ABSTRACT
BACKGROUND: Sinonasal malignancies (SNMs) frequently present with orbital invasion. Orbital exenteration (OE) can lead to significant morbidity. Induction chemotherapy (IC) is a promising treatment alternative that may allow for orbit preserving (OP) treatments without compromising patient survival. This systematic review was conducted to synthesize the published data on SNM patients with orbital invasion who underwent IC, including tumor response, orbital outcomes, and survival. METHODS: The study protocol was designed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. Databases Embase, Cochrane, Medline, and Scopus, from inception to July 17, 2023, were searched. RESULTS: Nineteen studies were included, encompassing 305 SNM patients with orbital invasion treated with IC. Fourteen studies reported an overall IC response rate (positive response defined as complete or partial tumor volume reduction) of 77.2%. Among included studies, OE rates after IC ranged from 0 to 40%. Three studies reported a high rate of posttreatment functional orbital preservation (89.8-96.0%). Five studies specifically reported that 62.5% (60 out of 96) of patients were downgraded from planned OE to OP treatment following IC. Three studies reported a significant overall survival (OS) improvement in IC responders versus IC nonresponders. Following IC, 5-year OS ranged from 44.2 to 55.5%. Patients with olfactory neuroblastoma demonstrated the highest IC response rate and lowest OE rate (100 and 0%, respectively) versus those with sinonasal undifferentiated carcinomas (68.4 and 0%) or squamous cell carcinomas (76.7 and 16%). CONCLUSIONS: For select patients, IC may allow for OP in locally advanced SNMs with orbital involvement.
Subject(s)
Induction Chemotherapy , Orbital Neoplasms , Paranasal Sinus Neoplasms , Humans , Paranasal Sinus Neoplasms/drug therapy , Paranasal Sinus Neoplasms/pathology , Orbital Neoplasms/drug therapy , Orbital Neoplasms/pathology , Neoplasm Invasiveness , Treatment Outcome , Orbit/pathologyABSTRACT
Ocular adnexa region (OAR) primary lymphomas are uncommon, accounting for 1-2% of non-Hodgkin lymphomas and 8% of extranodal lymphomas. Extranodal marginal zone lymphoma (EMZL) originates from several epithelial tissues, including the stomach, salivary gland, lung, small intestine, thyroid gland, and ocular adnexa region. Here, we report a 66-year-old female patient who was diagnosed with EMZL of OAR. In consideration of the possible side effect of radiotherapy, such as conjunctivitis, visual acuity impairment, and even retinal complications, she received six cycles of triweekly targeted chemotherapy with rituximab, cyclophosphamide, vincristine, and prednisone (R-CVP) without radiotherapy. Then, she remained in complete remission up to the present day.
Subject(s)
Lymphoma, B-Cell, Marginal Zone , Humans , Female , Aged , Lymphoma, B-Cell, Marginal Zone/drug therapy , Orbital Neoplasms/drug therapy , Rituximab/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/therapeutic useSubject(s)
Hidrocystoma , Sweat Gland Neoplasms , Humans , Mexico/epidemiology , Hidrocystoma/pathology , Hidrocystoma/drug therapy , Hidrocystoma/diagnosis , Female , Male , Middle Aged , Sweat Gland Neoplasms/pathology , Sweat Gland Neoplasms/diagnosis , Adult , Referral and Consultation , Orbital Neoplasms/drug therapy , Orbital Neoplasms/diagnosis , Aged , Botulinum Toxins, Type A/administration & dosage , Botulinum Toxins/administration & dosageABSTRACT
The free radical generation efficiency of nanozymes in cancer therapy is crucial, but current methods fall short. Alloy nanoparticles (ANs) hold promise for improving catalytic performance due to their inherent electronic effect, but there are limited ways to modulate this effect. Here, a self-driven electric field (E) system utilizing triboelectric nanogenerator (TENG) and AuPd ANs with glucose oxidase (GOx)-like, catalase (CAT)-like, and peroxidase (POD)-like activities is presented to enhance the treatment of 4T1 breast cancer in mice. The E stimulation from TENG enhances the orbital electrons of AuPd ANs, resulting in increased CAT-like, GOx-like, and POD-like activities. Meanwhile, the catalytic cascade reaction of AuPd ANs is further amplified after catalyzing the production of H2 O2 from the GOx-like activities. This leads to 89.5% tumor inhibition after treatment. The self-driven E strategy offers a new way to enhance electronic effects and improve cascade catalytic therapeutic performance of AuPd ANs in cancer therapy.
Subject(s)
Nanoparticles , Neoplasms , Orbital Neoplasms , Animals , Mice , Electrons , Orbital Neoplasms/drug therapy , Neoplasms/drug therapy , Glucose Oxidase , Hydrogen PeroxideABSTRACT
RATIONALE: Adenoid cystic carcinoma (ACC) of orbit is a very rare epithelial tumor, often originating from the lacrimal glands. At the same time, treatment options are currently limited, such as radiation, chemotherapy. We report a case of a patient treated with antirotinib combined with radiotherapy. PATIENT CONCERNS: A 13-year-old girl was initially admitted with "left eye swelling for over half a year, 12 days after surgery for left orbital adenoid cystic carcinoma". Initial swelling of the lateral upper eyelid of the left eye, with gradual enlargement and occasional pain. DIAGNOSES: Left orbital adenoid cystic carcinoma. INTERVENTIONS: After diagnosis of orbital ACC, she underwent resection of the left orbital mass, and received 33 times of adjuvant radiotherapy, but brain metastases appeared later. She refused further treatment, and received 25 times of radiotherapy and anlotinib therapy after the disease progressed again. OUTCOMES: Now the patient has been followed up for 8 months, but no progress was found. LESSONS: Based on this, we hypothesized that radiation therapy in combination with anlotinib is effective for ACC or ACC metastases.
Subject(s)
Carcinoma, Adenoid Cystic , Orbital Neoplasms , Radiation Oncology , Female , Humans , Adolescent , Carcinoma, Adenoid Cystic/drug therapy , Orbital Neoplasms/drug therapy , EyelidsABSTRACT
A man in his 70s presented with painless bilateral eyelid oedema and vertical diplopia. Evaluation showed a restrictive pattern of extraocular motility testing with MRI demonstrating significant enlargement of the right superior rectus and left superior oblique muscles along with right orbital fat stranding. Subsequent right orbital biopsy revealed poorly differentiated high-grade neuroendocrine carcinoma without a systemic primary site on further diagnostic workup. The patient was treated with carboplatin and etoposide and passed away from an infection a month after diagnosis. This case along with a review of other published cases highlights the varied presentation of orbital neuroendocrine carcinomas that may mimic a broad differential of orbital processes, thus requiring careful diagnostic workup. Subsequently, additional considerations in metastatic evaluation should be based on tumour histological features.
Subject(s)
Angioedema , Carcinoma, Neuroendocrine , Neuroendocrine Tumors , Orbital Neoplasms , Male , Humans , Orbital Neoplasms/diagnostic imaging , Orbital Neoplasms/drug therapy , Carcinoma, Neuroendocrine/diagnostic imaging , Carcinoma, Neuroendocrine/drug therapy , Adipose TissueABSTRACT
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.
Subject(s)
Melanoma , Orbital Neoplasms , Skin Neoplasms , Humans , Skin Neoplasms/pathology , Proto-Oncogene Proteins B-raf/genetics , Retrospective Studies , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Orbital Neoplasms/drug therapy , Orbital Neoplasms/genetics , China , Melanoma/drug therapy , Melanoma/genetics , Melanoma/pathology , Prognosis , MutationABSTRACT
Primary yolk sac tumor of the orbit is a rare entity. Orbital involvement is usually seen in young children and proptosis is the commonest presentation. Aggressive orbital involvement and presentation as a fungating mass is rarely seen. We report a case of primary orbital yolk sac tumor with an aggressive presentation that responded well to systemic chemotherapy.
Subject(s)
Endodermal Sinus Tumor , Exophthalmos , Orbital Neoplasms , Child , Humans , Child, Preschool , Endodermal Sinus Tumor/diagnostic imaging , Endodermal Sinus Tumor/drug therapy , Orbital Neoplasms/diagnostic imaging , Orbital Neoplasms/drug therapy , Orbit/pathology , Exophthalmos/diagnosis , Exophthalmos/pathologyABSTRACT
A 2-year-old boy presented with left periorbital edema, proptosis, hyperglobus and esotropia. Imaging revealed an inferotemporal orbital mass with adjacent bony erosion. Histological evaluation of an orbital biopsy revealed B-cell acute lymphoblastic leukemia/lymphoma (B-ALL/BLL). The patient was subsequently treated with chemotherapy. Although orbital involvement in acute myelogenous leukemia has been well-described, orbital manifestations of B-ALL/BLL are uncommon, with only a limited number of previous reports in the literature.
Subject(s)
Exophthalmos , Lymphoma , Orbital Neoplasms , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Male , Humans , Child, Preschool , Orbital Neoplasms/diagnostic imaging , Orbital Neoplasms/drug therapy , Orbital Neoplasms/pathology , Precursor Cell Lymphoblastic Leukemia-Lymphoma/diagnostic imaging , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Precursor Cell Lymphoblastic Leukemia-Lymphoma/pathology , Exophthalmos/diagnosis , Exophthalmos/etiology , Tomography, X-Ray ComputedABSTRACT
PURPOSE: To review the demographics, clinical features, and response of orbital squamous cell carcinoma treated with cemiplimab. METHODS: This is a retrospective multi-institutional series. Patient characteristics, drug dosing, duration, and response to treatment were evaluated. RESULTS: The study cohort consisted of 11 patients from 5 institutions. All patients received a regimen of 350 mg q 3 weeks and an average of 11.2 cycles (SD 5.8). No patient experienced significant side effects requiring treatment or cessation of cemiplimab. Complete response was achieved in 9 patients (82%) treated with cemiplimab. CONCLUSIONS: Immune checkpoint inhibitors, such as cemiplimab provide a globe-sparing option for the treatment of orbital squamous cell carcinoma. It is important to consider these agents especially when orbital exenteration is the alternative.
Subject(s)
Carcinoma, Squamous Cell , Orbital Neoplasms , Skin Neoplasms , Antibodies, Monoclonal, Humanized/therapeutic use , Carcinoma, Squamous Cell/drug therapy , Carcinoma, Squamous Cell/pathology , Humans , Orbital Neoplasms/drug therapy , Retrospective Studies , Skin Neoplasms/drug therapySubject(s)
Carboplatin/administration & dosage , Melphalan/administration & dosage , Orbital Neoplasms/drug therapy , Retinal Neoplasms/drug therapy , Topotecan/administration & dosage , Antineoplastic Agents/administration & dosage , Antineoplastic Agents, Alkylating/administration & dosage , Child, Preschool , Drug Therapy, Combination , Humans , Infusions, Intra-Arterial , Magnetic Resonance Imaging/methods , Male , Ophthalmic Artery , Orbital Neoplasms/diagnosis , Retinal Neoplasms/diagnosis , Retinoblastoma/drug therapy , Topoisomerase I Inhibitors/administration & dosageABSTRACT
INTRODUCTION: Management of orbital lymphangioma is challenging. Complete surgical excision is often impossible due to its infiltrative nature. Sclerosing agents have been used in its management with variable outcomes. We report a case of recurrent orbital lymphangioma managed with intralesional bleomycin. CASE: A 14-year-old female presented with proptosis of the right eye for two weeks. She had a similar history at five years of age for which she underwent surgical excision. We performed negative pressure aspiration using a 20-gauge angiocatheter, injected bleomycin, and left the cannula in situ for repeat aspiration to maintain cyst collapse. OBSERVATION: The lymphangioma regressed, and there was no recurrence at six months of follow-up. CONCLUSION: This report highlights the use of negative pressure aspiration and intralesional bleomycin injection by minimal intervention using angiocatheter in the successful management of orbital lymphangioma.
Subject(s)
Lymphangioma , Orbital Neoplasms , Adolescent , Bleomycin/therapeutic use , Female , Humans , Injections, Intralesional , Lymphangioma/diagnosis , Lymphangioma/drug therapy , Neoplasm Recurrence, Local/diagnosis , Neoplasm Recurrence, Local/drug therapy , Orbital Neoplasms/diagnosis , Orbital Neoplasms/drug therapySubject(s)
Abdominal Neoplasms/pathology , Eye Hemorrhage/etiology , Neuroblastoma/secondary , Orbital Neoplasms/secondary , Abdominal Neoplasms/drug therapy , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biopsy , Cyclophosphamide/therapeutic use , Eye Hemorrhage/diagnostic imaging , Female , Humans , Infant , Neuroblastoma/complications , Neuroblastoma/diagnostic imaging , Neuroblastoma/drug therapy , Orbital Neoplasms/complications , Orbital Neoplasms/diagnostic imaging , Orbital Neoplasms/drug therapy , Recurrence , Topotecan/therapeutic use , Treatment Outcome , UltrasonographyABSTRACT
A 50-year-old female patient presented with protrusion of the left eye for 1 month. Examination showed abaxial proptosis, restriction of extraocular movements, and elevated intraocular pressure. Computed tomography of the orbits showed soft tissue enhancing lesion in the superolateral aspect of the left orbit with lytic lesions in calvarium. Fine needle aspiration cytology of the lesion revealed a diagnosis of plasmacytoma with positive CD138 and CD38 immunohistochemical stains. Erythrocyte sedimentation rate, C-reactive protein and serum lactate dehydrogenase were elevated. Serum protein electrophoresis revealed hypergammaglobulinemia, and bone marrow biopsy revealed 6% plasma cells. The patient was started on chemotherapy with bortezomib, dexamethasone and lenalidomide by the medical oncologist. Significant improvement in proptosis and extraocular movements noted on follow-up. Orbital myeloma may be the first manifestation of systemic disease.
Subject(s)
Exophthalmos , Multiple Myeloma , Orbital Neoplasms , Plasmacytoma , Biopsy, Fine-Needle , Exophthalmos/diagnosis , Exophthalmos/drug therapy , Exophthalmos/etiology , Female , Humans , Middle Aged , Multiple Myeloma/diagnosis , Multiple Myeloma/drug therapy , Orbital Neoplasms/diagnosis , Orbital Neoplasms/drug therapy , Plasmacytoma/diagnosis , Plasmacytoma/drug therapy , Tomography, X-Ray ComputedSubject(s)
Aortic Coarctation , Eye Abnormalities , Hemangioma, Capillary , Neurocutaneous Syndromes , Orbital Neoplasms , Propranolol/administration & dosage , Adrenergic beta-Antagonists/administration & dosage , Aortic Coarctation/diagnosis , Aortic Coarctation/drug therapy , Aortic Coarctation/physiopathology , Diagnosis, Differential , Eye Abnormalities/diagnosis , Eye Abnormalities/drug therapy , Eye Abnormalities/physiopathology , Female , Hemangioma, Capillary/drug therapy , Hemangioma, Capillary/pathology , Hemangioma, Capillary/physiopathology , Humans , Infant , Magnetic Resonance Imaging/methods , Neurocutaneous Syndromes/diagnosis , Neurocutaneous Syndromes/drug therapy , Neurocutaneous Syndromes/physiopathology , Orbital Neoplasms/drug therapy , Orbital Neoplasms/pathology , Orbital Neoplasms/physiopathology , Prefrontal Cortex/blood supply , Prefrontal Cortex/diagnostic imaging , Treatment OutcomeABSTRACT
A 2-year-old female patient with a recent history of head trauma was admitted to the Ophthalmology Clinic with left exophthalmos. A differential diagnosis between traumatic and tumoral etiology was made. The orbitocranial MRI and fine needle ganglion biopsy settled the malignant etiology of the exophthalmia. Further investigations at the Pediatric Oncology Clinic decided on the diagnosis of orbital metastatic neuroblastoma. This case report presented an unusual association: orbital metastatic neuroblastoma becoming clinically positive soon after a head trauma.
Subject(s)
Abdominal Neoplasms/pathology , Brain Injuries/diagnosis , Exophthalmos/diagnosis , Neuroblastoma/secondary , Orbital Neoplasms/secondary , Abdominal Neoplasms/diagnostic imaging , Abdominal Neoplasms/drug therapy , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biopsy, Fine-Needle , Child, Preschool , Diagnosis, Differential , Fatal Outcome , Female , Humans , Magnetic Resonance Imaging , Neuroblastoma/diagnostic imaging , Neuroblastoma/drug therapy , Orbital Neoplasms/diagnostic imaging , Orbital Neoplasms/drug therapy , Tomography, X-Ray ComputedSubject(s)
Conjunctival Neoplasms , Eye Neoplasms , Immunologic Factors , Lymphoma , Orbital Diseases , Orbital Neoplasms , Rituximab , Eye Neoplasms/drug therapy , Humans , Immunologic Factors/therapeutic use , Lymphoma/drug therapy , Orbital Diseases/drug therapy , Orbital Neoplasms/drug therapy , Rituximab/therapeutic useABSTRACT
The management of metastatic melanoma to the orbit may involve a variety of therapeutic modalities including external-beam radiation, chemotherapy, and varying degrees of surgical resection or debulking. Pembrolizumab is an immunotherapeutic agent that has demonstrated efficacy in the treatment of metastatic melanoma. The authors present a case of metastatic melanoma to the orbit demonstrating profound pseudoprogression within hours of beginning pembrolizumab therapy, with associated mass effect and vision loss. Systemic corticosteroids, orbital external-beam radiation therapy, and a brief interruption in pembrolizumab halted expansion of the orbital lesion and vision loss. This case illustrates that rapid increase in orbital melanoma size, due to acute inflammatory response, may occur after initiation of systemic pembrolizumab therapy. Clinicians should be aware of this pseudoprogression mechanism as a potential cause of vision compromise in metastatic orbital melanoma. Prompt recognition and treatment may be needed to prevent permanent vision loss.