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Indian Journal of Ophthalmology Feb 2022Dermoid cyst, a developmental benign choristoma, is the most common orbital tumor of childhood, arising from ectodermal sequestration along the lines of embryonic fusion...
BACKGROUND
Dermoid cyst, a developmental benign choristoma, is the most common orbital tumor of childhood, arising from ectodermal sequestration along the lines of embryonic fusion of mesodermal processes, lined by keratinized stratified squamous epithelium and expanding slowly due to constant desquamation and dermal glandular elements. Approximately 80% are found in the head and neck region and comprise 3-9% all orbital masses.
PURPOSE
It is mandatory to know about the variable presentations of orbital dermoids and the surgical techniques that can be adopted based on the site, extent, age and aesthetic needs, presence of inflammation and possibility of intraoperative rupture.
SYNOPSIS
Orbital dermoids can be classified as juxta-sutural, sutural or soft tissue cysts; superficial or deep; intraosseous or extraosseous, and intraorbital or extraorbital. These smooth, painless, mobile or partially mobile lesions mostly present at the fronto-zygomatic suture with proptosis, displacement, ptosis or diplopia, depending on depth and extent. Therefore, it is important to understand the various presentations and the appropriate surgical techniques.
HIGHLIGHTS
We describe the embryological origin, types and clinical features of dermoids in this video and demonstrate the surgical and minimally invasive techniques for their management.
VIDEO LINK
https://youtu.be/-q3xD2igjcQ.
Topics: Blepharoptosis; Choristoma; Dermoid Cyst; Humans; Orbital Diseases; Orbital Neoplasms
PubMed: 35086291
DOI: 10.4103/ijo.IJO_145_22 -
Klinische Monatsblatter Fur... Sep 2023Malignant masses of the orbit include a large variety of neoplasms of epithelial or mesenchymal origin. The treatment of orbital malignancies is an essential...
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.
Topics: Humans; Orbit; Orbital Neoplasms; Magnetic Resonance Imaging; Orbital Diseases; Exophthalmos
PubMed: 37586398
DOI: 10.1055/a-2129-1194 -
Survey of Ophthalmology 2019Orbital lymphomas constitute 50-60% of ocular adnexal lymphomas. A total of 2211 cases of orbital lymphoma with a known subtype have been reported in the last 24 years... (Review)
Review
Orbital lymphomas constitute 50-60% of ocular adnexal lymphomas. A total of 2211 cases of orbital lymphoma with a known subtype have been reported in the last 24 years (1994-2017). The vast majority of orbital lymphomas are of B-cell origin (97%), of which extranodal marginal zone B-cell lymphoma (EMZL) (59%) is the most common subtype, followed by diffuse large B-cell lymphoma (23%), follicular lymphoma (9%), and mantle cell lymphoma (5%). Orbital lymphoma is primarily a disease of the elderly. Gender distribution varies according to lymphoma subtype. However, extranodal marginal zone B-cell lymphoma (53%) and follicular lymphoma (75%) show a female predominance, whereas diffuse large B-cell lymphoma shows an even gender distribution. Mantle cell lymphoma has a striking male predominance of 80%. The histopathological subtype and the clinical stage of the disease are the best indicators of prognosis and patient outcome. Low-grade lymphomas such as extranodal marginal zone B-cell lymphoma and FL have a good prognosis, whereas high-grade lymphomas (diffuse large B-cell lymphoma and mantle cell lymphoma) are associated with a poor prognosis. When managing solitary low-grade lymphomas, radiotherapy is the treatment of choice. Chemotherapy, with or without radiotherapy, should be chosen for disseminated and high-grade lymphomas.
Topics: Combined Modality Therapy; Global Health; Humans; Incidence; Lymphoma; Orbit; Orbital Neoplasms; Survival Rate
PubMed: 30144455
DOI: 10.1016/j.survophthal.2018.08.002 -
Harefuah Feb 2023Orbital dermoid cysts are benign choristomas that arise from the entrapment of ectodermal elements adjacent to the fetal bony suture lines. They are considered... (Review)
Review
Orbital dermoid cysts are benign choristomas that arise from the entrapment of ectodermal elements adjacent to the fetal bony suture lines. They are considered congenital, but not all are diagnosed at birth. They are the most common orbital tumors in children. While superficial dermoid cysts appear early in life, deep dermoid cysts remain clinically occult until adolescence or adulthood, when they enlarge and may cause proptosis, ocular and orbital symptoms, and even neurological symptoms. In addition, many deep orbital dermoid cysts present with chronic inflammation resulting from lipid leakage from the cysts. They pose a diagnostic and therapeutic challenge, require radiological imaging for planning the surgical approach and may be difficult to remove. Early diagnosis and complete surgical removal of the cysts are the recommended therapeutic approach. In this paper, we present a literature review of deep orbital dermoid cysts to provide useful guidance for their diagnosis and management.
Topics: Child; Adolescent; Infant, Newborn; Humans; Adult; Dermoid Cyst; Orbital Neoplasms; Inflammation; Tomography, X-Ray Computed; Radiology
PubMed: 36916079
DOI: No ID Found -
Archivos de La Sociedad Espanola de... Feb 2017The case is presented of a 46-year-old male with right eye proptosis and conjunctival hyperaemia, of 18 months onset. A well-defined intraconal mass was found in the... (Review)
Review
CASE REPORT
The case is presented of a 46-year-old male with right eye proptosis and conjunctival hyperaemia, of 18 months onset. A well-defined intraconal mass was found in the computed tomography. In magnetic resonance this was hypo-intense on T1, enhanced with gadolinium and hyperintense on T2. Excisional biopsy was performed, which was reported as a well-differentiated liposarcoma in the histopathology study.
DISCUSSION
Liposarcoma is a malignant adipose tissue tumour. It is very rare in the orbit, with 5 histological types, the most common being myxoid. The treatment of choice is wide surgical excision and may be accompanied with radiotherapy. As it is an infiltrative tumour, It has a high rate of recurrence.
Topics: Biopsy; Combined Modality Therapy; Contrast Media; Eye Evisceration; Gadolinium; Humans; Liposarcoma; Magnetic Resonance Imaging; Male; Middle Aged; Orbital Neoplasms; Radiotherapy, Adjuvant; Tomography, X-Ray Computed
PubMed: 27236610
DOI: 10.1016/j.oftal.2016.04.001 -
Laryngo- Rhino- Otologie Apr 2021This article provides an overview of rare orbital diseases. Congenital malformations, inflammatory diseases, benign and malignant neoplasias are described. Although it... (Review)
Review
This article provides an overview of rare orbital diseases. Congenital malformations, inflammatory diseases, benign and malignant neoplasias are described. Although it represents a relatively small area of the body the orbit contains multiple different tissues. Therefore, a great variety of diseases can be found within the orbital space. That is the reason, why both the completeness and the level of detail in the description of particular diseases must be somewhat limited. Nevertheless, clinical manifestations, important aspects of diagnosis, treatment strategies, and, when specific data are available, the prognosis are described. The authors tried to highlight the most characteristic aspects of the different diseases to describe their relevant aspects in spite of the brevity of the subsections.
Topics: Humans; Orbit; Orbital Diseases; Orbital Neoplasms; Rare Diseases
PubMed: 34352903
DOI: 10.1055/a-1384-4641 -
Journal Francais D'ophtalmologie Apr 2016Orbital tumors are a rather frequent pathology. Their diagnosis and treatment may be difficult. They can be benign or malignant. All the tissues of the orbit can give... (Review)
Review
Orbital tumors are a rather frequent pathology. Their diagnosis and treatment may be difficult. They can be benign or malignant. All the tissues of the orbit can give rise to a tumor, resulting in their large number. Among the benign tumors, we have meningiomas and cavernous hemangiomas, and for the malignant tumors, lymphomas, metastasis, ENT tumors and lacrimal gland tumors in the adult. Usually the signs are nonspecific, with proptosis, oculomotor disturbance, inflammatory signs, pain and sometimes a mass. Imaging (CT, MRI and color Doppler ultrasound) shows the tumor, its location, extent and possible metastases. Biopsy and anatomic and cytopathologic examination confirm the type of benign or malignant tumor. Based on these three elements: clinical appearance, imaging and histology, the tumor will be treated, usually by a surgical approach according to the recommendations of a multidisciplinary tumor conference. Radiation therapy and chemotherapy may supplement the treatment.
Topics: Adult; Age of Onset; Diagnostic Techniques, Ophthalmological; Hemangioma, Cavernous; Humans; Magnetic Resonance Imaging; Orbit; Orbital Neoplasms; Tomography, X-Ray Computed
PubMed: 27017475
DOI: 10.1016/j.jfo.2015.11.009 -
Asia-Pacific Journal of Ophthalmology... 2018The recent International Society for the Study of Vascular Anomalies (ISSVA) classification of vascular anomalies can be applied to orbital lesions, dividing them into... (Review)
Review
The recent International Society for the Study of Vascular Anomalies (ISSVA) classification of vascular anomalies can be applied to orbital lesions, dividing them into vascular tumors and vascular malformations. Orbital cavernous hemangiomas are probably best considered cavernous venous malformations under this classification. Management of symptomatic lesions can be with surgical excision or stereotactic fractionated radiotherapy in selected cases. Beta-blockers including propranolol and topical timolol maleate represent first-line therapy for infantile hemangiomas, although surgery has a role in selected cases. Orbital venous-lymphatic malformations are problematic but with improved imaging, neuroradiological intervention, and a multidisciplinary approach to management, outcomes are improving.
Topics: Hemangioma; Hemangioma, Cavernous; Humans; Orbit; Orbital Neoplasms; Vascular Malformations
PubMed: 29633786
DOI: 10.22608/APO.2017151 -
Middle East African Journal of... 2018Incidental orbital masses that are asymptomatic and appear benign are often observed without surgical intervention unless there is a clinical or radiographic change in... (Review)
Review
Incidental orbital masses that are asymptomatic and appear benign are often observed without surgical intervention unless there is a clinical or radiographic change in the mass. There is a burgeoning population of cancer patients with incidental masses that have been detected while under surveillance for metastasis. This population of patients is growing due to a number of reasons, including more extensive imaging, an aging population, and more effective cancer treatments. Closer scrutiny should be applied to these patients, due to the possibility of the mass being an orbital metastasis. In addition, the approach to these patients may have implications regarding the adult patient without a cancer history who presents with a symptomatic orbital mass. The purpose of this paper is to explore the approach to the patient with and without a cancer history who presents with an orbital mass.
Topics: Biopsy; Decision Making; Humans; Neoplasm Metastasis; Orbital Diseases; Orbital Neoplasms
PubMed: 30122850
DOI: 10.4103/meajo.MEAJO_93_18 -
Der Ophthalmologe : Zeitschrift Der... Oct 2021There are various options for the conservative treatment of the most frequent orbital tumors. These can delay, complement or be superior to the surgical approach, which... (Review)
Review
BACKGROUND
There are various options for the conservative treatment of the most frequent orbital tumors. These can delay, complement or be superior to the surgical approach, which is often prone to complications.
OBJECTIVE
This article gives a summary of the possible treatment options for the most common orbital tumors in childhood and adulthood.
METHODS
A literature search was carried out and the possible treatment pathways are presented.
RESULTS
1. Frequent orbital tumors in childhood: a systemic treatment with noncardioselective beta blockers is the primary treatment for capillary orbital hemangiomas. In cases of no response, steroids, interferon alpha or cyclophosphamide are treatment options. Observation is a possible option for smaller dermoid cysts, in cases of progression excision can become necessary. Symptomatic optic nerve gliomas can also be observed and in cases of progression treated with chemotherapy, mTOR/MEK inhibitors or radiotherapy (children > 5 years). Rhabdomyosarcomas are biopsied and subsequently treated by radiotherapy and chemotherapy. 2. Frequent orbital tumors in adulthood: asymptomatic cases of cavernous hemangiomas of the orbit can just be observed. Symptomatic hemangiomas can be surgically excised or treated with radiotherapy. For meningiomas of the optic nerve sheath radiotherapy is a very effective treatment. Surgical excision should be reserved for cases with no prognosis of visual acuity. There is also the option to treat with antiprogesterone. Orbital lymphomas with purely orbital involvement can be treated with radiotherapy, chemotherapy or the application of rituximab.
CONCLUSION
There are now very effective conservative treatment options for many orbital tumors. In some cases a surgical procedure can be avoided and a good visual function can be retained.
Topics: Adult; Child; Humans; Meningeal Neoplasms; Meningioma; Orbit; Orbital Neoplasms; Retrospective Studies; Treatment Outcome
PubMed: 34406460
DOI: 10.1007/s00347-021-01471-9