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Surgical Neurology International 2024Orbital tumors, arising within the bony orbit and its contents, present diverse challenges due to their varied origins and complex anatomical context. These tumors,...
BACKGROUND
Orbital tumors, arising within the bony orbit and its contents, present diverse challenges due to their varied origins and complex anatomical context. These tumors, classified as primary, secondary, or metastatic, are further subdivided into intraconal and extraconal based on their relationship with the muscle cone. This classification significantly influences surgical approach and management. This study highlights surgical experiences with orbital tumors, underscoring the importance of tailored surgical approaches based on the lesion's site and its proximity to the optic nerve.
METHODS
This retrospective study at the National Institute of Cancer's Head and Neck Department (2005-2014) analyzed 29 patients with orbital tumors treated with surgery, radiotherapy, chemotherapy, or combinations of them. Patient demographics, tumor characteristics, and treatment responses were evaluated using computed tomography (CT), magnetic resonance imaging, and positron emission tomography-CT imaging. Malignant tumors often required orbital exenteration and reconstruction, highlighting the study's commitment to advancing orbital tumor treatment.
RESULTS
29 patients (18 females and 11 males, age 18-88 years, mean 53.5 years) with orbital tumors exhibited symptoms such as decreased vision and exophthalmos. Tumors included primary lesions like choroidal melanoma and secondary types like epidermoid carcinoma. Treatments varied, involving a multidisciplinary team for surgical approaches like exenteration, with follow-up from 1 to 9 years. Radiotherapy and chemotherapy were used for specific cases.
CONCLUSION
Our study underscores the need for a multidisciplinary approach in treating orbital tumors, involving various surgical specialists and advanced technologies like neuronavigation for tailored treatment. The integration of surgery with radiotherapy and chemotherapy highlights the effectiveness of multidimensional treatment strategies.
PubMed: 38741993
DOI: 10.25259/SNI_1016_2023 -
Cureus Apr 2024B-cell lymphoblastic lymphoma (B-LBL) is an abnormal proliferation of lymphocyte precursor cells located primarily outside of the bone marrow and peripheral blood,...
B-cell lymphoblastic lymphoma (B-LBL) is an abnormal proliferation of lymphocyte precursor cells located primarily outside of the bone marrow and peripheral blood, typically in the mediastinum or other lymph nodes. It is often a disease of childhood that presents with lymphadenopathy, fatigue, pallor, bone pain, and weight loss with laboratory findings of anemia and thrombocytopenia. Initial presentations prompted by head and neck manifestations are exceedingly rare. A five-year-old girl with no significant past medical history presented with right facial swelling and mild proptosis on ophthalmologic evaluation. She was referred to a tertiary care facility by her local otolaryngologist for further management after computed tomographic imaging revealed right maxillary sinus opacification and erosion of the anterior maxillary bone. Her symptoms were initially responsive to prednisone and amoxicillin-clavulanate, and only right unilateral nasal discharge persisted with a near-complete resolution of other sinonasal symptoms. Notably, laboratory values, including complete blood count, were within normal limits. Given concern for the etiology of the bony erosion, the patient presented for a second opinion, where imaging and biopsy resulted in flow cytometry findings consistent with B-ALL/LBL. After a bone marrow biopsy, the ultimate diagnosis was Murphy's stage III B-cell lymphoblastic lymphoma. Malignant neoplasms of the sinonasal region are rare in children, where primary sinonasal B-LBL is a unique occurrence. Clinical features of sinonasal B-LBL in the paranasal sinuses may masquerade as pathologies such as acute sinusitis, orbital cellulitis, and benign tumors or polyps that can lead to a confounding diagnosis. In this case presentation, an initial response to steroids and antibiotics should not provide false reassurance when other features and signs, such as maxillary bone erosion, may suggest the presence of malignancy.
PubMed: 38738089
DOI: 10.7759/cureus.58132 -
Cureus Apr 2024Orbital hemangiomas are benign vascular tumors commonly affecting infants and young children, often manifesting with proptosis, eyelid swelling, or discoloration....
Orbital hemangiomas are benign vascular tumors commonly affecting infants and young children, often manifesting with proptosis, eyelid swelling, or discoloration. Propranolol has emerged as the primary therapy due to its efficacy in promoting regression and minimizing complications. Here, we present a case of a previously healthy six-month-old male infant with progressive right eyelid swelling and discoloration. Magnetic resonance imaging confirmed a large orbital hemangioma. The patient was referred to an ophthalmology center where treatment with propranolol resulted in substantial improvement. Early recognition and initiation of propranolol therapy are crucial in managing orbital hemangiomas in pediatric patients. This case underscores the successful outcome achievable with pharmacologic intervention and emphasizes the importance of long-term follow-up for monitoring and optimizing patient outcomes.
PubMed: 38738043
DOI: 10.7759/cureus.58062 -
Journal of Medicine and Life Jan 2024Ultrasound can identify important characteristics in primary hypothyroidism and diffuse hyperthyroidism (Graves' disease). Therefore, sonologists are actively...
Ultrasound can identify important characteristics in primary hypothyroidism and diffuse hyperthyroidism (Graves' disease). Therefore, sonologists are actively investigating ultrasound criteria to differentiate between these two conditions. Nevertheless, practice shows the absence of such ultrasonic landmarks. For the first time in the literature, three cases of primary hypothyroidism have demonstrated an ultrasound pattern identical to that of Graves' disease. This pattern includes the presence of goiter, marked total hypoechogenicity of the parenchyma, significantly or moderately increased blood flow intensity ('thyroid inferno'), and elevated peak systolic velocity of the superior thyroid arteries. These signs are less common in hypothyroidism compared to hyperthyroidism. Diagnostic data suggest that the pathogeneses of primary hypothyroidism and Graves' disease share the same mechanisms, leading to similar thyroid ultrasound patterns. One of these shared mechanisms is presumably thyroid overstimulation by the autonomic nervous system, which is adequate to the body's hormonal requirements in hypothyroidism but excessive in hyperthyroidism.
Topics: Humans; Graves Disease; Hypothyroidism; Ultrasonography; Thyroid Gland; Female; Middle Aged; Adult; Male
PubMed: 38737666
DOI: 10.25122/jml-2023-0507 -
Journal of Family Medicine and Primary... Mar 2024To study the varied clinical presentation and outcome of management of orbital diseases and to ascertain the concordance of FNAC with histopathology.
PURPOSE
To study the varied clinical presentation and outcome of management of orbital diseases and to ascertain the concordance of FNAC with histopathology.
MATERIALS AND METHODS
This is a prospective interventional study wherein each patient's clinical presentation was noted. Outcome of management was evaluated based on symptomatic relief, regression of signs, noting any intraoperative or postsurgical complication, recurrence, and cosmetic result in terms of surgical scar.
RESULTS
Neoplasia was commonest 33/76; (43.42%), followed by infective and inflammatory conditions 21/76; (27.63% cases), thyroid-associated orbitopathy accounting for 12/76 cases (15.78%), whereas cystic lesions and vascular malformations were 4/76 (5.26%) each. Other disorders constituted a mere 2.65%. Proptosis was the commonest mode of presentation. It was seen in seventy out of seventy-six patients (92.1%). In 86.3% of the cases, there was successful surgical outcome. Of 22 surgically managed cases, recurrence was noted in three cases (13.6%). 90% concordance was seen with histopathology in cases where FNAC was done prior to surgery for diagnosing nature of disease.
CONCLUSIONS
Majority of orbital and adnexal conditions can be provisionally diagnosed based on imaging; however, an adjunct invasive investigation such as FNAC or biopsy may be required. In the present study, in seven cases (9.2%) FNAC was needed, whereas in two cases (2.63%) biopsy was needed. A definitive diagnosis was confirmed by histopathology in 22 surgically managed cases and by excision biopsy in two. 22/76 (28.94%) of the cases needed surgical treatment, and other cases required conservative or palliative approach.
PubMed: 38736778
DOI: 10.4103/jfmpc.jfmpc_761_23 -
BMC Immunology May 2024Thyroid eye disease (TED) is an inflammatory process involving lymphocyte-mediated immune response and orbital tissue damage. The anti-insulin-like growth factor-1...
Potential role of IGF-1R in the interaction between orbital fibroblasts and B lymphocytes: an implication for B lymphocyte depletion in the active inflammatory phase of thyroid-associated ophthalmopathy.
BACKGROUND
Thyroid eye disease (TED) is an inflammatory process involving lymphocyte-mediated immune response and orbital tissue damage. The anti-insulin-like growth factor-1 receptor (IGF-1R) antibodies produced by B lymphocytes are involved in the activation of orbital fibroblasts and the inflammatory process of orbital tissue damage in TED. The purpose of this study was to explore the role of IGF-1R in the mechanistic connection between orbital fibroblasts and B lymphocytes in TED.
METHODS
Orbital fibroblasts sampled from orbital connective tissues and peripheral B lymphocytes isolated from peripheral blood, which were obtained from 15 patients with TED and 15 control patients, were co-cultured at a ratio of 1:20. The level of IGF-1R expression in orbital fibroblasts was evaluated by flow cytometry and confocal microscopy. Transient B lymphocyte depletion was induced with anti-CD20 monoclonal antibody rituximab, while the IGF-1R pathway was blocked by the IGF-1R binding protein. The expression levels of interleukin-6 (IL-6) and regulated upon activation, normal T cell expressed and secreted (RANTES) in the co-culture model were quantified via ELISA.
RESULTS
IGF-1R expression was significantly elevated in TED orbital fibroblasts compared to that of controls. A 24-h co-culture of orbital fibroblasts with peripheral B lymphocytes induced elevated expression levels of IL-6 and RANTES in each group (TED patients and controls), with the highest levels occurring in TED patients (T + T group). Rituximab and IGF-1R binding protein significantly inhibited increased levels of IL-6 and RANTES in the co-culture model of TED patients.
CONCLUSIONS
IGF-1R may mediate interaction between orbital fibroblasts and peripheral B lymphocytes; thus, blocking IGF-1R may reduce the local inflammatory response in TED. Rituximab-mediated B lymphocyte depletion played a role in inhibiting inflammatory responses in this in vitro co-culture model, providing a theoretical basis for the clinical application of anti-CD20 monoclonal antibodies in TED.
Topics: Female; Humans; Male; B-Lymphocytes; Cell Communication; Cells, Cultured; Chemokine CCL5; Coculture Techniques; Fibroblasts; Graves Ophthalmopathy; Interleukin-6; Lymphocyte Depletion; Orbit; Receptor, IGF Type 1; Rituximab
PubMed: 38734625
DOI: 10.1186/s12865-024-00613-3 -
Chemico-biological Interactions Jun 2024Orbital connective tissue changes are contributors to the pathogenesis in thyroid eye disease (TED). Activated fibroblasts respond to immune stimuli with proliferation...
Orbital connective tissue changes are contributors to the pathogenesis in thyroid eye disease (TED). Activated fibroblasts respond to immune stimuli with proliferation and increased hyaluronan (HA) production. Cyclosporin A (CsA) was reported to be beneficial in the treatment of TED. PDGF isoforms are increased in orbital tissue of TED patients and enhance HA production. We aimed to study the effect of CsA on HA production and hyaluronan synthase (HAS1, 2 and 3) and hyaluronidase (HYAL1 and 2) mRNA expressions in orbital fibroblasts (OFs). Measurements were performed in the presence or absence of CsA (10 μM) in unstimulated or PDGF-BB (10 ng/ml) stimulated OFs. The HA production of TED OFs (n = 7) and NON-TED OFs (n = 6) were measured by ELISA. The levels of mRNA expressions were examined using RT-PCR. The proliferation rate and metabolic activity were measured by BrdU incorporation and MTT assays, respectively. Treatment with CsA resulted in an average 42% decrease in HA production of OFs (p < 0.0001). CsA decreased the expression levels of HAS2, HAS3 and HYAL2 (p = 0.005, p = 0.005 and p = 0.002, respectively.) PDGF-BB increased HA production (p < 0.001) and HAS2 expression (p = 0.004). CsA could reduce the PDGF-BB-stimulated HA production (p < 0.001) and HAS2 expression (p = 0.005) below the untreated level. In addition, CsA treatment caused a decrease in proliferation potential (p = 0.002) and metabolic activity (p < 0.0001). These findings point to the fact that CsA affects HA metabolism via HAS2, HAS3 and HYAL2 inhibition in OFs. In addition to its well characterized immunosuppressant properties, CsA's beneficial effect in TED may be related to its direct inhibitory effect on basal and growth factor stimulated HA production.
Topics: Hyaluronic Acid; Humans; Becaplermin; Fibroblasts; Hyaluronan Synthases; Cyclosporine; Hyaluronoglucosaminidase; Cell Proliferation; Proto-Oncogene Proteins c-sis; Glucuronosyltransferase; Graves Ophthalmopathy; Cells, Cultured; Orbit; RNA, Messenger; Cell Adhesion Molecules; GPI-Linked Proteins
PubMed: 38729283
DOI: 10.1016/j.cbi.2024.111045 -
Aging May 2024Thyroid-associated ophthalmopathy (TAO) is the most prevalent orbital disease in adults caused by an autoimmune disorder, which can lead to disfigurement and vision...
Thyroid-associated ophthalmopathy (TAO) is the most prevalent orbital disease in adults caused by an autoimmune disorder, which can lead to disfigurement and vision impairment. Developing effective treatments for this condition presents challenges due to our limited understanding of its underlying immune aberrations. In this study, we profiled the immune components in the peripheral blood of patients with TAO as well as healthy individuals, utilizing single-cell RNA sequencing and B-cell receptor repertoires (BCR) analysis. We observed a significant reduction in the proportions of regulatory B cells (Bregs) and type 2 conventional dendritic cells (DCs) in patients with TAO during the active phase. Conversely, there was a significant increase in the proportion of type 1 DCs. Further analysis of cell differentiation trajectory revealed potential impairment in the transition of B cells towards Breg phenotype during the active phase of TAO. Besides, the activation process of TAO appeared to involve inflammation and immune dysfunction, as indicated by the dynamic changes in the activities of key regulators. The abnormalities in the peripheral immune system, such as the reduced capacity of Bregs to suppress inflammation, were primarily driven by the enhanced interaction among Breg, DCs, and monocytes (i.e., CD22-PTPRC and BTLA-TNFRSF14). Collectively, our findings offer a comprehensive insight into the molecular regulation and cellular reconfiguration during the active phase of TAO at the single-cell level, in order to explore the pathogenesis of TAO and provide new ideas for the future treatment of TAO.
Topics: Humans; Graves Ophthalmopathy; Single-Cell Analysis; Gene Expression Profiling; Female; Middle Aged; Male; Receptors, Antigen, B-Cell; Dendritic Cells; Adult; Transcriptome; B-Lymphocytes, Regulatory
PubMed: 38728262
DOI: 10.18632/aging.205814 -
F1000Research 2023Introduction Orbital lipoma is an extremely rare tumor, representing less than 1% of all orbital tumors. We review the literature and describe the presentation, the...
Introduction Orbital lipoma is an extremely rare tumor, representing less than 1% of all orbital tumors. We review the literature and describe the presentation, the differential diagnosis and the management of this tumor. Case report We report the case of a 63-year-old patient who was referred for a diplopia with recent hemi-cranial headache. Physical examination showed no exophthalmos nor decrease in visual acuity. The patient complained of diplopia on elevation and oculomotricity examination showed limited elevation of the right eye. The Hess Lancaster test was in favor of a limited course of the right inferior rectus muscle. Magnetic resonance imaging revealed a fusiform tissue process in the right inferior rectus muscle with a fatty signal. A complete excision of the tumor was performed by a trasncunjonctival approach. Cytopathological examination was consistent with a pleomorphic lipoma. The postoperative period was uneventful. The definitive histopathologic diagnosis was a lipoma. The postoperative Magnetic resonance imaging showed the complete disappearance of the lesion. With 3 years of follow up, there is no sign of recurrence or ocular motility trouble. Lipomas are rare tumors in the orbit. The clinic is variable depending on the size and the site. The clinical diagnosis is difficult to make. Only histology allows the final diagnosis.
Topics: Humans; Middle Aged; Orbital Neoplasms; Magnetic Resonance Imaging; Lipoma; Male
PubMed: 38726301
DOI: 10.12688/f1000research.130056.2 -
Journal of Neuroendovascular Therapy 2024Intraorbital dural arteriovenous fistula (IO-dAVF) is a rare condition, and treatment options vary from case to case. We report a case of transarterial embolization...
OBJECTIVE
Intraorbital dural arteriovenous fistula (IO-dAVF) is a rare condition, and treatment options vary from case to case. We report a case of transarterial embolization (TAE) for IO-dAVF.
CASE PRESENTATION
A 62-year-old male complained of gradually worsening pain, hyperemia, and visual impairment in the right eye. He did not exhibit diplopia or exophthalmos. Cerebral angiography revealed an arteriovenous fistula in the right orbit. The feeding arteries were the ophthalmic artery (OphA) and the artery of the superior orbital fissure (ASOF), with the superior ophthalmic vein (SOV) as the main draining vein. The venous pathway from the SOV was not clearly visible, and considering the risk of blindness with TAE from the OphA, TAE from the ASOF was performed. Onyx 18 was selected as the liquid embolic material and injected through a microcatheter placed in the internal maxillary artery. Occlusion up to the SOV was achieved, and the shunt flow completely disappeared. Normal blood flow in the OphA was maintained, hyperemia improved, and no complications were observed.
CONCLUSION
In cases of IO-dAVF, when transvenous embolization is difficult to perform, TAE using Onyx from the vessel of the external carotid artery system may be preferred over OphA.
PubMed: 38721617
DOI: 10.5797/jnet.cr.2023-0079