1. Bakhshaee M, MD Rhinologist Azar 1388

2. Presentation 45 man complain from diplopia and headache

3. History Headache : Six months ago Diplopia: One month ago Trauma: Three years ago from car accident. Background Diseases: Neg Recurrent URTI: Neg Epistaxis: Neg Rhinorrhea: Neg Nasal Obstruction: Neg Visual Disturbance: Neg Smoking: Pos Job: Driver Smell: Ok Facial pain: three months ago Dizziness: Pos Hoarseness: Neg Other cranial nerve: Neg Long tract sign

4. Examination Nasal Endoscopy: Mild SD Visual acuity: 9/10 Eye movement: Right eye limitation to most lateral gaze Ptosis & Proptosis: Neg Neck Mass: Neg Sinus palpation: No tenderness Blood Pressure: 13/9 Craniofacial deformity: Neg Ear, Throat and oral cavity: No noticible point Retinal Examination: Ok

5. Paraclinic Lab Imaging CBC: Ok FBS: Ok Biochemistry : Ok Conventional CT Scan MRI

6. CT Scan

9. MRI

11. What is the Best? Diagnostic Endoscopy Angiograghy Theraputic Endoscopy

12. Diagnostic Endoscopy

13. Diagnosis Chordoma

14. Management Surgery Radiotherapy Chemotherapy

16. The sphenoid sinus has often been referred to as the neglected sinus because of its isolated position and difficult accessibility. Disease restricted to the sphenoid sinus is rare and often manifests with nonspecific or subtle signs and symptoms. Typically, patients are referred to the otolaryngologist because of a finding of isolated sinus opacification on CT scans ordered by the patient’s primary care physician or neurologist to evaluate vague symptoms such as headache.

17. Classification Isolated sphenoid sinus disease can be broadly divided 1. Inflammatory 2. Neoplastic 3. Miscellaneous categories

18. Inflammatory lesions Sinusitis  Acute  Chronic Fungal infections  Invasive: Mucormycoses, disseminated Aspergillosis  Noninvasive: Mycetoma, Aspergilloma Mucoceles Pyoceles

19. Neoplastic lesions Benign Malignant Intrinsic: 1. Inverting papillomas 2. Myofibroma 3. Schwannoma 4. Osteochondroma 5. Fibro-osseous disorders (fibrous dysplasia, ossifying fibroma) Extrinsic: 1. Meningioma 2. Paraganglioma 3. Pituitary macroadenomas Primary: 1. Squamous cell carcinoma 2. Adenocarcinoma 3. Adenoid cystic carcinoma 4. Mucoepidermoid carcinoma 5. Undifferentiated carcinoma 6. Transitional cell carcinoma Metastatic: 1. Renal cell carcinoma 2. Thyroid carcinoma 3. Prostate adenocarcinoma 4. Breast carcinoma 5. Lung carcinoma 6. Melanoma 7. Multiple myeloma and lymphoma

20. Miscellaneous Cerebrospinal leaks 1. Traumatic 2. Spontaneous Encephaloceles

21. Clinical signs and symptoms The most common symptom of sphenoid sinus disease is headache Visual disturbance is the second most frequently reported symptom Nasal obstruction Smell and taste Cranial nerve palsies Dizziness

22. Evaluation CT Scan: Air-fluid level 1. Acute & Chronic sinusitis 2. Polyp 3. Aneurism Thining & Expansion 1. Mucocel 2. tumor Sclerosis 1. Fungal 2. Fibroossous Bone erosion 1. Malignant Tumor

23. Chordomas Relatively rare tumors deriving from rests of embryonal notochord tissue located in the skull base, spine, and sacrococcygeal regions. Skull base chordomas are typically located in the midline, in the clival and basisphenoid regions

24. Symptoms Slow-growing tumors; therefore, most patients will relate a fairly long history of symptoms on initial presentation. Symptoms in a given patient will depend on the exact location of the tumor. Most commonly, the patient will report headaches and visual changes Diplopia secondary to cranial nerve (CN)VI paresis, Facial numbness, facial droop, Dysphagia, Hoarseness CN XII paresis

25. Evaluation CT Scan Reveal destruction of bone in a lytic pattern MRI T1: Isointense or hypointense lesion T2: bright signal that may appear heterogeneous. Gd: Enhancement is typically moderate to high

26. Management Current and historical management of skull base chordomas has involved : 1. Surgical excision 2. Radiation therapy 3. Both