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Grand Rounds An Incidental Orbital Mass

Grand Rounds An Incidental Orbital Mass. Josh Gross, MD December 15, 2017. Patient Presentation. CC Worsening blurry vision for 8 months HPI

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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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