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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 -
Radiologie (Heidelberg, Germany) Mar 2024Orbital tumours include a variety of orbital diseases of different origins. In the case of malignant orbital tumours, early detection is important so that treatment can... (Review)
Review
Orbital tumours include a variety of orbital diseases of different origins. In the case of malignant orbital tumours, early detection is important so that treatment can be initiated promptly. Neuroradiological imaging, in particular magnetic resonance imaging (MRI), plays an important role in the diagnostic of orbital tumours. In adults, lymphoproliferative diseases, inflammations and secondary orbital tumours are most frequently found, whereas in children mostly dermoid cysts, optic gliomas and capillary haemangiomas are found. Optic glioma is a pilocytic astrocytoma and accounts for two thirds of all primary optic tumours. Optic nerve sheath meningiomas mostly affect middle-aged women. In childhood, retinoblastoma is the most common intraocular tumour. This is an aggressive malignant tumour which can occur unilaterally or bilaterally. Based on the imaging findings, differential diagnoses can usually be easily narrowed down using criteria such as age of manifestation, frequency, localisation and imaging characteristics.
Topics: Adult; Child; Middle Aged; Humans; Female; Orbital Neoplasms; Orbital Diseases; Optic Nerve Neoplasms; Optic Nerve Glioma; Meningeal Neoplasms; Retinal Neoplasms
PubMed: 38194103
DOI: 10.1007/s00117-023-01257-x -
The Journal of Craniofacial Surgery Sep 2019Paragangliomas are groups of neuroendocrine neoplasms originating from neural crest cells throughout the body, but are rarely observed within the orbit.
BACKGROUND
Paragangliomas are groups of neuroendocrine neoplasms originating from neural crest cells throughout the body, but are rarely observed within the orbit.
CASE REPORT
Two patients, a 4-year-old male and 27-year-old female, presented with a slow-growing proptosis. Well-defined masses were located in the superomedial (male) and temporal (female) portion of the right orbit and involved the entire bodies of either the superior (male) or lateral (female) rectus muscles. B-mode ultrasound scan revealed a homogeneous, well-defined, hypoechoic mass, with rich blood flow signals inside the mass on CDI. CT scans indicated a well-defined, homogeneous mass with moderate enhancement, while MRI showed a well-defined mass with a salt and pepper appearance due to the prominence of blood vessels in the form of flow-void areas. Anterior orbitotomy and total tumor excision were performed on both patients. Subsequent histological and immunohistochemistry assays confirmed the diagnosis of orbital paraganglioma. The male patient is currently at 14 years of follow-up without recurrence or metastasis. The female patient developed recurrence at 3 months post-surgery. After a second surgery and local radiotherapy, there were no signs of the recurrence as assessed at 13 years of follow-up in this female patient.
CONCLUSION
Orbital paraganglioma is an extremely rare benign tumor. The salt-and-pepper appearance as observed with MRI scan represents a relatively salient characteristic of this condition. Total excision of the lesion by orbitotomy is the treatment of choice and the possibility of tumor recurrence warrants diligent consideration. Radiotherapy is useful in patients with tumor recurrence or subtotal surgical excision.
Topics: Adult; Exophthalmos; Female; Humans; Immunohistochemistry; Magnetic Resonance Imaging; Oculomotor Muscles; Orbital Neoplasms; Paraganglioma; Paraganglioma, Extra-Adrenal; Tomography, X-Ray Computed
PubMed: 30896513
DOI: 10.1097/SCS.0000000000005408 -
HNO May 2022Traditionally, external craniofacial approaches have been used for orbital tumor resection. Over the last 30 years, endoscopic sinonasal and skull base techniques have... (Review)
Review
Traditionally, external craniofacial approaches have been used for orbital tumor resection. Over the last 30 years, endoscopic sinonasal and skull base techniques have become widely used throughout the world. These experiences paved the way for the extension of transnasal endoscopic techniques to the intraconal orbit. Transnasal endoscopic intraconal surgery has several advantages regarding morbidity and outcome as compared with purely external approaches. However, the anatomical knowledge and experience of the surgeon is crucial for the success of the surgery. Endoscopic approaches for intraconal tumor removal are feasible for medial and inferior lesions as well as for lesions lateral to the optic nerve provided they remain inferior to the "plane of resectability" and no optic nerve retraction is required. As intraorbital tumors are rare, new international staging systems including CHEER (Cavernous Hemangioma Exclusively Endonasal Resection) and ORBIT (Orbital Resection by Intranasal Technique) help to standardize safety, efficacy, and outcome.
Topics: Endoscopy; Hemangioma, Cavernous; Humans; Nose; Orbital Neoplasms
PubMed: 35298667
DOI: 10.1007/s00106-022-01156-y -
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 -
The New England Journal of Medicine Jan 2023
Topics: Humans; Rhabdomyosarcoma; Orbital Neoplasms
PubMed: 36648076
DOI: 10.1056/NEJMicm2207328 -
RoFo : Fortschritte Auf Dem Gebiete Der... Apr 2022
Topics: Humans; Orbit; Orbital Neoplasms; Solitary Fibrous Tumors
PubMed: 35272362
DOI: 10.1055/a-1712-4405 -
American Journal of Ophthalmology Jan 2021To highlight the increasing importance of gene fusions in the diagnosis, prognosis, and therapy of ocular adnexal tumors. (Review)
Review
PURPOSE
To highlight the increasing importance of gene fusions in the diagnosis, prognosis, and therapy of ocular adnexal tumors.
DESIGN
Perspective.
METHODS
A focused review of gene fusions, their pathogenic mechanism, and gene fusion detection methods in lacrimal gland and primary orbital and ocular adnexal soft tissue tumors; reappraisal of diagnostic, prognostic, and therapeutic approach to ocular adnexal tumors in light of emerging molecular genetic data.
RESULTS
The widespread implementation of fluorescence in situ hybridization and next-generation sequencing methods in pathology practice has led to identification of recurrent gene rearrangements and fusions in a variety of tumors. As a result, molecular genetic methods have become the gold standard for diagnosis of tumors with overlapping histology and immunophenotype, such as small round blue cell tumors. Identification of canonic gene fusions has led to development of sensitive and specific immunohistochemical markers, such as STAT6 in solitary fibrous tumor. In addition to diagnostic accuracy, gene fusions have prognostic implications, such as unfavorable prognosis of PAX3-FOXO1 fusion in alveolar rhabdomyosarcoma. Finally, recognition of gene fusions as a driving mechanism in neoplasia has led to development of U.S. Food and Drug Administration-approved targeted therapies, such as TRK inhibitors for NTRK fusion-positive cancers.
CONCLUSION
The discovery of recurrent gene fusions in various tumors, including those involving ocular adnexa, has led to a deeper insight into the molecular mechanisms of these neoplasms, revolutionizing our approach to their diagnosis, prognostication, and therapy.
Topics: Biomarkers, Tumor; DNA Copy Number Variations; Eye Neoplasms; Gene Fusion; Gene Rearrangement; Genetic Testing; High-Throughput Nucleotide Sequencing; Humans; In Situ Hybridization, Fluorescence; Lacrimal Apparatus Diseases; Orbital Neoplasms; Prognosis; Soft Tissue Neoplasms
PubMed: 32800827
DOI: 10.1016/j.ajo.2020.08.012 -
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