Interventions for orbital lymphangioma Patel, Sheel R; Rosenberg, Jamie B; Barmettler, Anne ...
Cochrane database of systematic reviews,
05/2019, Letnik:
2019, Številka:
5
Journal Article
Recenzirano
Odprti dostop
Background
Orbital lymphangiomas are a subset of localized vascular and lymphatic malformations, which most commonly occur in the head and neck region. Orbital lymphangiomas typically present in the ...first decade of life with signs of ptosis, proptosis, restriction of ocular motility, compressive optic neuropathy, and disfigurement. Therefore, early and effective treatment is crucial to preserving vision. Due to proximity to vital structures, such as the globe, optic nerve, and extraocular muscles, treatment for these lesions is complicated and includes a large array of approaches including observation, sclerotherapy, systemic therapy, and surgical excision. Of these options, there is no clear gold standard of treatment.
Objectives
To assess the evidence supporting medical and surgical interventions for the reduction/treatment of orbital lymphangiomas in children and young adults.
Search methods
We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (which contains the Cochrane Eyes and Vision Trials Register) (2018, Issue 5); Ovid MEDLINE; Embase.com; PubMed; Latin American and Caribbean Health Sciences Literature Database (LILACS); ClinicalTrials.gov and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP). We did not use any date or language restrictions in the electronic search for trials. We last searched the electronic databases on 22 May 2018.
Selection criteria
We planned to include randomized controlled trials (RCTs) comparing at least two of the following interventions with each other for the treatment of orbital lymphangiomas: observation; sildenafil therapy; sirolimus therapy; sclerotherapy; surgery (partial or complete resection). We planned to include trials that enrolled children and adults up to 32 years of age, based on a prior clinical trial protocol. There were no restrictions regarding location or demographic factors.
Data collection and analysis
Two review authors independently screened the titles, s, and full articles to assess their suitability for inclusion in this review. No risk of bias or data extraction was performed because we did not find any trials for inclusion. If there had been RCTs, two authors would have assessed the risk of bias and ed data independently with discrepancies being settled by consensus or consultation with a third review author.
Main results
There were no RCTs that compared any two of the mentioned interventions (medical or surgical) for treating orbital lymphangiomas in children and young adults.
Authors' conclusions
Currently, there are no published RCTs of orbital lymphangioma treatments. Without these types of studies, conclusions cannot be drawn regarding the effectiveness of the medical and surgical treatment options for patients with orbital lymphangiomas. The presence of only case reports and case series on orbital lymphangiomas makes it clear that RCTs are needed to address the differences between these options and help guide treatment plans. Such trials would ideally compare outcomes between individuals randomized to one of the following treatment options: observation, sclerotherapy, systemic sirolimus therapy, systemic sildenafil therapy, and surgical excision.
Metastases of thyroid carcinomas to the choroid and/or orbit are infrequent. PubMed was searched for English-language articles and case reports published from 1977 to 2012. In our survey, we looked ...for reports of orbital and/or ocular metastases from the thyroid and found 31 reported cases from 1979 to 2012.
At the time of onset of ocular symptoms, the vast majority of patients had a long history of thyroid malignancy and evidence of widely disseminated metastatic disease. The age of the reported patients ranged from 29 to 83 years. Among the 22 reported cases of thyroid carcinomas with metastases to the choroid from 1979 to 2012, the most common primary tumor was papillary thyroid carcinoma (PTC); this occurred in seven patients. This was followed by medullary thyroid carcinoma in six cases and follicular thyroid carcinoma (FTC) in five cases. Orbital metastases were reported in nine patients with thyroid carcinomas (PTC=4, FTC=3, Hürthle cell=1, not specified=1). Patients with choroidal metastases presented with decreased or blurred vision, eye pain, and flashes in 81%, 5%, and 5% of cases, respectively. The diagnosis of a choroidal tumor was usually based on noninvasive diagnostic techniques such as ultrasonography, transillumination, computer tomography (CT), and/or magnetic resonance imaging (MRI) scanning. ¹³¹I scanning revealed uptake in the orbit in 26% of cases with choroidal and/or orbital PTC or FTC. For a metastasis that causes a definitive loss of vision and/or persistent pain, the treatment of choice was enucleation. The other treatment options were brachyradiotherapy using ¹²⁵I episcleral radioactive plaque insertion, external beam radiation, ¹³¹I therapy, chemotherapy, and/or targeted therapy with small molecules.
The orbit and globe are not common sites for metastatic thyroid carcinomas. Diagnosis of a choroidal tumor is usually based on clinical judgment and results of noninvasive diagnostic techniques such as ultrasonography, transillumination, CT, and/or MRI scanning. Fundoscopic examination and ocular ultrasonography by an ophthalmologist are recommended for identification and monitoring of choroidal metastatic deposits.
The transnasal endoscopic approach may provide better visualisation and a safer approach to the orbital apex. This study presents a case series of orbital apex lesions managed by this approach.
This ...study was an eight-year retrospective analysis of seven patients who were operated on for orbital apex lesions in two tertiary medical centres.
Complete tumour removal was performed in three patients and partial removal was performed in four patients. Visual acuity improved in three patients, remained stable in one patient and decreased in the other two patients. The visual field improved in four patients and did not change in two patients. Complications included worse vision and visual fields in 28.6 per cent of patients and late enophthalmos (of -1.25 ± 4.6 mm) in 2 patients.
The transnasal approach to orbital apex lesions in selected cases may provide a rational alternative to transorbital surgery. Complete tumour removal should be weighed against the risk of damage to the optic nerve.
This study aimed to analyze the role of the FcepsilonRI (FcεRI) signaling pathway in the pathogenesis of benign lymphoepithelial lesion of lacrimal gland (LGBLEL). Transcriptomic analysis was ...performed on LGBLEL and orbital cavernous hemangioma (CH) patients diagnosed via histopathology in Beijing Tongren Hospital, Capital Medical University, between July 2010 and October 2013. Four LGBLEL and three orbital CH patients, diagnosed between October 2018 and August 2019, were randomly selected as experimental and control groups, respectively. RT-PCR, immunohistochemical staining, and western blotting were used to verify genes and proteins related to the FcεRI signaling pathway. Transcriptomic analysis showed that the FcεRI signaling pathway was upregulated in the LGBLEL compared with the CH group. The mRNA expression levels of important genes including SYK, p38, JNK, PI3K, and ERK were significantly increased in the LGBLEL group (P = 0.0066, P = 0.0002, P = 0.0003, P < 0.0001, P < 0.0001, respectively). Immunohistochemical staining results showed that SYK, p38, and ERK were positively expressed in LGBLEL, while JNK and PI3K were not. The protein contents of P-SYK, P-p38, P-JNK, P-PI3K, and P-ERK were significantly higher in the LGBLEL than in the CH group (P = 0.0169, P = 0.0074, P = 0.0046, P = 0.0157, P = 0.0156, respectively). The FcεRI signaling pathway participates in the pathogenesis of LGBLEL.
Reconstruction of large cavities in the skull and facial regions is important not only to restore health but also for the correction of facial distortions. Every visible deformity in the facial ...region of the patient affects their mental wellness and perception by society, entailing both, deterioration of health, but also a decrease in the performance in society, which translates into its productivity. With the progressive degradation of the natural environment, cancer, in the coming years, will be on the leading causes of morbidity and mortality. The review focuses on two main aspects: (i) the causes of injuries leading to the necessity of removal of orbital cavities occupied by the tumor and then their reconstruction, with the focus on the anatomical structure of the orbital cavity, (ii) the materials used to reconstruct the orbital cavities and analyze their advantages and disadvantages. The manuscript also underlines the not yet fully met challenges in the area of facial- and craniofacial reconstruction in people affected by cancer.
Orbital peripheral nerve sheath tumors Sweeney, Adam R., MD; Gupta, Divakar, MD; Keene, C. Dirk, MD, PhD ...
Survey of ophthalmology,
01/2017, Letnik:
62, Številka:
1
Journal Article
Recenzirano
Abstract Peripheral nerve sheath tumors of the orbit and ocular adnexa are a rare group of neoplasms hallmarked by nonspecific clinical presentations, variable tumor locations, challenging ...therapeutic efforts, and occasional diagnostic dilemmas. We review these tumor types and provide an updated summary on their clinical, histopathologic, radiological, and emerging molecular features.
To analyze the effect of reduced-dose radiotherapy on local control in children with low-risk rhabdomyosarcoma (RMS) treated in the Children's Oncology Group D9602 study.
Patients with low-risk RMS ...were nonrandomly assigned to receive radiotherapy doses dependent on the completeness of surgical resection of the primary tumor (clinical group) and the presence of involved regional lymph nodes. After resection, most patients with microscopic residual and uninvolved nodes received 36 Gy, those with involved nodes received 41.4 to 50.4 Gy, and those with orbital primary tumors received 45 Gy. All patients received vincristine and dactinomycin, with cyclophosphamide added for patient subsets with a higher risk of relapse in Intergroup Rhabdomyosarcoma Study Group III and IV studies.
Three hundred forty-two patients were eligible for analysis; 172 received radiotherapy as part of their treatment. The cumulative incidence of local/regional failure was 15% in patients with microscopic involved margins when cyclophosphamide was not part of the treatment regimen and 0% when cyclophosphamide was included. The cumulative incidence of local/regional failure was 14% in patients with orbital tumors. Protocol-specified omission of radiotherapy in girls with Group IIA vaginal tumors (n = 5) resulted in three failures for this group.
In comparison with Intergroup Rhabdomyosarcoma Study Group III and IV results, reduced-dose radiotherapy does not compromise local control for patients with microscopic tumor after surgical resection or with orbital primary tumors when cyclophosphamide is added to the treatment program. Girls with unresected nonbladder genitourinary tumors require radiotherapy for postsurgical residual tumor for optimal local control to be achieved.
BACKGROUND: In the literature, central serous retinopathy (CSR) accompanying solitary fibrous tumors (SFT) in a pilot has not been reported. In airline pilots, mass effect-related symptoms such as ...diplopia, ptosis, etc., seen with orbital tumors may endanger flight safety.CASE
REPORT: A 62-yr-old male commercial airline pilot presented with blurred vision in the right eye. He had been receiving treatment for 2 mo because of CSR. His visual acuity was 10/20 in the right eye and 20/20 in the left. During examination, ptosis and exophthalmos were noticed
in the right eye. Ocular movements were free in all cardinal directions and there was downward displacement in the right eye. There was no diplopia. Magnetic resonance imaging revealed a 1.5- to 2-cm well-defined contrast-enhancing mass in the lateral extraconal orbit. His medical flight certificate
was suspended for 3 mo due to decreased visual acuity and superior visual defect. Superior orbitotomy was performed without any complication. Ptosis and CSR had regressed 1 wk after surgery. All systemic and ophthalmological examinations met aviation medical certificate requirements.
He returned to flight on the condition of being checked every 3 mo. At the 1-yr follow-up, there was no sign of recurrences of SFT or CSR.DISCUSSION: SFTs are slow-growing neoplasms that can manifest symptoms related to mass effect. In the current literature, there are no
reported cases of the coexistence of orbital SFT and CSR or pilots able to resume flight duties only 1 wk after a successful orbitotomy and tumor resection surgery.Altinbas M, Ozpınar A, Akbaba M, Nacaroglu SA, Sargolzaeimoghaddam M, Sargolzaeimoghaddam M. Orbital solitary
fibrous tumor in a commercial airline pilot. Aerosp Med Hum Perform. 2024; 95(6):333-336.
Postoperative radiation is often indicated in the treatment of malignant epithelial tumors of the orbit and ocular adnexa. We present details of radiation technique and toxicity data after ...orbit-sparing surgery followed by adjuvant proton radiation therapy.
Twenty patients underwent orbit-sparing surgery followed by proton therapy for newly diagnosed malignant epithelial tumors of the lacrimal gland (n=7), lacrimal sac/nasolacrimal duct (n=10), or eyelid (n=3). Tumor characteristics, treatment details, and visual outcomes were obtained from medical records. Acute and chronic toxicity were prospectively scored using Common Terminology Criteria for Adverse Events version 4.0.
The median radiation dose was 60 Gy(RBE) (relative biological effectiveness; range 50-70 Gy); 11 patients received concurrent chemotherapy. Dose to ipsilateral anterior optic structures was reduced in 13 patients by having them gaze away from the target during treatment. At a median follow-up time of 27.1 months (range 2.6-77.2 months), no patient had experienced local recurrence; 1 had regional and 1 had distant recurrence. Three patients developed chronic grade 3 epiphora, and 3 developed grade 3 exposure keratopathy. Four patients experienced a decrease in visual acuity from baseline but maintained vision sufficient to perform all activities of daily living without difficulty. Patients with grade ≥3 chronic ocular toxicity had higher maximum dose to the ipsilateral cornea (median 46.3 GyRBE, range 36.6-52.7 GyRBE vs median 37.4 GyRBE, range 9.0-47.3 Gy(RBE); P=.017).
Orbit-sparing surgery for epithelial tumors of the orbit and ocular adnexa followed by proton therapy successfully achieved disease control and was well tolerated. No patient required orbital exenteration or enucleation. Chronic grade 3 toxicity was associated with high maximum dose to the cornea. An eye-deviation technique can be used to limit the maximum corneal dose to <35 Gy(RBE).
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