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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 -
International Medical Case Reports... 2023To report a case of neurosarcoidosis (NS) who was initially diagnosed as (CI) infection.
PURPOSE
To report a case of neurosarcoidosis (NS) who was initially diagnosed as (CI) infection.
OBSERVATIONS
A 57-year-old diabetic man presented with sudden painless diminution of vision, metamorphopsia, and color vision deficits in the left eye (OS) for one month. His vision was 20/20 in the right eye (OD) and 20/40 OS. Ophthalmic examination revealed left relative afferent pupillary defect, blurred optic nerve margin, creamy chorioretinal infiltration around the optic disc, and mild macular edema. OD examination was non-revealing. Chest CT scan with contrast showed calcified mediastinal lymph nodes, but biopsy of the lymph nodes was normal. Brain and orbit MRI demonstrated soft tissue abnormality with enhancement in left orbital apex with involvement of the extraocular muscles. CSF culture was negative, but complement fixation had positive titer of 1:2 for CI. The patient was diagnosed with CI meningitis, and antifungal therapy was initiated. Slight visual and symptomatic improvement was observed, which was not completely satisfactory. Biopsy of extraocular orbital muscle five months later revealed non-caseating granulomatous inflammation, leading to initiation of prednisone trial therapy. Nine months later, the patient was referred to a tertiary center owing to persistence of optic disc edema OS. PET CT was consistent with a diagnosis of sarcoidosis. Antifungal treatment was discontinued, and oral prednisone with methotrexate was initiated. Subsequently, methotrexate was replaced by infliximab to further manage ocular inflammation and neurologic symptoms which was effective. Vision was 20/20 OD and 20/30 OS at the most recent visit.
CONCLUSION AND IMPORTANCE
Signs and symptoms of neurosarcoidosis and coccidioidomycosis can be similar and deceiving. The index case underscores importance of considering appropriate differential diagnoses in patients with similar symptoms and signs who may respond to preliminary designated treatment but not to the optimal extent. Considering such possibility could assist clinicians in managing the patients timely and efficiently.
PubMed: 38164515
DOI: 10.2147/IMCRJ.S434632 -
Pediatric Radiology May 2024Orbital pathologies can be broadly classified as ocular, extra-ocular soft-tissue (non-neoplastic and neoplastic), osseous, and traumatic. In part 1 of this orbital...
Orbital pathologies can be broadly classified as ocular, extra-ocular soft-tissue (non-neoplastic and neoplastic), osseous, and traumatic. In part 1 of this orbital series, the authors will discuss the differential diagnosis and key imaging features of pediatric ocular pathologies. These include congenital and developmental lesions (microphthalmos, anophthalmos, persistent fetal vasculature, coloboma, morning glory disc anomaly, retinopathy of prematurity, Coats disease), optic disc drusen, infective and inflammatory lesions (uveitis, toxocariasis, toxoplasmosis), and ocular neoplasms (retinoblastoma, retinal hamartoma, choroidal melanoma, choroidal nevus). This pictorial review provides a practical approach to the imaging work-up of these anomalies with a focus on ocular US as the first imaging modality and additional use of CT and/or MRI for the evaluation of intracranial abnormalities. The characteristic imaging features of the non-neoplastic mimics of retinoblastoma, such as persistent fetal vasculature and Coats disease, are also highlighted.
Topics: Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Diagnosis, Differential; Diagnostic Imaging; Eye Diseases; Orbital Diseases
PubMed: 38321238
DOI: 10.1007/s00247-024-05869-w -
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 -
Clinical Imaging Sep 2023Given the demands of a busy high-volume trauma center, trauma radiologists are expected to evaluate an enormous number of images covering a multitude of facial bones in... (Review)
Review
Given the demands of a busy high-volume trauma center, trauma radiologists are expected to evaluate an enormous number of images covering a multitude of facial bones in a short period of time in severely traumatized patients. Therefore, a comprehensive checklist, search pattern, and practical approach become indispensable for evaluation. Moreover, fracture complex classification conveys abundant information in a succinct shorthand fashion, which can be a large asset in a busy high-volume trauma center: reliably helping clinicians communicate urgent findings, make early treatment decisions, and effectively plan surgical approaches. Traditionally, radiologists' approach the CT axial dataset in top-down fashion: navigating their descent craniocaudal. However, a bottom-up approach may be advantageous, especially when it comes to facial fracture complex classification. Four key anatomic landmarks of the face, when evaluated sequentially in bottom-up fashion, are favorable to rapid single-sweep facial fracture characterization: the mandible, the pterygoid plates, the zygoma, and the bony orbits. That is, when done in succession: 1. Clearing the mandible rules out a panfacial smash fracture. 2. Clearing the pterygoid plates effectively rules out a Le Fort I, II, and III fracture. 3. Clearing the zygoma effectively rules out a zygomaticomaxillary complex (ZMC) type fracture. 4. Clearing the bony orbits effectively rules out a naso-orbital-ethmoid (NOE) fracture. Following this process of exclusion and elimination; as one ascends through the face, fracture characterization becomes more manageable and straightforward. Besides identifying all of the fractures and using the appropriate classification system, the radiologist also needs to recognize key clinically relevant soft tissue injuries that may be associated with facial fractures and thus should address these in the report.
Topics: Humans; Tomography, X-Ray Computed; Skull Fractures; Facial Bones; Ethmoid Bone; Orbit
PubMed: 37379713
DOI: 10.1016/j.clinimag.2023.06.015 -
Clinics in Dermatology Feb 2024Pediatric tumors of the eye and orbit can be benign or malignant as well as congenital or acquired and are usually distinctively different than those seen in adults....
Pediatric tumors of the eye and orbit can be benign or malignant as well as congenital or acquired and are usually distinctively different than those seen in adults. Although most of these neoplasms are benign (eg, dermoid cyst, chalazion, molluscum), their location near and within a vital organ can result in serious dermatologic and ophthalmologic sequelae. Lesions discussed include vascular lesions, retinoblastomas (the most common primary pediatric intraocular malignancy), rhabdomyosarcoma (the most common primary pediatric orbital malignancy), Langerhans cell histiocytosis, and metastatic lesions to the orbit (neuroblastoma, Ewing sarcoma). Although cysts and ocular melanoma can occur within the pediatric population, these conditions are covered in other contributions in this issue of Clinics in Dermatology.
PubMed: 38301859
DOI: 10.1016/j.clindermatol.2024.01.012 -
Orbit (Amsterdam, Netherlands) Sep 2023Traditionally, for patients who are poor candidates for surgery and/or radiotherapy, palliative chemotherapy is often offered but with significant toxic side effects.... (Review)
Review
Traditionally, for patients who are poor candidates for surgery and/or radiotherapy, palliative chemotherapy is often offered but with significant toxic side effects. However, recent advancements in our understanding of tumor biology and molecular genetics have brought new understanding to the molecular pathways of certain tumors and cancers. This has ushered in a new era of precision medicine specific to a tumor or cancer treatment pathway (targeted therapy) or directed to host-tumor responses (immunotherapy). This article will focus on recent updates in the application of available targeted and immunotherapy for managing orbital and periorbital tumors and tumor-like conditions, which include cutaneous basal cell carcinoma, cutaneous squamous cell carcinoma, cutaneous melanoma, Merkel cell carcinoma, sebaceous gland carcinoma, solitary fibrous tumor, dermatofibrosarcoma protuberans, orbital meningioma, neurofibromatosis, Langerhans cell histiocytosis, ocular adnexal lymphoma, orbital lymphatic malformation, and adenoid cystic carcinoma.
PubMed: 37728602
DOI: 10.1080/01676830.2023.2256848 -
Cancers Dec 2023Orbital and ocular adnexa lymphomas are rare neoplasms confined to the orbital region. The prognosis is generally favorable, with a high proportion of localized disease,... (Review)
Review
Orbital and ocular adnexa lymphomas are rare neoplasms confined to the orbital region. The prognosis is generally favorable, with a high proportion of localized disease, indolent clinical course, prolonged disease-free intervals, and low lymphoma-related mortality rate. We report our experience on eleven patients with confirmed histological diagnosis of lymphoma stage IE-IIE, treated between 2010 and 2021 with radiotherapy alone or in association with chemotherapy or immunotherapy. Eight patients were treated with primary radiotherapy only, while three received previous systemic treatments. Six patients were treated with Proton beam therapy (PBT), and five with external beam radiotherapy (EBRT). The five-year local control rate was 98%; only one patient developed an out-of-field recurrence. We also conducted a comprehensive literature review using electronic databases (PubMed, EMBASE, and Cochrane Library). Articles were selected based on their pertinence to treatment of the ocular and adnexal lymphoma focusing on radiotherapy techniques (electron beam radiotherapy, photon beam radiotherapy, or proton beam radiotherapy), treatment total dose, fractionation schedule, early and late radio-induced toxicities, and patient's clinical outcome. Radiotherapy is an effective treatment option for orbital lymphoma, especially as standard treatment in the early stage of orbital lymphoma, with excellent local control rate and low rates of toxicity.
PubMed: 38136328
DOI: 10.3390/cancers15245782 -
The British Journal of Radiology Aug 2023To investigate the interdisciplinary interobserver reproducibility of Hertel-exophthalmometry-like protrusion measurements on multidetector-row-computed-tomography-... (Observational Study)
Observational Study
OBJECTIVES
To investigate the interdisciplinary interobserver reproducibility of Hertel-exophthalmometry-like protrusion measurements on multidetector-row-computed-tomography- (MDCT-) images of the orbit to facilitate structured evaluation of the orbit and mid-face.
METHODS
Respective reproducibility of base-length along the interfronto-zygomatic line, right and left ocular protrusion, and deriving interocular difference was measured in this retrospective (04/2009-03/2020) single-centre observational study. MDCT-series and slice-positions were selected independently, using picture-archiving-and-communication-system- (PACS-) tools on tilt-corrected axial MDCT-images (slice-thickness 0.6-3.0 mm, window/centre 350/50 HU) in 37 selected adult patients (24 female, age 57 ± 13 years, average±standard-deviation) with clinical indication for Hertel-exophthalmometry, by one radiology-attending, two ophthalmology-attendings, one critical-care-attending, and one ear-nose-throat-surgery resident, respectively. Bland-Altman plots and Wilcoxon-matched-pairs-signed-rank-tests compared interobserver results.
RESULTS
Mean and median interobserver and intraobserver (radiology-attending) deviations were within 1 mm of respective averages of base-length (98 ± 4 mm), right and left ocular protrusion (21 ± 4 mm) and interocular difference (2 ± 1 mm). Relative interobserver deviations were within 2.0% of average (all patients) for base-length, and 5.0% (>80% of patients) for ocular protrusion. Pairwise interobserver comparison showed no significant differences between interocular differences of protrusion.
CONCLUSIONS
Respective measurements of base-length, ocular protrusion, and deriving interocular difference show high interdisciplinary interobserver reproducibility in tilt-corrected axial MDCT-images of the orbit or mid-face.
ADVANCES IN KNOWLEDGE
Hertel-exophthalmometry-like protrusion measurements did not depend on the years of experience or the medical subspecialty of the observer. Measurements are objective, well reproducible and important for multiple medical disciplines and should thus be included in pertinent radiology reports.
Topics: Adult; Humans; Female; Middle Aged; Aged; Exophthalmos; Reproducibility of Results; Retrospective Studies; Diagnostic Techniques, Ophthalmological; Multidetector Computed Tomography; Observer Variation
PubMed: 37334789
DOI: 10.1259/bjr.20211408 -
Clinics in Dermatology Jan 2024Lymphomas of the eye and ocular adnexa are rare lymphoproliferative diseases of the ocular and ocular adnexal tissue. The incidence of these diseases has been rapidly...
Lymphomas of the eye and ocular adnexa are rare lymphoproliferative diseases of the ocular and ocular adnexal tissue. The incidence of these diseases has been rapidly increasing over the past few decades. The exact pathogenesis remains unknown, but it is postulated to be multifactorial and includes genetic aberrations, epigenetic and environmental factors, infectious agents, and chronic antigenic stimulation. The majority of ocular and ocular adnexal lymphomas are of B-cell origin, except for eyelid lymphomas, which are more often of T-cell type. Lymphoproliferative diseases of ocular and ocular adnexal structures are either primary, when they arise in the eye, orbit, lacrimal gland, eyelid, and/or conjunctiva, or secondary extranodal manifestation of systemic lymphoma. Diagnosis is challenging and requires a multidisciplinary approach involving ophthalmologists, dermatologists, oncologists, and radiation oncologists.
PubMed: 38281690
DOI: 10.1016/j.clindermatol.2024.01.009