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Ophthalmic Plastic and Reconstructive...Conventional modalities of external-beam radiation therapy (EBRT) are associated with high incidences of severe vision-threatening ocular and orbital toxicities when...
PURPOSE
Conventional modalities of external-beam radiation therapy (EBRT) are associated with high incidences of severe vision-threatening ocular and orbital toxicities when used to treat orbital malignancies. We investigate toxicities associated with high-dose volumetric modulated arc therapy (VMAT), a commonly used contemporary treatment modality for these tumors.
METHODS
Retrospective analysis of malignant orbital tumors managed with adjuvant high-dose VMAT preceded by globe-salvaging surgical therapy (GST) or exenteration. Dosimetric quantitation of target volumes and critical structures was performed. Incidence and severity of ocular, orbital, and adnexal toxicities were evaluated and assessed with regard to conventional EBRT toxicities for orbital malignancies described in the literature.
RESULTS
Eighty-four subjects (mean age = 65.9 ± 9.7 years) were included (N = 48 and N = 36 in GST and exenteration subgroups, respectively). Mean dose was 64.8 ± 2.1 Gy to the planning target volume. Dosing to critical structures typically did not surpass known tissue tolerance limits. Median follow up was 18.3 months. Visual acuity in the GST subgroup was not significantly different after VMAT (0.25 ± 0.06) compared with baseline (0.23 ± 0.02; P = 0.302). Whereas severe toxicities reported by major systematic analyses in the literature with older EBRT modalities were relatively common-for example, retinopathy (16-40%), optic neuropathy (16%), and corneal perforation (13%)-toxicities with VMAT were typically mild and less common. The most common toxicities with VMAT were mild dry eye (81.3%; 39/48), cataract (21.1%; 8/38 phakic eyes), and periocular dermatitis (15.5%; 13/84). Vision-threatening toxicities, including severe corneal pathologies, retinopathy, or optic neuropathy, were rare. There were no contralateral ocular or adnexal toxicities.
CONCLUSIONS
High-dose VMAT for orbital malignancies demonstrated low incidence and severity of eye-related toxicity, in contradistinction to adverse events reported from conventional forms of radiotherapy.
Topics: Aged; Humans; Middle Aged; Optic Nerve Diseases; Orbital Neoplasms; Radiotherapy Dosage; Radiotherapy, Intensity-Modulated; Retinal Diseases; Retrospective Studies
PubMed: 34284423
DOI: 10.1097/IOP.0000000000002001 -
International Ophthalmology Jun 2023Orbital lymphoma is one of the most common adult orbital malignancies, accounting for approximately 10% of all orbital tumors. This study aimed to analyze the effects of...
OBJECTIVES
Orbital lymphoma is one of the most common adult orbital malignancies, accounting for approximately 10% of all orbital tumors. This study aimed to analyze the effects of surgical resection and orbital iodine-125 brachytherapy implantation for orbital lymphoma.
PATIENTS AND METHODS
This was a retrospective study. Clinical data of 10 patients were collected from October 2016 to November 2018 and followed up to March 2022. Patients underwent the primary surgery for maximal safe removal of the tumor. After a pathologic diagnosis of a primary orbital lymphoma was established, iodine-125 seed tubes were designed based on the tumor size and invasion range, and direct vision was placed into the nasolacrimal canal or/and under the orbital periosteum around the resection cavity during the secondary surgery. Then, follow-up data, including the general situation, ocular condition, and tumor recurrence, were recorded.
RESULTS
Of the 10 patients, the pathologic diagnoses included extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (6 cases), small lymphocytic lymphoma (1 case), mantle cell lymphoma (2 cases), and diffuse large B-cell lymphoma (1 case). The number of seeds implanted ranged from 16 to 40. The follow-up period ranged between 40 and 65 months. All patients in this study were alive and well had tumors that were completely controlled. No tumor recurrences or metastases occurred. Three patients had dry eye syndrome and two patients had abnormal facial sensation. No patient had radiodermatitis involving the skin around the eye, and no patient had radiation-related ophthalmopathy.
CONCLUSIONS
Based on preliminary observations, iodine-125 brachytherapy implantation appeared to be a reasonable alternative to external irradiation for orbital lymphoma.
Topics: Adult; Humans; Orbital Neoplasms; Brachytherapy; Retrospective Studies; Neoplasm Recurrence, Local; Lymphoma, B-Cell, Marginal Zone
PubMed: 36906873
DOI: 10.1007/s10792-022-02594-x -
Radiographics : a Review Publication of... 2022Pediatric orbital masses are not common but encompass a wide spectrum of benign and malignant entities that range from developmental anomalies to primary and secondary...
Pediatric orbital masses are not common but encompass a wide spectrum of benign and malignant entities that range from developmental anomalies to primary and secondary orbital malignancies and metastatic disease. Certain orbital tumors are unique to pediatric patients, such as retinoblastoma and neuroblastoma. Clinical symptoms and signs are often insufficient to differentiate between orbital lesions, and imaging is essential for narrowing the diagnostic considerations and determining the most appropriate management strategy. MRI is the primary imaging modality for evaluating orbital masses in children, with US and CT playing complementary roles. The authors review a spectrum of masses and tumor mimics that affect the pediatric globe and orbit. The shared and differentiating characteristics of pediatric orbital lesions are reviewed. Emphasis is placed on utilizing an orbital compartment-based approach to narrow the differential diagnosis. By using this organizational scheme, the authors describe intraocular processes (retinoblastoma, persistent fetal vasculature, and Coats disease), intraconal lesions (lymphatic malformation, schwannoma, optic nerve sheath meningioma, and optic pathway glioma), extraconal lesions (infantile hemangioma, rhabdomyosarcoma, idiopathic orbital inflammation, lymphoma, venous varix, plexiform neurofibroma, and pleomorphic adenoma of the lacrimal gland), and lesions involving the bony orbit (dermoid cyst, metastatic neuroblastoma, and Langerhans cell histiocytosis). The authors describe the basic management of each entity. Orbital infections and traumatic lesions are beyond the scope of this article. RSNA, 2022.
Topics: Child; Humans; Magnetic Resonance Imaging; Meningeal Neoplasms; Neoplasms, Second Primary; Neuroblastoma; Orbital Neoplasms; Retinal Neoplasms; Retinoblastoma
PubMed: 35245105
DOI: 10.1148/rg.210116 -
Orbit (Amsterdam, Netherlands) Jun 2022To report the clinical characteristics and intraoperative findings of periorbital and orbital dermoid cysts and their relationship to location and rupture.
PURPOSE
To report the clinical characteristics and intraoperative findings of periorbital and orbital dermoid cysts and their relationship to location and rupture.
METHODS
Retrospective review of 270 cases with orbital or periorbital dermoid cysts that presented over a period of 11 years. Patients were included if diagnosis of dermoid cyst was made by histopathologic analysis. Clinical characteristics and operative outcomes were recorded and analyzed with Chi-squared analyses or univariate regression. Multivariate binary logistic regression was performed to assess predictors of location and rupture.
RESULTS
Dermoids frequently occurred unilaterally and were more frequently described as mobile (61.5%), followed by fixed (30.8%), and partially fixed (7.7%). Dermoid rigidity was most commonly described as firm (75.5%), followed by cystic, soft, and rubbery. The most common dermoid location was superotemporal (60.4%). On Chi-squared analysis, superotemporal lesions were less likely to have orbital extension than non-superotemporal lesions (OR 0.28, 95% CI: 0.11-0.70, = .01), less likely to undergo CT (OR 0.16, 95% CI: 0.06-0.41, < .01) or MRI (OR 0.23, 95% CI: 0.13-0.41, < .01), more likely to be described as mobile (OR 2.91, 95% CI: 1.32-6.43, = .01), and less likely to rupture intraoperatively (OR 0.28, 95% CI: 0.11-0.73, = .01). No variables were associated with rupture in multivariate analysis.
CONCLUSION
Superotemporal dermoid cysts are common in the pediatric population, less likely to have orbital extension, undergo imaging, and have intraoperative rupture when compared to other locations in the orbit.
Topics: Child; Dermoid Cyst; Humans; Magnetic Resonance Imaging; Orbital Diseases; Orbital Neoplasms; Retrospective Studies
PubMed: 35549983
DOI: 10.1080/01676830.2021.1912114 -
European Journal of Ophthalmology Sep 2021To compare differences in clinical presentation and management outcomes of orbital and periorbital dermoid cysts in children and adults.
PURPOSE
To compare differences in clinical presentation and management outcomes of orbital and periorbital dermoid cysts in children and adults.
METHODS
A retrospective interventional comparative consecutive case series. 203 patients in (148 children [Age ⩽18 years] and 55 adults [Age >18 years]), who underwent excision biopsy for orbital/periorbital dermoid cyst were included. Outcome measures included the differences in demography, clinical presentations, radiological features, surgical outcomes and histopathology of cysts between children and adults with dermoid cysts.
RESULTS
The mean age noted at excision was 15 years in this cohort of 148 children and 55 adults. Commonest clinical finding observed was a subcutaneous painless mass (94%) and external angular dermoids were commonest in both groups 1 and 2 (51% and 56%). Diminution of vision ( = 0.007), proptosis ( = 0.008), extraocular motility limitation ( = 0.001), strabismus ( = 0.003) and relative afferent pupillary defect ( = 0.02) were significantly more in adults. Orbital involvement and dumbbell dermoids were not significantly different between children and adults. Radiologically, bony fossa formation ( = 0.03), temporal fossa extension ( = 0.04), full thickness bony defect ( = 0.03) and intraosseous presentation ( = 0.005) were significantly more in adults. Comparison of proportion of lesions having intra-operative rupture of dermoid cyst ( = 0.009) and evidence of inflammation on histopathology ( = 0.01) were significantly more in adults. However, recurrence rates were not different between children and adults.
CONCLUSIONS
Dermoid cysts are commoner in children, but can also present in adulthood. Secondary visual, orbital, radiologic changes and intra-operative rupture of dermoid cyst are more common in adults compared to children.
Topics: Adolescent; Adult; Child; Dermoid Cyst; Humans; Orbital Diseases; Orbital Neoplasms; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 33198489
DOI: 10.1177/1120672120964686 -
Eye (London, England) Apr 2023To describe a series of paediatric orbital lymphoma patients in a single tertiary referral centre. (Review)
Review
OBJECTIVE
To describe a series of paediatric orbital lymphoma patients in a single tertiary referral centre.
METHODS
A retrospective case-note search in the Oxford Eye Hospital of all patients under the age of 18 years with orbital lymphoma between 2010 and 2020. Demographic and clinical data were obtained, and a literature review was conducted.
RESULTS
Five patients were identified with orbital lymphoma, mean age 48.2 ± 36 months (1-109 months), three were males. Clinical presentation included: ptosis, proptosis, lethargy, visual loss, and strabismus. Two patients had bilateral orbital disease and one patient was diagnosed within the first month of life. The tissue diagnosis revealed four cases of Burkitt's lymphoma and one case of T- lymphoblastic lymphoma. Central nervous system (CNS) sampling was also positive in the four cases of Burkitt's lymphoma. All patients were treated systemically for the lymphoma with chemotherapy. Complete remission was achieved in all cases post chemotherapy. Follow-up of 36.4 ± 18.9 months (10-61 months).
CONCLUSION
This is the largest published case series of paediatric orbital lymphoma. We described a patient diagnosed within the first month of life and we believe this to have developed intra-uterine. In this series, patients were younger, had more bilateral disease and had better outcome than previously described. This rare condition should be considered in any child with an orbital mass, at any age. When managed appropriately, good outcomes can be achieved.
Topics: Male; Child; Humans; Infant; Child, Preschool; Adolescent; Female; Burkitt Lymphoma; Retrospective Studies; Lymphoma; Orbital Neoplasms
PubMed: 36171296
DOI: 10.1038/s41433-022-02266-1 -
American Journal of Ophthalmology Feb 2022To evaluate the frequency and clinical course of residual orbital masses on imaging studies after multimodality treatment for orbital rhabdomyosarcoma.
PURPOSE
To evaluate the frequency and clinical course of residual orbital masses on imaging studies after multimodality treatment for orbital rhabdomyosarcoma.
DESIGN
Retrospective case series.
METHODS
We reviewed records of patients with primary orbital rhabdomyosarcoma who underwent chemotherapy and radiotherapy after surgical biopsy or debulking at 4 US centers during 1998-2019. Demographics, histologic subtype, tumor response 12 weeks after chemotherapy initiation and after completion of all treatment, and imaging findings were analyzed.
RESULTS
Thirty-two patients met inclusion criteria. Twenty-two were male, and 30 were younger than 18 years. Histologic subtype was embryonal in 22 patients, alveolar in 8, and mixed embryonal/alveolar in 2. Median follow-up time was 46 months (range, 4.9-199 months). Two patients died. Twenty-seven patients had reliable end-of-treatment imaging findings, of whom 9 had a residual mass. Three residual masses disappeared spontaneously (by 4, 32, and 53 months), 2 remained at last contact, at 2 and 7 years of follow-up, and 3 were excised; 1 progressed and underwent an exenteration. Complete response at 12 weeks was associated with complete response at the end of treatment (P < .001). Patients with T1 or T2 tumor at presentation were more likely to have complete response at last contact than were those with T3 or T4 tumor (P < .05). Biopsy type (incisional or excisional) was not associated with response to treatment at any time point.
CONCLUSION
A residual orbital mass on imaging may be present after multimodality treatment in approximately one-third of patients. Resolution without biopsy or excision varied from months to years.
Topics: Combined Modality Therapy; Humans; Male; Orbital Neoplasms; Remission Induction; Retrospective Studies; Rhabdomyosarcoma
PubMed: 34280364
DOI: 10.1016/j.ajo.2021.06.035 -
The Journal of Craniofacial SurgeryTo report the clinical features, treatment, and outcome of 11 patients with orbital schwannoma in Korean patients.
PURPOSE
To report the clinical features, treatment, and outcome of 11 patients with orbital schwannoma in Korean patients.
METHODS
The medical records of 11 orbital schwannoma patients treated between April 2007 and April 2021 were retrospectively reviewed. The demographic data, clinical characteristics, radiological features, and outcomes were reviewed.
RESULTS
The mean age at the time of diagnosis was 49.00 ± 14.45 years. The most common initial symptom was ocular protrusion (n = 7), and other symptoms were decreased visual acuity ( n = 5), restriction of eye movement ( n = 4), swelling ( n = 3), and pain ( n = 2). Locations of tumors were superomedial; followed by the orbital apex and inferolateral. The most common shape seen in our patients was beads like multilobulated appearance; followed by a round, oval, fusiform, and dumbbell shape. MRI of T1-weighted revealed isointense or hypointense, whereas the T2-weighted indicated hyper or isointense lesion. Five patients had optic neuropathy at presentation, and 1 of them showed improved vision after surgery. Complete or incomplete excision was performed for all. Surgical complications include decreased vision and paraesthesia. There has been no recurrence to date.
CONCLUSIONS
Orbital schwannoma is a rare disease and it is difficult to distinguish it from other orbital tumors because the initial symptoms are nonspecific. Differential diagnosis by combining the shape, location, and contrast enhancement findings seen on computed tomography and magnetic resonance imaging can be helpful in surgical treatment. Complete excision gives the best results without recurrence, but if the patient is in an inaccessible location, only surgery to reduce the volume can satisfy the patient without recurrence.
Topics: Humans; Adult; Middle Aged; Retrospective Studies; Orbital Neoplasms; Eye Neoplasms; Magnetic Resonance Imaging; Neurilemmoma
PubMed: 35261368
DOI: 10.1097/SCS.0000000000008573 -
Ophthalmic Plastic and Reconstructive...To study the multidisciplinary management and survival outcomes of orbital metastasis (OM).
PURPOSE
To study the multidisciplinary management and survival outcomes of orbital metastasis (OM).
METHODS
All patients with a diagnosis of OM treated during 1999-2019 were included. Clinical data were retrospectively collected and analyzed.
RESULTS
The study included 118 patients, 71 females and 47 males, with a median age of 61 years. The most common primary tumor types were breast carcinoma (43 patients), melanoma (17), and lung (13), thyroid (7), renal cell (6), and neuroendocrine carcinoma (6). Ninety-six patients had a known history of cancer at OM diagnosis. The median time from diagnosis of primary cancer to OM was 31 months (range, 0-304). In 22 patients, OM was the first sign of cancer. In 47 patients, the orbit was the only site of metastasis. The most common presenting features were restricted by extraocular motility (77 patients) and proptosis (61). Eight patients had enophthalmos. OM was diagnosed based on clinical history and imaging studies in 81 patients and orbital biopsy in 37. One hundred nine patients were treated with chemotherapy and immunotherapy, 75 with radiation, and 21 with palliative surgical resection. Eighty-two patients died during follow up. The median overall survival (OS) time after diagnosis of OM was 17 months (95% CI: 12-28). OM from renal cell carcinoma was associated with the best and OM from thyroid cancer with the worst OS. Patients with breast cancer had longer median survival (28 months; 95% CI: 15-60) than patients with lung, melanoma, neuroendocrine, or thyroid cancer.
CONCLUSION
In this large series, breast cancer and melanoma were the most common causes of OM. Most patients had a known history of cancer at OM diagnosis and did not require orbital biopsy to confirm the diagnosis. Patients with renal cell carcinoma and breast carcinoma had the best prognosis after diagnosis of OM.
Topics: Breast Neoplasms; Female; Humans; Male; Melanoma; Middle Aged; Orbital Neoplasms; Prognosis; Retrospective Studies
PubMed: 33587417
DOI: 10.1097/IOP.0000000000001939 -
Orbital diseases mimicking graves' orbitopathy: a long-standing challenge in differential diagnosis.Journal of Endocrinological... Apr 2020Graves' orbitopathy (GO) is the most common cause of orbital tissue inflammation, accounting for ~ 60% of all orbital inflammatory conditions in the population aged... (Review)
Review
Graves' orbitopathy (GO) is the most common cause of orbital tissue inflammation, accounting for ~ 60% of all orbital inflammatory conditions in the population aged 21-60 years, and for ~ 40% in the population aged > 60 year. GO is observed in 25-30% of patients with Graves' hyperthyroidism and more rarely in association with hypothyroid autoimmune thyroiditis. In addition, a small proportion of GO patients (1-2%) do not have a clinically overt thyroid dysfunction. Clinically, GO is characterized by proptosis, inflammation involving the eyelids and the conjunctiva, extraocular muscle hypertrophy, with consequent reduction of ocular motility and diplopia, and in the most severe cases, compression of the optic nerves at the orbital apex, with reduction of visual acuity. At CT scan or MRI, a muscle increase involving the superior, medial and inferior rectus is quite typical. In the most severe forms, compression of the optic nerves at the orbital apex can be observed. Euthyroid GO is usually an early sign of a full-blown Graves' disease; however, in some cases, the orbital disease can remain isolated. Moreover, euthyroid GO can rarely be unilateral, which makes the picture even more confusing. Under those circumstances, the diagnostic process becomes obviously quite difficult, having other conditions mimicking GO been excluded. A number of inflammatory conditions affecting orbital tissue can mimic GO, thereby requiring an accurate evaluation for a proper differential diagnosis. The majority of these conditions are immune mediated. Most of them are benign, but they can be rather aggressive and some can cause visual loss. The most common inflammatory condition affecting orbital tissues and mimicking GO is idiopathic orbital inflammation. Other, more rare, orbital diseases that should be considered in the differential diagnosis are infections, orbital manifestations of systemic diseases, primitive and secondary orbital neoplasms, and orbital vascular alterations. In most instances, when an orbitopathy occurs in the absence of hyperthyroidism, the diagnosis of the disease underlying the ocular symptoms and signs is based on exclusion of the other conditions. Here we review the conditions that can mimic GO and how to distinguish them from this obnoxious eye disease.
Topics: Diagnosis, Differential; Graves Ophthalmopathy; Humans; Inflammation; Lymphoma; Magnetic Resonance Imaging; Orbital Diseases; Orbital Neoplasms; Tomography, X-Ray Computed
PubMed: 31691261
DOI: 10.1007/s40618-019-01141-3