Solitary fibrous tumor of the orbit in a 1-year-old patient: a case report.

Solitary fibrous tumors (SFTs) are rare neoplasms of mesenchymal origin. While the mean age of presentation is 58 years old, we report the case of the youngest documented patient with an SFT of the orbit. A 13-month-old child was evaluated for eyelid asymmetry and then referred to the oculoplastic service. On examination, a soft tissue mass of the right inferomedial orbit was observed. MRI demonstrated a well-circumscribed, extraocular lesion in the inferomedial right orbit, potentially fibrous in nature. Excision was performed without complications. Pathologic examination demonstrated fibrous tissue proliferation with a staghorn vascular pattern, as well as benign fibrous cells with tapering nuclei and abundant pericellular reticulin. Immunohistochemistry (IHC) demonstrated that the cells stain diffusely positive for CD34 and vimentin. With the MRI findings, pathology, and IHC, the diagnosis was confirmed to be SFT. SFTs of the orbit, although rare, may occur in the pediatric population.

Angiolymphoid Hyperplasia of the Orbit: A Rare Case Report and Review of the Literature.

Background: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare vascular tumour of the skin which mainly involves the head and neck regions. Case Report: A 41 year old male presented to the ENT outpatient department with a swelling in the anterosuperomedial aspect of right orbital rim since two year. Following a contrast enhanced CT of the Head, complete surgical excision was done and diagnosis of ALHE was confirmed by histopathology. Methodology: We performed a systematic review of the literature following the preferred reporting items for Systematic reviews. Literature searches were conducted in web based search engines using MeSH terms and key words. We found seven publications that fit the inclusion criteria which included case reports and case series. Conclusion: ALHE involving the orbit has been very rarely reported and is a challenging diagnosis. Complete surgical excision is the treatment of choice and recurrence can occur with incomplete removal.

Unilateral proptosis as a neglected case of sphenoid wing meningioma: a case report and healthcare challenges in rural Indonesia.

Proptosis, an abnormal protrusion of the eyeball, is a manifestation of a wide variety of pathologies. The complication to be vision- or life-threatening makes early diagnosis is important, especially in rural primary health centers (PHCs) with far-distance referrals to capable hospitals. This case report examines a patient with obvious unilateral proptosis and blurry vision on the right eye for 4 years, with neglect because of prior inadequate diagnosis and explanation before the current complication. Examination shows no light perception, obvious proptosis (30 mm), exodeviation, and ophthalmoplegia. Referral for radiological examination showed a regular extra-axial lesion, well-defined, and broad-based on the right sphenoid wing with hyperostosis. The patient was diagnosed with sphenoid wing meningioma, which complicated into proptosis and blindness. This report aims to explain the current challenges of rural PHCs in Indonesia critically and to emphasize that rural PHCs should overcome the low level of public education, self-awareness of health, and tendency to reject the referral process. Clinicians also have a crucial role in early detection and prompt treatment to reduce further neglected cases.

Orbital dermoid cyst.

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.

Orbital malignant schwannoma and embryonal rhabdomyosarcoma in rats caused by leakage of locally injected nickel subsulfide to the orbit.

Nickel subsulfide (Ni3S2) is known to induce intraocular neoplasms when injected intravitreally into the eyes of rats. Here, we found two extraocular orbital neoplasms in two different rat strains, presumably due to the leakage of locally injected Ni3S2 to the extraocular orbital tissues. In the F344/DuCrlCrlj rat, an orbital mass arose at 30 weeks after injection, and invaded into the cranium. Histologically, the orbital mass was composed of areas arranged in parallel bundles formed by densely packed elongated or spindle-shaped cells with indistinct cytoplasmic borders, and of areas of hypocellular arrangement consisting of round cells in eosinophilic myxoid-like substances. Metastases were observed in the right submandibular and cervical lymph nodes. The neoplastic cells were immunopositive for S-100 protein and vimentin. Transmission electron microscopy revealed that the neoplastic cells had cellular processes and pericytoplasmic basal laminae. In the RccHanTM:WIST rat, an orbital mass arose at 36 weeks after injection. Histologically, the mass consisted of rhabdoid-like large round cells with proliferation of small round-to-polygonal cells, and these neoplastic cells infiltrated into the extraocular muscles. Immunohistochemically, the neoplastic cells were positive for desmin and vimentin. Transmission electron microscopy detected immature myofibrils with Z-band structures in the cytoplasm of these neoplastic cells. Consequently, the tumors were diagnosed as an orbital malignant schwannoma in an F344/DuCrlCrlj rat and an orbital embryonal rhabdomyosarcoma in a RccHanTM:WIST rat. The results of this case report suggest that leakage of Ni3S2 to the orbit caused the induction of orbital malignant schwannoma or rhabdomyosarcoma in rats.

Immunoglobulin G4-related lymph node disease with an orbital mass mimicking Castleman disease: A case report.

BACKGROUND: Immunoglobulin (Ig) G4-associated diseases are a group of systemic diseases involving multiple organs and are also known as IgG4-associated sclerosing diseases. IgG4-associated lymphadenopathy occurring in the lymph nodes is characterized by a lack of specificity due to its clinicopathological characteristics and must be differentiated from a variety of lesions, such as Castleman disease, lymphatic follicular reactive hyperplasia, and lymphoma. CASE SUMMARY: A 65-year-old male patient, with Guillain-Barre syndrome for 5 years, presented to our hospital complaining of bilateral orbital mass for 2 years. After hospitalization, the results of the patient's laboratory tests showed that immunoglobulin subgroup IgG4 was 33.90 g/L and IgG was 30.30 g/L, but serum interleukin-6 was normal. The pathological morphology of orbital mass and cervical lymph node were consistent, which showed that a large number of plasma cells and eosinophils were observed in the lymphatic follicles, and the interstitial fibrous tissue was proliferative. Immunohistochemistry showed that CD20 (B cells) (+), CD3 (T cells) (+), CD38 (+), IgG (+), IgG4 positive cells > 100/high powered field, and IgG4/IgG > 40%. Combined with clinical and immunohistochemical results, lymphadenopathy was consistent with Castleman disease-like IgG4-associated sclerosing disease. Prednisone acetate treatment was given at 40 mg/d. After 2 wk, the superficial lymph nodes and orbital masses shrank, and the IgG4 level decreased. As prednisone acetate was regularly used at a reduced dosage, no recurrence of the disease has been observed. CONCLUSION: This case suggested that it is necessary to proceed cautiously in clinical practice with such patients, and immunoglobulin, complement, interleukin-6, C-reactive protein, and other examinations should be performed to confirm the diagnosis.

Hypodiploid B-Lymphoblastic Leukemia Presenting as an Isolated Orbital Mass Prior to Systemic Involvement: A Case Report and Review of the Literature.

We describe a 4-year-old boy who presented with progressive right periorbital edema and proptosis, with no systemic symptoms, who was found to have B-lymphoblastic leukemia (B-ALL). Magnetic resonance imaging (MRI) showed an enhancing mass centered in the right superolateral extraconal orbit. Orbital biopsy was consistent with B-ALL (CD99, TdT, LCA cocktail, CD34, CD79, CD10, PAX5, MIB1 positive; CD3, CD20 negative). A subsequent bone marrow aspirate confirmed a diagnosis of B-ALL with 80% blasts by flow cytometry and haploid cytogenetic findings. The patient improved clinically after chemotherapy. There are seven cases previously reported in the literature with hematogenous orbital masses at initial presentation of childhood ALL, but all with systemic symptoms or an abnormal complete blood count (CBC) at presentation. Our case is the first report in which an orbital mass preceded detectable systemic or laboratory evidence of ALL. This patient highlights the importance of differentiating benign causes of eyelid swelling from malignant ones.

Orbital Schwannoma.

Orbital schwannomas are rare neoplasms of the orbit. The presenting symptoms are often nonspecific. Classic imaging characteristics seen on magnetic resonance imaging (MRI) and orbital ultrasound can be useful to help aid in the diagnosis of orbital schwannoma. When diagnosed, the goal of treatment is complete surgical excision. The location of the tumor within the orbit dictates which surgical approach would provide the best exposure. When complete excision is achieved, recurrence rates are very low. This article addresses the etiology, patient population, presentation, natural history, and differential diagnosis of orbital schwannomas. Imaging characteristics and histopathologic subtypes are reviewed. Treatment goals, approaches, and specialties involved in the management of these patients is discussed. Finally, a representative case is presented.

Improving diagnostic performance of differentiating ocular adnexal lymphoma and idiopathic orbital inflammation using intravoxel incoherent motion diffusion-weighted MRI.

PURPOSE: To investigate the utility of intravoxel incoherent motion diffusion-weighted MRI (IVIM-DWI) derived diffusion and perfusion parameters in differentiating ocular adnexal lymphoma (OAL) from idiopathic orbital inflammation (IOI), and to assess whether IVIM-DWI provides improved diagnostic performance for the distinction. METHOD: Twenty-one patients with OAL and 24 patients with IOI underwent IVIM-DWI. Apparent diffusion coefficient (ADC) and IVIM-DWI parameters including true diffusion coefficient (D), pseudo-diffusion coefficient (D*), and perfusion fraction (f) were measured in lesions by two independent radiologists. The MRI parameter differences between OAL and IOI were tested using two-sample t-test. The receiver operating characteristic (ROC) analysis curves were used to determine the diagnostic performance of significant parameters for differentiation between OAL and IOI. RESULTS: The ADC, D, and f were lower in OAL than those in IOI (ADC = 0.78 ± 0.12 vs. 0.99 ± 0.16 × 10-3 mm2/s, P < 0.001; D = 0.34 ± 0.15 vs. 0.76 ± 0.25 × 10-3 mm2/s, P < 0.001; f = 0.31 ± 0.06 vs. 0.41 ± 0.08 × 100 %, P < 0.001). There was no significant difference in D* between OAL and IOI (P = 0.235). The optimal cut-off values of ADC, D, and f in differentiating OAL from IOI were 0.83 × 10-3 mm2/s, 0.56 × 10-3 mm2/s, and 0.36 × 100 %, respectively. No significant differences were found in areas under the curve (AUCs) among ADC, D and f (all P > 0.05). The combination of D and f provided significantly higher AUC than ADC (AUC = 0.984 vs. 0.838, Z = 2.128, P = 0.033), and had higher sensitivity of 95.24 %, specificity of 95.83 %, and accuracy of 95.56 %. CONCLUSIONS: IVIM-DWI is valuable in differentiating OAL from IOI, and D combined f can improve the performance of differential diagnosis.

Solitary neurofibroma of the orbit with intracerebral extension associated with ocular surface melanocytoma: a case report.

Solitary or isolated neurofibroma is uncommonly observed in the orbit. Neurofibromas typically involve peripheral nerves and occasionally the cranial nerves. A 29-year-old man presented with recent onset left eye proptosis and exotropia. Physical examination was positive for hyperpigmented lesions of the ipsilateral ocular surface and hard palate. Imaging revealed an infiltrative orbital mass with extension through superior orbital fissure into the brain. There was also bone defect of greater sphenoid wing. Medial orbitotomy was performed to obtain biopsies of the orbital mass and the pigmented ocular surface lesions. Histopathologic diagnosis of neurofibroma was confirmed for the former and melanocytoma for the latter. His symptoms and examinations remained stable during the follow up. This case is unique due to several features, including extensive intracerebral spread of orbital neurofibroma in a patient without definite diagnosis of neurofibromatosis type 1 and association with ipsilateral ocular surface melanocytoma and palatal pigmented lesions. ABBREVIATIONS: CT: computed tomography; GFAP: glial fibrillary acid protein; MRI: magnetic resonance imaging; NF-1: neurofibromatosis type 1.

Orbital metastases from neuroendocrine neoplasms: clinical implications and outcomes.

PURPOSE: Neuroendocrine neoplasms (NENs) may rarely metastasise to the orbit. Published data on epidemiology, incidence and preferred treatment is limited. We present the largest cohort of symptomatic and asymptomatic NEN patients with orbital metastases and data on epidemiological parameters, symptoms as well as diagnostic/treatment modalities used. METHODS: We identified patients from our internal NEN database of patients who had also undergone Gallium68-DOTATATE PET (Ga68-DOTA). The diagnosis of orbital metastatic NEN was made on somatostatin receptor imaging and confirmed on a dedicated MRI of orbits. RESULTS: We identified 27 patients of 994 patients evaluated with Ga68-DOTATATE PET imaging during their surveillance monitoring in our department; 15 female, average age at NEN diagnosis 53 years and orbital metastatic NEN diagnosis 59 years. The majority of NEN primaries originated from small bowel (18/27, 66.4%) or pancreas 4/27 (4/27, 14.8%). Hepatic with or without concomitant skeletal metastases were present in 23/27 (85%) of patients. Ocular symptoms and/or signs were evident in 11/27 (41%) of patients. 5/11 symptomatic patients underwent external beam radiotherapy (EBRT) resulting in complete symptoms resolution. The 5-year survival was estimated at 84.1%. CONCLUSIONS: Orbital metastases of NEN have a relatively low prevalence, more commonly associated with small bowel primary. Extraocular muscles are primarily affected, irrespectively of liver disease burden. Survival does not seem to be affected. EBRT is an efficacious treatment modality for both symptom relief and tumour growth control. Administration of peptide receptor radionuclide therapy may occasionally induce temporary ocular symptoms, which resolve following treatment with a short course of steroids.

Mid-Gut Carcinoid Tumour Presenting as Suspected Orbital Metastasis.

Although metastasis of carcinoid tumours of the intestine is rare, it has been reported in several organs, mainly in the lungs, the liver and less commonly in the orbits. We report a 50-year-old male patient who presented at Hamad General Hospital, Doha, Qatar, in 2016 with central abdominal pain, distention, nausea and vomiting for the previous four days. The patient had unilateral right-sided exophthalmos for two years prior to presentation. Following an abdominal computed tomography (CT) scan and an ultrasound guided biopsy, the patient was diagnosed with extensive multi-focal metastatic carcinoid tumour of the small bowel and mesentery; histopathology confirmed the diagnosis. Subsequently, the patient underwent a laparotomy and small bowel resection and was administered somatostatin therapy. One week postoperatively, the patient developed an acute increase in his right eye exophthalmos. CT, magnetic resonance imaging and scintigraphy scans revealed an orbital metastatic lesion, which probably originated from the previously diagnosed carcinoid tumour. The orbital metastasis was treated with somatostatin therapy and the patient was lost to follow-up when he left the country.

Histiocitosis de células de Langerhans en su presentación focalizada en órbita. Revisión del tema a propósito de un caso / Langerhans cell histiocytosis in their orbitfocused presentation. Review of the topic regarding a case

Resumen Objetivo: Describir un caso de histocitosis de células de Langerhans en un paciente pediátrico. Método: Paciente de 4 años con proptosis, diplopía, fiebre y pérdida de agudeza visual, con imagen en tomografía computarizada evidente de masa orbitaria con erosión ósea. Se realizó biopsia excisional por craneotomía coronal, el resultado histopatológico fue histiocitosis de células de Langerhans. Resultados: El estudio anatomopatológico transoperatorio mostró una neoplasia de células pequeñas redondas y azules, compatible con rabdomiosarcoma. Las pruebas de inmunohistoquímica concluyeron el diagnóstico de histiocitosis de células de Langerhans. Conclusión: La histiocitosis de células de Langerhans es una enfermedad poco frecuente que requiere de una sospecha clínica y diagnóstico oportuno, adecuar el tratamiento y mejorar la sobrevida de los pacientes.

Abstract Objective: To describe a case of Langerhans cell histiocytosis in a pediatric patient. Method: A 4-year-old patient with proptosis, diplopia, fever and loss of visual acuity, with evident image in a computed tomography of orbital mass with bone erosion. An excisional biopsy was performed by coronal craniotomy. The histopathological result was Langerhans cell histiocytosis. Results: The anatomopathological study showed a neoplasm of small round and blue cells, compatible with Rhabdomyosarcoma. Immunohistochemical tests concluded the diagnosis of Langerhans cell histiocytosis. Conclusion: Langerhans cell histiocytosis is a rare disease that requires clinical suspicion and a timely diagnosis to adjust the treatment and improve the survival of patients.

A Rare Case of Congenital Orbital Cystic Neuroblastoma in an Infant.

Neuroblastoma, a relatively common tumor in infants, is rarely present at birth. A solid mass is the typical appearance of this neoplasm, while cystic formation is uncommon. Congenital cystic neuroblastoma is extremely rare and no previous report exists of its occurrence in the orbit. A newborn girl was referred with an extremely large mass on the right side of her face on the third day of her life. Prenatal ultrasonography had revealed a large mass (80 × 80 mm) in the frontal area. MRI revealed a heterogeneous mass composed of cystic and solid, contrast-enhancing components. Excisional biopsy was successfully performed and histopathologic diagnosis of neuroblastoma was confirmed. Systemic workup was negative for involvement of other organs and levels of catecholamine metabolites of serum and urine (homovanillic acid and vanillylmandelic acid) were normal. Postoperative course was uneventful with complete healing of the anophthalmic socket and normal physical and mental growth and development of the infant. To our knowledge, this is the first report of congenital cystic neuroblastoma primarily arising in the orbit.

Successful Total Resection of an Orbital Epithelioid Hemangioendothelioma with the Aid of Endovascular Embolization.

Hemangioendothelioma is rarely encountered in the orbit. We present a patient with a growing orbital mass for whom surgical excision was planned. Two previous attempts at removing the mass failed due to profuse bleeding. Endovascular embolization was performed before surgery to prevent massive hemorrhage. After embolization, retinal vascular accident (combined central retinal artery and vein occlusion) occurred. However, surgical excision (lateral orbitotomy and transcoronal craniotomy) was successful, and the vision improved postoperatively. Histopathologic examination and immunohistochemistry study confirmed the diagnosis of epithelioid hemangioendothelioma. The combined approach by a team of specialists, including an interventional radiologist performing preoperative embolization of the feeding vessel and joint surgery by a neurosurgeon and oculoplastic surgeon, was the key to the effective treatment of this vascular orbital neoplasm.

Unusual occurrence of orbital hemangiopericytoma in the zygomatic bone of an adolescent: a case report.

BACKGROUND: Hemangiopericytoma and solitary fibrous tumor are considered related variants on the same spectrum and both may essentially be the same tumor. They are infrequently encountered in the orbital region while the zygomatic bone is an extremely rare location for these neoplasms to occur. CASE PRESENTATION: A 14-year-old boy presented with complaint of deformity of left infraorbital area and a firm, regular mass in the region. Orbital CT scan revealed a well-defined round isodense intraosseous lesion in the lowermost portion of the lateral orbital wall (zygomatic bone), expanding the bone and protruding anteriorly and medially. MRI showed the mass to be heterogenous and strongly enhancing with contrast medium. Inferior transconjunctival orbitotomy was performed and the mass was removed. The histopathologic examination and immunohistochemistry staining results (positive for CD34, CD31 and smooth muscle actin, but negative for CD99, S100, B-cell lymphoma 2 (bcl-2) and desmin) confirmed the diagnosis of hemangiopericytoma. The postoperative course was uneventful, with no evidence of recurrence after 5 years follow up. CONCLUSIONS: This case represents the second hemangiopericytoma reported in the zygomatic bone. Although extremely rare, hemangiopericytoma/solitary fibrous tumor might be considered in the differential diagnosis of intraosseous lesions of the orbital and zygomatic region.

Isolated orbital mucoceles in the absence of obstructive sinus disease.

Orbital surgeons are quite familiar with the diagnosis and management of paranasal sinus mucoceles due their frequent involvement of the orbit. These benign masses form and expand following sinus outflow obstruction secondary to various causes including trauma, inflammation and malignancy, amongst others. The authors present two cases of isolated orbital mucoceles without associated sinus outflow obstruction. There were notably no connections between the paranasal sinuses and the lesions. The mucoceles were diagnosed following orbitotomies and excisional biopsies with confirmatory histopathology. These rare orbital lesions should be considered when evaluating cystic orbital lesions, and the orbital surgeon should be familiar with the proposed pathophysiology and treatment recommendations.

Squamous cell carcinoma of the conjunctiva with extraocular involvement: case report and literature review. / Carcinoma escamoso de la conjuntiva con compromiso extraocular: reporte de caso y revisión de la literatura.

Squamous cell carcinoma of the conjunctiva is the most frequent malignant tumor of the ocular surface. It is a rare disease with an incidence of 0.13 to 1.9 per 100,000 inhabitants, mainly affecting individuals between 50 and 75 years of age. It tends to have a slow, non-aggressive course. Treatment depends on the extent of the tumor. If there is intraocular involvement, enucleation is indicated, and in the presence of extraocular involvement the orbital exenteration is the standard treatment. We report and discuss the case of an 82 year old male patient having a conjunctival squamous cell carcinoma with intra and extraocular involvement, together with a review of the literature.

El carcinoma escamoso de la conjuntiva es el tumor maligno más frecuente de la superficie ocular. Constituye una enfermedad rara con una incidencia de 0,13 a 1,9 por 100 000 habitantes que afecta principalmente a individuos entre los 50 y los 75 años. Suele tener un curso lento y poco agresivo. El tratamiento depende de la extensión tumoral. En presencia de compromiso intraocular la enucleación está indicada y en presencia de compromiso extraocular la exanteración orbitaria es el tratamiento estándar. Reportamos el caso de un paciente de 82 años con carcinoma escamoso conjuntival con compromiso intra y extraocular, se discute el caso y se revisa la literatura.

Rehabilitation of orbital cavity after orbital exenteration using polymethyl methacrylate orbital prosthesis.

Squamous cell carcinoma of the eyelid is the second most common malignant neoplasm of the eye with the incidence of 0.09 and 2.42 cases/100 000 people. Orbital invasion is a rare complication but, if recognized early, can be treated effectively with exenteration. Although with advancements in technology such as computer-aided design and computer-aided manufacturing, material science, and retentive methods like implants, orbital prosthesis with stock ocular prosthesis made of methyl methacrylate retained by anatomic undercuts is quiet effective and should not be overlooked and forgotten. This clinical report describes prosthetic rehabilitation of two male patients with polymethyl methacrylate resin orbital prosthesis after orbital exenteration, for squamous cell carcinoma of the upper eyelid. The orbital prosthesis was sufficiently retained by hard and soft tissue undercuts without any complications. The patients using the prosthesis are quite satisfied with the cosmetic results and felt comfortable attending the social events.

Lymphoid hyperplasia of the orbit and ocular adnexa: A clinical ​pathologic review.

Lymphoid hyperplasia (LH) is a benign lymphoproliferative disorder that, in a minority of cases, may be associated with concurrent or metachronous non-Hodgkin lymphoma. LH cases are further subdivided into "reactive" and "atypical" categories based on the presence or absence of unequivocal malignant features. With improving molecular diagnostic technologies, "reactive" LH is by far the most common category of LH, with atypical LH accounting for only a small minority of specimens. Similarly, lesions previously diagnosed as LH are now being revised as low-grade B-cell non-Hodgkin lymphoma or diagnosed as newly described benign conditions such as IgG4-related disease. Additional differential diagnoses include specific and nonspecific orbital inflammations, infiltrative processes, and depositions. Hence, there are emerging changes in the patterns and proportions of entities that fall within the spectrum of lymphoproliferative disorders of the orbit and ocular adnexa. Reactive LH and low-grade malignant lymphoproliferative disorders in the orbit and ocular adnexa are clinically and radiologically indistinguishable from each other, requiring tissue biopsy in all cases. The prognosis of ocular adnexal LH is generally favorable, but the small risk of non-Hodgkin lymphoma mandates follow-up for at least 5 years. We summarize the current state of knowledge on LH occurring in the orbit and ocular adnexa.