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Grand Rounds An Incidental Orbital Mass

Learn about a case study of a 42-year-old female with an orbital mass, its diagnosis, treatment options, and prognosis. This article discusses the clinical presentation, imaging findings, World Health Organization classification, treatment approaches, and recurrence rates. Gain insights into managing orbital tumors effectively.

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Grand Rounds An Incidental Orbital Mass

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  1. Grand RoundsAn Incidental Orbital Mass Josh Gross, MD December 15, 2017

  2. Patient Presentation CC Worsening blurry vision for 8 months HPI 42 yoWF in motorcycle crash, with incidental left orbital lesion found on trauma CT scan of the brain. C/o blurry vision OU, intermittent edema of left upper eyelid, headaches, and burning pain in the left side of her face with tenderness

  3. History (Hx) Past Ocular Hx: none Past Medical Hx: HTN, HLD, Anemia, Anxiety, Depression, GERD, IBS, Migraines Past Surgical Hx: shoulder surgery, tubal ligation Meds: Buspirone, Citalopram, Diclofenac cream, Fish Oil, Lyrica, Pantoprazole, Percocet, Pravastatin, Topiramate, Vitamin B12 Fam Hx: unremarkable Allergies: Amoxicillin Social Hx: non-smoker, non-EtOH ROS: fatigue

  4. Anterior Segment Exam

  5. Posterior Segment Exam

  6. CT Brain wo contrast

  7. T1 pre contrast

  8. T1 post contrast w Fat Suppression

  9. T2 w Fat Suppression

  10. Assessment 42yo WF with new expansile, sclerotic mass in the greater sphenoid wing of the left orbit without optic neuropathy DDX: - Meningioma • Leptomeningeal carcinomatosis • Osteosarcoma • Lymphoma - Pagets disease

  11. Plan • 7/28/17 Oculoplastics Follow up • Vision, IOP, pupils, extraocular movements normal; Hertelexophthalmometry showed OS proptosis; HVF full OU • Patient offered orbitotomy and biopsy, but elected for total excision • 10/10/17 Elective orbitozygomatic craniotomy • Meningothelial meningioma WHO Grade I without atypical features and en plaque middle fossa component

  12. 1 2 4 3

  13. Histology B A C

  14. Follow up 10/18/17 OculoplasticsClinic • BCVA 20/25-3, 20/20, no rapd, improving CN 3 palsy OS 10/21/17 Admission for Serratiamarcescenswound infection • s/p wound washout with removal and revision of implants • Discharged on IV cefepime with peripheral inserted central catheter 11/15/17 Oculoplastics Clinic • BCVA 20/25, 20/40, no rapd, ab/supraduction restriction OS • Plan for delayed lateral orbit repair

  15. Sphenoid Wing Meningioma • Up to 18% of all intracranial meningiomas • 23% of all optic nerve and meningeal orbital tumors • 2% of all orbital tumors • Visual loss occurs in 20-35% of cases • Risk factors: Female, 3rd-4th decade of life, Neurofibromatosis 2 gene mutations, ionizing radiation

  16. Clinical Presentation • Headache • Exophthalmos • Diplopia • Hemipareiss/Aphasia

  17. Diagnosis CT • Rounded or elongated extra-axial masses • Usually iso-dense to cerebrum • Homogenous enhancement prior and after contrast MRI • T1/T2 – similar intensity to cortex • Hypo-intensity within tumor • Homogeneous enhancement after gadolinium • Enhancing “dural tail” • Does not respect the dural boundary

  18. World Health Organization Classification • Grade I • Low mitotic rate, < four per ten high power fields (HPF) • Absence of brain invasion • Nine subtypes • Grade II (atypical) • Mitotic rate 4-19 per HPF • Brain invasion • 3/5 histologies: spontaneous necrosis, sheeting, prominent nucleoi, high cellularity, small cells • Grade III (anaplastic) • Mitotic rate > 20 per HPF • Papillary or rhabdoid meningioma

  19. Treatment • Goal of total gross resection • Limited the more medial the lesion • Medial lesions -bony decompression of superior orbital fissure and optic canal • Pterional approach • Frontotemporalorbitozygomatic (FTOZ) approach • Chemotherapy/radiotherapy

  20. Treatment Dotted Red line –Pterional approach Red and Black lines – FTOZ approach

  21. Prognosis • WHO Grade • Grade I has lower recurrence • Location • More lateral the better due to less resection • Extent of surgical resection • Increased complications with increased resection • Recurrence • WHO Grade, extent of resection

  22. Recurrence-free survivalOverall Recurrence Rate 3 years = 90% Pterional 36.5% 5 years = 80% FTOZ 12.2% 10 years = 65% Median recurrence –free survivalCranial nerve palsies Pterional114 months Trigeminal (V1/2) = 15% FTOZ 145 months Oculomotor = 7%

  23. 66yo F with stage IV metastatic lung adenocarcinoma and large right sphenoid wing meningioma • Lung cancer resistant to 3 previous lines of therapy • Treated with Nivolumab, a novel PD-1 checkpoint inhibitor • After 6 months of therapy, regression of lung cancer and 24% reduction in the volume of the meningioma

  24. Conclusions • Relatively common tumors of the orbit • Middle aged females; headaches, progressive proptosis, decreased vision • Treatment • Observation • Surgery • Radiotherapy/Chemotherapy • Prognosis depends on WHO grade, location, surgical approach

  25. References • Shields, Jerry A, Shields, Carol L. (2016) Eyelid, Conjuctival, and Orbital Tumors. Third Edition. Printed in China: Wolters Kluwer. Ch. 30. pp 588. • Chaichana KL, et al. Predictors of visual outcome following surgical meningiomas. J NeurolSurg B Skull Base. 2012 Oct;73(5):321-326. • Rogers L, et al. Meningiomas: knowledge base, treatment outcomes, and uncertainties: a RANO review. J Neurosurg. 2015 Jan;122(1):4-23. • Saloner D, et al. Modern meningioma imaging techniques. J Neurooncol. 2010 Sep;99(3):333-340. • Bir SC, et al. Comparison of the surgical outcome of pterional and frontotemporal-orbitozygomatic approaches and determination of predictors of recurrence of sphenoid wing meningiomas. World Neurosurg. 2017 Mar;99:308-319. • Rincon-Torroella J, Chaichana KL, Quinones-Hinojoas A. (2017) Video Atlas of Neurosurgery. First edition. China: Elsevier. Ch 32. pp 215-220.

  26. Thank you Dr. Compton Dr. Gerber Dr. Piri Dr. Puri

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