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International Journal of Oncology Feb 2020Orbital and ocular anatomy is quite complex, consisting of several tissues, which can give rise to both benign and malignant tumors, while several primary neoplasms can... (Review)
Review
Orbital and ocular anatomy is quite complex, consisting of several tissues, which can give rise to both benign and malignant tumors, while several primary neoplasms can metastasize to the orbital and ocular space. Early detection, accurate staging and re‑staging, efficient monitoring of treatment response, non‑invasive differentiation between benign and malignant lesions, and accurate planning of external radiation treatment, are of utmost importance for the optimal and individualized management of ophthalmic oncology patients. Addressing these challenges requires the employment of several diagnostic imaging techniques, such as high‑definition digital fundus photography, ultrasound imaging, optical coherence tomography, optical coherence tomography (OCT)‑angiography, computed tomography (CT) and magnetic resonance imaging (MRI). In recent years, technological advances have enabled the development of hybrid positron emission tomography (PET)/CT and PET/MRI systems, setting new standards in cancer diagnosis and treatment. The capability of simultaneously targeting several cancer‑related biochemical procedures using positron emitting‑radiopharmaceuticals, while morphologically characterizing lesions by CT or MRI, together with the intrinsic quantitative capabilities of PET‑imaging, provide incremental diagnostic information, enabling accurate, highly efficient and personalized treatment strategies. Aim of the current review is to discuss the current applications of hybrid PET/CT and PET/MRI imaging in the management of patients presenting with the most commonly encountered orbital and ocular tumors.
Topics: Eye Neoplasms; Humans; Magnetic Resonance Imaging; Multimodal Imaging; Orbital Neoplasms; Positron-Emission Tomography; Radiopharmaceuticals; Tomography, X-Ray Computed
PubMed: 31939615
DOI: 10.3892/ijo.2020.4955 -
Pediatrics in Review Mar 2024Tumors of the eye, orbit, and ocular adnexa can arise in the pediatric population. These entities can be both vision- and life-threatening and may be associated with... (Review)
Review
Tumors of the eye, orbit, and ocular adnexa can arise in the pediatric population. These entities can be both vision- and life-threatening and may be associated with systemic disease. Given their relative rarity, pediatricians must be aware of these conditions and understand what findings warrant immediate referral to an ophthalmologist for initiation of further testing. We aimed to review these conditions and highlight clinical features to promote awareness and expedite diagnosis. Tumors are subdivided into the following categories for review: anterior tumors of the eyelid and ocular surface, orbital tumors, and intraocular tumors.
Topics: Humans; Child; Eye Neoplasms; Orbital Neoplasms; Face
PubMed: 38425168
DOI: 10.1542/pir.2023-006163 -
Der Ophthalmologe : Zeitschrift Der... Oct 2021The indications for orbital tumor surgery are an incisional biopsy to confirm the diagnosis or in malignant operable tumors a complete excision or a debulking to avoid... (Review)
Review
The indications for orbital tumor surgery are an incisional biopsy to confirm the diagnosis or in malignant operable tumors a complete excision or a debulking to avoid complications in large invasively infiltrating tumors. In the case of benign tumors, the indications for surgery depend mostly on the clinical symptoms and cosmetic esthetic disfigurement. In the present article the preoperative examinations as well as surgical access approaches to different orbital regions, endoscopic procedures and methods of intraoperative navigation are presented. Magnetic resonance imaging is the instrument of choice, whereby in many cases computed tomography (CT) adds further information. Depending on the indications, diffusion-weighted sequences, CT angiography and digital subtraction angiography (DSA, catheter angiography) are added to the preoperative diagnostics. For space-occupying lesions located anterior to the bulbar equator, an anterior orbitotomy can be performed transconjunctivally or transpalpebrally. A lateral orbitotomy is used to reach lateral, laterocranial, and lateroinferior orbital segments, whereas transcranial approaches are suitable for processes located far posterior and for those with retro-orbital intracranial extension as well as for processes in the optic foramen/superior orbital fissure. The indications for an endonasal access approach are processes medial to the bulb or optic nerve and up to the orbital apex. A transantral access can be chosen for caudal, mediolateral, and medioinferior space-occupying lesions. Modern orbital surgery is complemented by endoscopic procedures and intraoperative navigation. Orbital tumors belong to the interdisciplinary relevant diseases. Therefore, an optimal management takes place at specialized multidisciplinary centers.
Topics: Biopsy; Endoscopy; Humans; Orbit; Orbital Neoplasms; Tomography, X-Ray Computed
PubMed: 33893529
DOI: 10.1007/s00347-021-01386-5 -
Ophthalmic Plastic and Reconstructive...Tumors of the medial orbital apex can be challenging to resect. Primary endonasal resection can be limited by requirements for fat retraction and poor angulation for...
PURPOSE
Tumors of the medial orbital apex can be challenging to resect. Primary endonasal resection can be limited by requirements for fat retraction and poor angulation for lateral orbital dissection. Orbital resection alone can have space limitations and a limited view for dissection of the posterior aspect of the tumor. The combined transorbital transnasal approach integrates the retraction and instrumentation advantages of the transorbital approach with the optical and space advantages of the transnasal approach.
METHODS
In this cross-sectional cohort study, patients who underwent surgical resection of an apical orbital tumor via a combined endoscopic and orbital approach from 2014 to 2019 were identified. Clinical history, examination findings, imaging, surgical technique, histopathology, and complications were reviewed.
RESULTS
Six patients were included. Patients presented with proptosis (5), optic neuropathy (3), and motility restriction (2). Surgery involved a medial orbitotomy and endoscopic ethmoidectomy ± sphenoidotomy, medial wall removal, and periosteum opening. The lesion was prolapsed into the sinus, dissected at the apex via the orbital approach, and removed through the nasal cavity. Pathologic diagnoses were cavernous venous malformation (4), venolymphatic malformation (1), and metastatic carcinoid (1). Improvement in pain (2/2), optic neuropathy (2/3), and proptosis (5/5) were noted. Complications included ethmoid mucocele and nasal polyps.
CONCLUSIONS
The combined endonasal and orbital approach involves prolapsing the lesion into the nasal cavity to aid in dissection on the lateral and posterior aspects, maximizing visibility of the tight intraconal space. This technique can be performed by a team of orbital surgeons experienced in both orbital and endonasal surgery.
Topics: Cross-Sectional Studies; Endoscopy; Exophthalmos; Humans; Optic Nerve Diseases; Orbit; Orbital Neoplasms
PubMed: 35170569
DOI: 10.1097/IOP.0000000000002139 -
Cancer Medicine Aug 2023Metastatic ocular and orbital melanomas are extremely rare. The clinical characteristics and standard treatments for these patients are not fully established.
PURPOSE
Metastatic ocular and orbital melanomas are extremely rare. The clinical characteristics and standard treatments for these patients are not fully established.
MATERIALS AND METHODS
We retrospectively analyzed patients with metastatic ocular and orbital melanoma from Fudan University Shanghai Cancer Center and Eye & ENT Hospital of Fudan University between January 2012 and May 2022.
RESULTS
Overall, 51 patients with metastatic ocular and orbital melanoma were included. The most common primary sites were uvea (73%), followed by conjunctiva (22%), lacrimal sac (4%), and orbit (2%). Patients with uveal melanoma (UM) had a significantly younger age (48 vs. 68 years, p < 0.001), higher incidence of liver metastases (89% vs. 9%, p<0.001), a lower incidence of lymph nodes metastases (16% vs. 46%, p = 0.043) and a lower incidence of BRAF mutation (0% vs. 55%, p<0.001) compared with patients with conjunctival melanoma (CM). The overall response rate of the first-line treatment was 18%. Three of the four patients with BRAF-mutated CM responded to dabrafenib and trametinib treatment. The median progression-free survival (PFS) and overall survival (OS) of first-line treatment were 5.1 and 11.9 months, respectively. Among patients with liver metastases, liver-directed treatment was correlated with better patient PFS (p < 0.001) and OS (p < 0.001) after adjusting for number of metastatic sites and primary sites.
CONCLUSION
CM and UM have different characteristics. Patient with CM had a high incidence of BRAF mutation, and the treatment of BRAF and MEK inhibitors conferred clinical benefit. Liver directed therapies had a potential benefit in disease control in patients with liver metastases.
Topics: Humans; Skin Neoplasms; Proto-Oncogene Proteins B-raf; Retrospective Studies; Antineoplastic Combined Chemotherapy Protocols; Orbital Neoplasms; China; Melanoma; Prognosis; Mutation
PubMed: 37409486
DOI: 10.1002/cam4.6273 -
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 -
Laryngo- Rhino- Otologie May 2024Diagnosis and therapy of orbital diseases is an interdisciplinary challenge, in which i.e. otorhinolaryngologists, ophthalmologists, radiologists, radiation therapists,... (Review)
Review
Diagnosis and therapy of orbital diseases is an interdisciplinary challenge, in which i.e. otorhinolaryngologists, ophthalmologists, radiologists, radiation therapists, maxillo-facial surgeons, endocrinologists, and pediatricians are involved. This review article describes frequent diseases which both, otolaryngologists and ophthalmologists are concerned with in interdisciplinary settings. In particular the inflammatory diseases of the orbit including orbital complications, autoimmunological diseases of the orbit including Grave´s orbitopathy, and primary and secondary tumors of the orbit are discussed. Beside describing the clinical characteristics and diagnostic steps the article focusses on the interdisciplinary therapy. The review is completed by the presentation of most important surgical approaches to the orbit, their indications and possible complications. The authors tried to highlight the relevant facts despite the shortness of the text.
Topics: Humans; Orbital Diseases; Interdisciplinary Communication; Patient Care Team; Intersectoral Collaboration; Orbital Neoplasms
PubMed: 38697143
DOI: 10.1055/a-2216-8879 -
Die Ophthalmologie May 2024Orbital and periocular metastatic tumors used to be considered very rare; however, with the constant updating of drugs and detection methods for cancer treatment, new... (Review)
Review
Orbital and periocular metastatic tumors used to be considered very rare; however, with the constant updating of drugs and detection methods for cancer treatment, new chemotherapies and radiation treatments are being used. The life expectancy of cancer patients has become longer and periocular metastases are becoming easier to detect. Our knowledge of this rare disease of metastases also needs to be updated. This article reviews the incidence, symptomatic presentation, clinical features, diagnostic approaches and current treatment of metastatic tumors of the orbit and ocular adnexa in these patients.
Topics: Humans; Orbital Neoplasms; Eyelid Neoplasms; Eye Neoplasms
PubMed: 38700760
DOI: 10.1007/s00347-024-02035-3 -
Hong Kong Medical Journal = Xianggang... Jun 2021
Topics: Humans; Melanoma; Orbital Neoplasms
PubMed: 34168091
DOI: 10.12809/hkmj208788 -
World Neurosurgery Jul 2020Primary dedifferentiated liposarcoma of the orbit is a rare condition. (Review)
Review
BACKGROUND
Primary dedifferentiated liposarcoma of the orbit is a rare condition.
CASE DESCRIPTION
A 41-year-old man presented with a history of right ophthalmalgia with eye movement and exophthalmos that had developed 2 months earlier. Neuroimages revealed a right orbital mass located above the superior rectus muscle. Tumor resection through the right frontal craniotomy was performed. Histopathologic findings showed the dedifferentiated liposarcoma.
CONCLUSIONS
Primary orbital dedifferentiated liposarcomas remain challenging for their diagnosis and standard treatment. Clinicians should keep in mind the possibility of liposarcoma with orbital tumors.
Topics: Adult; Humans; Liposarcoma; Male; Orbital Neoplasms
PubMed: 32339743
DOI: 10.1016/j.wneu.2020.04.069