Peripheral T-cell lymphoma occurring in the orbit and ocular adnexa is extremely rare. Advanced stage at diagnosis leads to almost certain death from the disease despite aggressive local and systemic treatment. Early diagnosis improves the chances of survival but can be hindered by this condition's ability to simulate benign inflammatory conditions both clinically and histologically.

Incidence and Risk Factors for Poor Postoperative Visual Outcome After Excision of Orbital Cavernous Venous Malformations.

AIMS: To determine the incidence and risk factors for poor postoperative visual outcome (PPVO) after removal of orbital cavernous venous malformations (OCVMs). METHODS: Retrospective case-note and imaging review for patients undergoing excision of OCVMs, with estimation of odds-ratios (ORs) and relative risks (RRs) for visual loss in relation to the position of the mass, surgical approach, and patient factors. RESULTS: The 290 patients (179 female; 62%) presented at a mean age of 46.4 years: 243/287 (85%) OCVMs were intraconal, with 213/243 (88%) located freely in the posterior two-thirds of the orbit, and 30/243 (12%) wedged tightly in the apex. PPVO was observed in 6.9% (20/290) patients, solely after removal of intraconal lesions, Univariate analysis showed increased risk with preoperative relative afferent pupillary defect (RAPD) (14/107 [13%]; RR 2.9; P = 0.011), apical lesions (9/30 [30%]; RR 5.8; P < 0.001), situated below optic nerve (15/115 [13%]; RR 3.3; P = 0.007), fibrous masses (14/78 [18%]; RR 6.7; P = 0.005), or intraoperative diastolic blood pressure below 50mmHg (10/64 [16%]; RR 2.8; P = 0.007). Multivariate analysis found apical extension (OR 4.9; P = 0.036) and fibrous lesions (OR 10.0; P = 0.035) as strongest predictors for PPVO. The incidence of complete visual loss (no light perception) was 4.1% (12/290); half of these patients had preoperative acuity of counting fingers or worse, 8 (67%) had RAPD, 7 (58%) wedged apical lesions, and 8 (67%) were below the optic nerve. CONCLUSION: PPVO after excision of OCVMs can occur in up to 5% of "free" retrobulbar intraconal lesions and in approximately one-third of apical lesions.