Late dental and bone alterations in patients after orbital rhabdomyosarcoma treatment.

PURPOSE: Orbital rhabdomyosarcoma is a rare soft tissue sarcoma in childhood but with a good prognosis. Treatment usually includes surgery, chemotherapy, and radiotherapy. This study aimed to evaluate long-term alterations in teeth and cranial bones in children, adolescents, and young adults after oncologic treatment for childhood orbital rhabdomyosarcoma. METHOD: This was a cross-sectional study that evaluated patients treated for orbital rhabdomyosarcoma between 1988 and 2011. Demographic, clinical, and treatment data were collected during the study period; also, panoramic radiographs, cephalometric study, and photographs of the face were taken. RESULTS: Eight long-term survivors were studied. Of those, 50% were male, 75% had less than 5 years of treatment, and 88% had only one of the orbits affected by the tumor. Regarding treatment, 50% received 50.4 Gy of radiotherapy in the orbit; the chemotherapy included vincristine, actinomycin D, and cyclophosphamide in 75% of the cases and also ifosfamide and etoposide in 25%. The children presented craniofacial alterations, mainly when radiotherapy occurred between 0 and 5 years old (p = 0.01). The mandibles also showed dental alterations, probably due to chemotherapy. CONCLUSION: In conclusion, orbital RMS patients treated with chemoradiotherapy, important dental, and facial bone alterations were found. The most significant were in the maxilla and close to the irradiation field. Dental and mandibular bone alterations were also found, indicating the probable chemotherapy action, as this region was not included in the irradiation field.

Ocular adnexal lymphoma - a retrospective study and review of the literature.

BACKGROUND: To review the characteristics of all Slovenian patients with ocular adnexal lymphoma (OAL) in the period of 24 years with the aim of evaluating demographic data, lymphoma location and type, disease stage, treatment modality, local control rate and survival rate. PATIENTS AND METHODS: All patients with histologically diagnosed OAL in the main tertiary centre of Slovenia, Eye Hospital, University Medical Centre Ljubljana, who were treated at Institute of Oncology Ljubljana were included in the study. Patients' data were collected from October 1995 through April 2019. RESULTS: Seventy-four patients were included in the study having a median age of 68 years at diagnosis. The majority of lymphomas were of B-cell origin (98.6%). The most frequent type was the extranodal marginal zone B-cell lymphoma (MALT) (71.6%). Orbital lymphomas were diagnosed in 56 cases (75.7%) and conjunctival in 18 cases (24.3%). Ocular manifestation was the first sign of the disease in 78.4% of patients and in 67.6% of patients ocular adnexa were the only disease location. Fifty-one patients (68.9%) were treated with radiotherapy, 7 patients (9.4%) with systemic treatment, 5 patients (6.8%) with combined radiotherapy and systemic treatment and in 11 patients, biopsy and active surveillance strategy was applied (14.9%). Local control of the disease was achieved in 96.6% of treated patients. Median overall survival of the whole study group has not been reached yet. Five-year overall survival rate was 80.1% (95% CI 68.1% - 88.5%) and 5-year lymphoma specific survival rate was 87.2% (95% CI 83.2%-91.2%). CONCLUSIONS: OALs comprise a group of heterogeneous diseases with variable outcomes depending predominately on the patient's age and lymphoma type, with low grade lymphomas carrying good prognosis even in elderly patients.

Case Report: Primary Squamous Cell Carcinoma of the Orbit in a Patient With Carney's Syndrome Treated With Multidisciplinary Approaches.

BACKGROUND: Squamous cell carcinoma (SCC) is a rare malignancy of invasive epithelium with keratinocyte differentiation, and it is the most common form of eyelid malignant neoplasm, comprising 5%-10% of malignancies. While SCC rarely affects the orbit, it may be involved through local invasion from a cutaneous primary site or extension by perineural invasion. Only 12 cases of primary orbital SCC have been reported until now. Here, we present a case of primary carcinoma of the right orbit with coexisting Carney's syndrome, a rare genetic disorder associated with multiple endocrine neoplasias (MEN) syndromes. CASE: A 62-year-old South Asian male presented with a painful swelling in the lateral aspect of the right eyebrow and protrusion of the eyeball in August 2020. He had a history of excision of Right atrial Myxoma in March 2020. Orbital computerized tomography (CT) and positron emission tomography (PET-CT) scans revealed an enhancing soft tissue lesion in the right orbit with the involvement of frontal and ethmoid sinuses. Biopsy confirmed HPV-related poorly differentiated SCC, positive for HPV-related markers. The patient received concurrent chemo irradiation with Cisplatin. Follow-up PET-CT done 3 months later showed a new lesion appeared in the right orbital region and right lobe of thyroid. Later had surgical excision and total thyroidectomy, and histopathological examination (HPE) from orbit was reported as invasive SCC and from the thyroid was reported as synchronous papillary thyroid cancer. The patient's proptosis resolved, and subsequent PET-CT and magnetic resonance imaging (MRI) scans did not show any residual or recurrent disease. CONCLUSION: Primary SCC of the orbit is an extremely rare disease, and this case report presents the 13th reported case and the first one associated with Carney's syndrome. As there is no standard treatment regimen for primary SCC of the orbit, this case highlights the use of multimodality treatment, including surgical excision and chemo irradiation. The findings emphasize the importance of early detection and management of this uncommon and life-threatening condition, providing hope for patients and aiding in the prevention of recurrence.

AB018. It's not always olfactory neuroblastoma-sinonasal undifferentiated carcinoma with frontal base and orbital involvement, a rare disease with poor prognosis: a case report.

BACKGROUND: Malignant neoplasms of the paranasal sinuses and nasal cavity are rare, comprising only 3% of all head and neck malignancies. Sinonasal undifferentiated carcinoma is a rare malignancy and an aggressive neoplasm that was clinic-pathologically distinct from other poorly differentiated malignancies of the nasal cavity and sinuses. The lesion is thought to originate from the epithelium, grows rapidly and invades nearby structures, often resulting in bony destruction while rapid progression of symptoms over weeks to months is characteristic. CASE DESCRIPTION: A 75-year-old man initially presented to ophthalmologist due to blurry vision and diplopia for 2 months. There was also progressive right eye swelling and pain for the past 12 months. Further image study revealed a right superomedial orbital mass with frontal/ethmoid sinus and frontal base extension, causing significant right eye gaze limitation, visual loss, ptosis, chemosis and exophthalmos. He was then transferred to neurosurgical clinic for further treatment where combined surgery was advocated to relieve the mass effect and also for pathology proof. Orbital roof/medial wall and frontal skull base were invaded and destroyed by the tumor. A bicoronal craniotomy was performed and extensive tumor removal was achieved except the orbital fossa due to difficult separation from the ocular muscle and optic nerve. Frontal base was reconstructed in a layered fashion to avoid cerebrospinal fluid (CSF) leakage and infection. He was sent to intensive care unit (ICU) for overnight observation where no further neurologic deterioration occurred. Instant orbital pain relief was noted with significantly decreased proptosis and improving visual acuity. Detailed pathological staining and molecular tests revealed a sinonasal undifferentiated carcinoma, adjuvant chemotherapy was arranged, followed by 30 cycles of radiotherapy. Slight disease progression was noted 12 months after the surgery, chemotherapy regimen was adjusted according to clinical response and he is still currently under regular adjuvant treatment. CONCLUSIONS: Sinonasal undifferentiated carcinoma typically carries a poor prognosis despite aggressive medical and surgical treatment, as it always presents at an advanced stage. Different combinations of chemotherapy, radiotherapy, radical surgical resection have been used to improve the outcome yet there is still not a universal treatment strategy. Early diagnosis, prompt treatment, and comprehensive medical care are crucial to achieve the best possible outcome for affected patients.

AB090. A rare case report: spheno-orbital meningioma with dural involvement.

BACKGROUND: Spheno-orbital meningiomas are rare, slow-growing tumors originating from the sphenoid ridge, causing proptosis and visual impairment. Surgical intervention can be complex due to the tumor's proximity to critical structures. CASE DESCRIPTION: A 67-year-old woman presented with a gradually enlarging protrusion of her left eye over three years. Referred to the neurosurgery clinic at Hasan Sadikin Hospital, Bandung, she underwent a craniectomy with concomitant cranioplasty to remove the tumor. The surgery included resection of tissue beneath the dura. Postoperatively, the patient's eye returned to its normal position, and her vision improved to 4/60. Spheno-orbital meningiomas, though primarily involving the sphenoid wing, can extend to surrounding tissues, complicating surgical resection. The patient's gradual proptosis over three years signifies the slow-growing nature of these tumors. Preoperative imaging and careful surgical planning are crucial for optimal outcomes. The craniectomy approach, combined with cranioplasty, allows for effective tumor removal and restoration of cranial aesthetics. The inclusion of subdural tissue resection addresses potential residual tumor cells, minimizing recurrence risk. Postoperative recovery in this case was favorable, with significant improvement in both ocular alignment and vision. However, the vision improved to 4/60 suggests some degree of irreversible optic nerve damage, which is a common challenge in these cases. Continuous monitoring and adjunct therapies may be necessary to manage any long-term sequelae. CONCLUSIONS: The patient's significant improvement in visual acuity and visual field following craniectomy and tumor removal demonstrates the potential for successful treatment of these conditions. Early detection and treatment are crucial in preventing long-term visual impairment and blindness.

23-year review of spheno-orbital meningioma: clinical, radiological, and pathological insights from 100 cases.

BACKGROUND: Spheno-orbital meningioma (SOM) represents a unique variant of sphenoid wing meningiomas, distinguished by its propensity for bone infiltration and cranio-orbital involvement. SOM exhibits a considerable incidence of misdiagnosis and recurrence. PURPOSES: To elucidate the clinical, radiological, and pathological characteristics of SOM. METHODS: Review of electronic medical records, histopathology, radiological images and follow-up information of 100 SOM patients. RESULTS: Of the 100 patients (28 males, 72 females) with SOM, mean age was 46.8 ± 12.6 years and prevalent symptoms were proptosis (99%). All the CT scans showed hyperostosis with 89.3% of the hyperostosis having an irregular edge. In MRI scans, dural tail sign was observed across all patients and the cranio-orbital tumors often penetrated temporal muscle (74.1%), extraocular muscle (74.1%) and lacrimal gland (63%). All the 100 patients underwent surgical intervention, and among them, 62 individuals received postoperative radiotherapy. Grade I resections had a lower recurrence rate(16.7%), which further decreased with the addition of radiotherapy(13.9%). In contrast, all patients with grade II or higher grade resections without radiotherapy experienced recurrence, indicating a higher risk associated with less complete tumor removal. The pathological examination revealed that intraorbital sections exhibited comparable tumor density to intraorbital SOM tumors, along with increased fibrous density but decreased vascular distribution. CONCLUSIONS: Radiological characteristics of SOM included cranio-orbital tumors, hyperostosis of the sphenoid wing with an irregular edge, and dural tail sign. Combination of gross total resection and adjuvant radiotherapy was recommended to minimize recurrence rate. Intracranial SOM tumors tended to be softer and more bleed-prone than intraorbital sections, necessitating surgical precision.

Frontotemporal Approach for Spheno-Orbital Meningioma and Orbital Compartment Resection: Technical Case Instruction: 2-Dimensional Operative Video.

BACKGROUND AND IMPORTANCE: Spheno-orbital meningiomas (SOMs) pose a challenge to the skull base neurosurgeon because of their variable presentation and involvement of critical structures within the orbit. There is no consensus on optimal management of these patients and how to achieve maximal safe resection. The authors share an illustrative case with an accompanying video to demonstrate their aggressive approach to resect SOMs and their intraorbital components. CLINICAL PRESENTATION: A 75-year-old-woman presented with progressive vision loss and proptosis. Magnetic resonance imaging was consistent with a large, left-sided sphenoid wing meningioma with extension to the orbital wall and compression of the optic nerve medially. The patient elected to undergo surgical excision and optic nerve decompression. She did well postoperatively with resolution of proptosis and good resection margins on follow-up imaging. CONCLUSION: Aggressive resection of SOMs is possible with an understanding of the underlying anatomy. Familiarity with the orbit can facilitate a maximal safe resection with optic nerve decompression.

Extraconal orbital craniopharyngioma.

A female, in her 60s, presented with pain and swelling of the right eye for 3 years. The radiological work-up revealed an extraconal solid-cystic orbital tumour suggestive of an epidermoid cyst. The patient underwent supraorbital craniotomy with a gross total excision of the tumour. An intraoperative diagnosis was sought, which on both squash smear and frozen section showed features of craniopharyngioma (CP), later confirmed on paraffin sections and immunohistochemistry. The orbit is a very rare site for ectopic CP, with only two cases reported in the literature. Many theories have been proposed to explain the occurrence of CP at ectopic sites. This report aims to provide insight into the different hypotheses of the pathogenesis of ectopic CP through a review of the literature.

Modified Orbitozygomatic Craniotomy Approach for a Recurrent Orbital Tumor in a Pediatric Patient.

Background: This report aims to present the case of a pediatric patient with a recurrent tumor in the superolateral orbit. Clinical Presentation: An 8-year-old patient was initially treated for a tumor in the superolateral orbit via a transconjunctival approach. The histopathological diagnosis was epidermoid cyst. Postoperatively, chronic inflammation and fistula developed in the lateral canthus area. Magnetic resonance imaging revealed a residual tumor posterior to the original tumor location. The patient was treated via a modified orbitozygomatic (mOZ) craniotomy approach that was originally applied in neurosurgery, and complete tumor removal was achieved. A temporary paralysis of the frontotemporal branch of the facial nerve was observed and fully resolved within one month following surgery. At the 18th month of follow-up, the visual, neurological, and cosmetic results were found to be satisfactory. Conclusions: mOZ craniotomy can be used to access and operate on recurrent orbital tumors in pediatric patients where other more aggressive surgical approaches should be avoided.

A Novel Melanin-Targeted 18F-PFPN Positron Emission Tomography Imaging for Diagnosing Ocular and Orbital Melanoma.

OBJECTIVE: 18F-N-(2-(Diethylamino)ethyl)-5-(2-(2-(2-fluoroethoxy)ethoxy)ethoxy) picolinamide (18F-PFPN) is a novel positron emission tomography (PET) probe designed to specifically targets melanin. This study aimed to evaluate the diagnostic feasibility of 18F-PFPN in patients with ocular or orbital melanoma. MATERIALS AND METHODS: Three patients with pathologically confirmed ocular or orbital melanoma (one male, two females; age 41-59 years) were retrospectively reviewed. Each patient underwent comprehensive 18F-PFPN and 18F-fluorodeoxyglucose (18F-FDG) PET scans. The maximum standardized uptake value (SUVmax) of the lesion and the interference caused by background tissue were compared between 18F-PFPN and 18F-FDG PET imaging. In addition, the effect of intrinsic pigments in the uvea and retina on the interpretation of the results was examined. The contralateral non-tumorous eye of each patient served as a control. RESULTS: All primary tumors (3/3) were detected using 18F-PFPN PET, while only two primary tumors were detected using 18F-FDG PET. Within each lesion, the SUVmax of 18F-PFPN was 2.6 to 8.3 times higher than that of 18F-FDG. Regarding the quality of PET imaging, the physiological uptake of 18F-FDG PET in the brain and periocular tissues limited the imaging of tumors. However, 18F-PFPN PET minimized this interference. Notably, intrinsic pigments in the uvea and retina did not cause abnormal concentrations of 18F-PFPN, as no anomalous uptake of 18F-PFPN was detected in the healthy contralateral eyes. CONCLUSION: Compared to 18F-FDG, 18F-PFPN demonstrated higher detection rates for ocular and orbital melanomas with minimal interference from surrounding tissues. This suggests that 18F-PFPN could be a promising clinical diagnostic tool for distinguishing malignant melanoma from benign pigmentation in ocular and orbital melanomas.

Rituximab-induced leukocytoclastic vasculitis in a patient with low-grade orbital B-cell lymphoma: a case report.

Rituximab is an anti-CD20 chimeric murine/human mAb mainly used to treat certain types of lymphoproliferative malignancies and autoimmune diseases. Although it has been used in the treatment of vasculitis in recent years, it rarely triggers severe vascular skin reactions such as leukocytoclastic vasculitis (LCV). Physicians should be aware of this rare adverse event that requires discontinuation of rituximab, which can occur days or even weeks after rituximab treatment. Here, we report a case of LCV observed in a patient with low-grade orbital B-cell lymphoma treated with weekly rituximab and local radiotherapy. In our case, discontinuation of rituximab and initiation of oral methylprednisolone therapy were sufficient to achieve complete resolution of the LCV.

Response-Adapted Ultralow-Dose Radiation Therapy for Orbital Indolent B-Cell Lymphoma: A Phase 2 Nonrandomized Controlled Trial.

Importance: Radiation therapy to doses of 24 to 36 Gy is currently used to treat indolent B-cell lymphoma of the ocular adnexa; however, ocular adverse effects are common. Objective: To determine if a response-adapted radiation therapy strategy will result in excellent disease outcomes while reducing orbital morbidity. Design, Setting, and Participants: This single-institution, phase 2 prospective nonrandomized controlled trial of a response-adapted strategy involved 50 evaluable patients with stage I to IV indolent B-cell lymphoma of the ocular adnexa enrolled between July 2015 and January 2021. This treatment approach was also retrospectively evaluated with a separate 55-patient cohort treated between March 2013 and October 2021. All data were analyzed between November 2021 and December 2023. Interventions: Patients were treated with ultralow-dose radiation therapy to 4 Gy in 2 fractions and assessed for response at 3-month intervals. Patients with persistent orbital lymphoma were offered an additional 20 Gy in 10 fractions to complete the response-adapted treatment. Main Outcome and Measures: The primary end point was 2-year local orbital control within the irradiated field after response-adapted therapy. Secondary end points included overall survival and complete response rate. Results: The 50 prospective patients were a median (range) of 63 (29-88) years old, and 31 (62%) were female. Among the 50 patients, 32 (64%) had mucosa-associated lymphoid tissue lymphoma, 12 (24%) had follicular lymphoma, and 6 (12%) had unclassifiable low-grade B-cell lymphoma. Thirty-one patients (62%) had stage I disease, and 36 (72%) were newly diagnosed. At a median follow-up of 37.4 (95% CI, 33.7-52.5) months, the 2-year local control rate was 89.4% (95% CI, 81.0%-98.7%), and the 2-year overall survival rate was 98.0% (95% CI, 94.1%-100%); 45 patients (90.0%; 95% CI, 78.2%-96.7%) experienced a complete response to response-adapted radiation, including 44 patients with a complete response to ultralow-dose radiation and 1 patient with a complete response after an additional 20 Gy. No local recurrences were observed among patients with a complete response to response-adapted therapy. No grade 3 or higher toxic effects were observed. In a planned subset analysis of 22 patients with newly diagnosed, untreated stage I mucosa-associated lymphoid tissue lymphoma, the 2-year local control rate was 90.7% (95% CI, 79.2%-100%), and the 2-year freedom from distant relapse rate was 95.2% (95% CI, 86.6%-100%). Conclusion and Relevance: In this nonrandomized controlled trial, response-adapted ultralow-dose therapy for indolent orbital B-cell lymphoma resulted in reduced radiation exposure, negligible toxic effects, and excellent disease outcomes. Trial Registration: ClinicalTrials.gov Identifier: NCT02494700.

Double trouble: orbital rhabdomyoma with trichinellosis.

Rhabdomyoma of the orbit is a rare tumor with very few cases reported in the literature. We herein describe a 5-year-old boy who presented to us with a deviation of his left eye. Magnetic Resonance Imaging (MRI) showed a well-defined homogeneous intraconal mass in the superomedial aspect compressing the optic nerve. An excision biopsy was performed and the diagnosis of rhabdomyoma was confirmed on histopathology and immunohistochemistry with a coincidental finding of Trichinella spiralis larvae within the excised specimen. We report this phenomenon in two rare diseases with a predilection for striated muscle occurring simultaneously in a single patient.

Endoscopic-assisted transorbital extended orbital exenteration: A multi-institutional preclinical study.

BACKGROUND: Sinonasal malignancies with orbital invasion have dismal prognosis even when treated with orbital exenteration (OE). Sugawara et al. developed a surgical strategy called "extended-OE (EOE)," showing encouraging outcomes. We hypothesized that a similar resection is achievable under endoscopic guidance through the exenterated orbit (endoscopic-EOE). METHODS: The study was conducted in three institutions: University of Vienna; Mayo Clinic; University of Insubria; 48 orbital dissections were performed. A questionnaire was developed to evaluate feasibility and safety of each step, scoring from 1 to 10, ("impossible" to "easy," and "high risk" to "low risk," respectively), most likely complication(s) were hypothesized. RESULTS: The step-by-step technique is thoroughly described. The questionnaire was answered by 25 anterior skull base surgeons from six countries. Mean, median, range, and interquartile range of both feasibility and safety scores are reported. CONCLUSIONS: Endoscopic-EOE is a challenging but feasible procedure. Clinical validation is required to assess real-life outcomes.

Endoscopic resection of primary sinonasal mucosal melanoma with orbital invasion: How I do it.

Primary sinonasal mucosal melanoma is a rare aggressive malignancy. In this video, a case of a 68-year-old female who presented with diplopia for 2 weeks is described. The present video reports the endoscopic endonasal surgical excision of a primary sinonasal mucosal melanoma. The video contains patient's medical history, preoperative radiological evaluations and step-by-step description of surgical steps of the procedure with the utilization of computer-assisted navigation system.

Gamma knife radiosurgery for orbital cavernous hemangioma: a systematic review and single-arm meta-analysis.

PURPOSE: Gamma knife radiosurgery (GKRS) for orbital cavernous hemangioma (OCH) has emerged as a promising method due to its significant clinical improvement and low incidence of complications. This study aimed to evaluate the safety and efficacy of GKRS for the treatment of OCH. METHODS: In accordance with the PRISMA framework, we searched PubMed, Cochrane Central, and Embase for studies reporting outcomes of GKRS for OCH. Studies reporting complications, visual improvement, proptosis, tumor reduction rate, and tumor progression rate for OCH following GKRS were included. RESULTS: Six studies, out of 1856 search results, with 100 patients were included. Among them, only 5 minor complications were related to GKRS, including 3 with orbital pain and 2 with periorbital chemosis. Thus, the complication rate was 13% (95% CI, 7-25%). Visual acuity and visual field improvement rates after GKRS were 80% (95% CI, 63-96%) and 71% (95% CI, 47-95%) respectively. Proptosis improved in 94% of cases (95% CI, 83-100%). The tumor reduction rate was 77% after GKRS (95% CI, 69-85%). CONCLUSION: GKRS for OCH appears to be a safe technique, as evidenced by the rate of clinical improvement and radiological improvement. However, studies are limited by an absence of a control group. Additional studies are needed to evaluate the relative efficacy of GKRS as compared with alternative surgical modalities for OCH.