Neuro-Ophthalmic Manifestations of Chordoma

Chordoma is a rare bone cancer that grows from leftover notochord tissue along the spine and skull base; when it forms at the skull base (the clivus), it can press on the optic pathways and the cranial nerves that move the eyes, causing double vision, droopy eyelid, unequal pupils, visual field loss, or swollen optic nerves. Chordoma FoundationStanford MedicineEyeWiki

The clivus sits directly under the optic nerves/chiasm and next to the cavernous sinus, where cranial nerves III (oculomotor), IV (trochlear), and VI (abducens) run. As a clival chordoma expands, it can compress, stretch, or invade these nerves, or raise pressure inside the skull. Among these nerves, cranial nerve VI (which turns the eye outward) is especially vulnerable, so horizontal double vision from a sixth-nerve palsy is very common. AjoEyeWiki

Chordoma is a rare cancer that grows from leftover cells of the embryonic notochord. In adults and children it often arises at the base of the skull (clivus) or along the spine. Even though it generally grows slowly, it tends to push into nearby structures and “comes back” locally unless treated with expert surgery and very focused radiation. When a tumor grows at the skull base, it can press on cranial nerves that move the eyes (III, IV, VI), on the optic nerves and optic chiasm that carry vision, and on nearby structures such as the cavernous sinus and pituitary region. The result can be double vision (diplopia), eye movement problems, droopy eyelid, changes in visual fields, or gradual vision loss. The most frequent early neuro-ophthalmic sign is sixth nerve (abducens) palsy, which causes inward eye turning and horizontal double vision. EyeWikiPubMedHouston Methodist Scholars

Standard tumor care for skull-base chordoma is maximal safe surgical removal by a high-volume skull-base team (often via an endoscopic endonasal route through the nose), followed by high-dose precision radiation (commonly proton-beam therapy) to control microscopic disease. Drug therapy is considered when the tumor cannot be fully removed or recurs/spreads; options include targeted therapies and, in select molecular subtypes, immunotherapies, but results vary and are often best pursued in clinical trials. Journal of NeurosurgeryChordoma FoundationCancer.govPMC

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Types

A) By tumor biology (pathology subtypes)

1. Conventional chordoma – the classic, slow-growing form composed of “physaliphorous” cells; shows brachyury positivity on immunostaining.

2. Chondroid chordoma – has cartilage-like areas but remains a chordoma by molecular markers.

3. Poorly differentiated chordoma – rarer, more aggressive; often shows loss of SMARCB1/INI1 protein; seen more in children/young adults.

4. Dedifferentiated chordoma – very aggressive, with areas resembling high-grade sarcoma.
   Brachyury (T gene product) is a highly specific diagnostic marker for chordoma and helps distinguish it from chondrosarcoma. PMCNatureModern PathologyThe American Journal of PathologyPubMed

B) By location

• Clival (skull base) – the one most linked to neuro-ophthalmic problems (double vision, headaches).

• Upper cervical – can also affect lower cranial nerves and head/neck posture; neuro-ophthalmic signs are less frequent than clival. Chordoma FoundationStanford Medicine

C) By neuro-ophthalmic pattern

• Cranial nerve palsies (III, IV, VI) → eye misalignment, double vision, droopy lid, pupil changes

• Optic pathway compression (optic nerve/chiasm) → blurred vision or bitemporal visual field loss

• Raised intracranial pressure → papilledema (swollen optic nerves), brief graying out of vision, headache

• Orbital or cavernous sinus involvement → painful eye movements, bulging eye (rare), multiple nerve deficits together EyeWikiStanford Medicine

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Causes/mechanisms of neuro-ophthalmic problems in chordoma

(Each item explains “what is happening” in simple terms.)

1. Direct pressure on cranial nerve VI in Dorello’s canal → the eye cannot turn outward; horizontal double vision. Ajo

2. Direct pressure on cranial nerve III → droopy eyelid, outward-down eye position, or big pupil that reacts poorly to light. EyeWiki

3. Direct pressure on cranial nerve IV → vertical/diagonal double vision that worsens when looking down stairs. EyeWiki

4. Spread into the cavernous sinus → several eye-movement nerves fail at once; complex double vision. EyeWiki

5. Compression of the optic nerve → blurred vision, color fading, or blind spots; sometimes one eye first. ResearchGate

6. Compression of the optic chiasm → loss of outer (“temporal”) side vision in both eyes (bitemporal field loss). Stanford Medicine

7. Raised intracranial pressure from blocked venous sinuses or CSF pathways → papilledema and transient dimming of vision. pubs.rsna.org

8. Hydrocephalus from brainstem/aqueduct compression → headaches, vomiting, papilledema, visual obscurations. pubs.rsna.org

9. Inflammatory edema around compressed nerves → worsens nerve function even without complete invasion. PMC

10. Ischemia of nerves from tumor pressure on nearby blood supply → sudden or stepwise nerve palsy. PMC

11. Pituitary stalk or gland compression near the chiasm → hormonal changes that indirectly affect vision comfort (fatigue, headaches). Stanford Medicine

12. Invasion of the clivus bone causing instability and brainstem contact → gaze palsies or nystagmus (rare). PMC

13. Post-surgical nerve irritation after skull-base surgery → temporary or persistent double vision. canadianjournalofophthalmology.ca

14. Radiation-related optic neuropathy (rare but serious, delayed) → sudden visual decline months after therapy. pubs.rsna.org

15. Radiation-related cranial neuropathy → delayed eye-movement weakness after high-dose skull-base radiation. PMC

16. Tumor cyst formation expanding near the optic pathways → stepwise visual field loss. pubs.rsna.org

17. Perineural spread along nerve sheaths in the cavernous sinus → multi-nerve palsies. PMC

18. Carotid artery encasement with chiasm compression (mass effect) → mixed visual and ocular motor deficits. pubs.rsna.org

19. Orbital apex extension (rare) → pain with eye movement, proptosis, optic neuropathy. pubs.rsna.org

20. Decompensation of a pre-existing, subtle eye misalignment due to illness and fatigue → new intermittent double vision. Ajo

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Common symptoms

1. Double vision (two of everything), often side-by-side and worse when looking far to one side. Stanford MedicineCleveland Clinic

2. Blurred vision that does not clear with blinking or rest. Cleveland Clinic

3. Loss of side vision (bumping into things at the edges). Stanford Medicine

4. Droopy eyelid on one side. EyeWiki

5. Unequal pupils or a pupil that reacts slowly to light. EyeWiki

6. Eye that will not move fully in one or more directions. EyeWiki

7. Eye strain or headache when reading, because the eyes are not working together. Stanford Medicine

8. Pain behind or around the eye, especially with movement (less common). Pacific Neuroscience Institute

9. Colors look faded (especially red) in one eye. ResearchGate

10. Brief “graying out” of vision when standing or with coughing (from papilledema). pubs.rsna.org

11. Headache that is new or getting worse. Stanford Medicine

12. Facial numbness or tingling (from trigeminal involvement). Stanford Medicine

13. Hearing changes or ringing (neighboring cranial nerves can be involved). Pacific Neuroscience Institute

14. Swallowing or speech difficulty (lower cranial nerves), sometimes accompanying the eye symptoms. Chordoma Foundation

15. Bulging eye or visible eye misalignment (rare, with orbital extension). pubs.rsna.org

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Diagnostic tests

A) Physical exam

1. Visual acuity (eye-chart): checks how clearly each eye sees at distance and near.

2. Color vision (e.g., Ishihara plates or red desaturation): sensitive to optic nerve dysfunction.

3. Pupil exam (swinging flashlight): looks for a relative afferent pupillary defect that points to optic nerve damage.

4. Ocular alignment and movements (the “H” test): identifies which cranial nerve is weak.

5. Funduscopy (dilated eye exam): looks for optic disc swelling (papilledema) or optic atrophy.
   Why these matter: they quickly flag optic-nerve problems, raised pressure, or specific nerve palsies that fit a skull-base lesion. EyeWikipubs.rsna.org

B) Manual/bedside tests

6. Cover–uncover test: reveals hidden eye drift.

7. Alternate cover test with prism: measures the amount of eye misalignment precisely.

8. Bielschowsky head-tilt test: helps confirm a trochlear (IV) palsy.

9. Near point of convergence: checks how well the eyes turn inward for near work.

10. Red glass or Maddox rod test: maps the direction and separation of double images to identify the weak muscle/nerve.
    Why these matter: they localize which nerve is failing and guide imaging to the clivus/cavernous sinus. canadianjournalofophthalmology.ca

C) Laboratory & pathology

11. Pituitary hormone panel (prolactin, morning cortisol, TSH/Free T4, LH/FSH, IGF-1): screens for stalk/gland effects near the chiasm. Stanford Medicine

12. Inflammatory markers (ESR/CRP) as baseline: not diagnostic of chordoma but helpful to rule in/out mimics.

13. Biopsy with histopathology: shows classic cell features (physaliphorous cells) and confirms the tumor type.

14. Immunohistochemistry for brachyury: key test—brachyury positivity strongly supports chordoma over chondrosarcoma. PMCNature

15. SMARCB1/INI1 immunostain (especially in children/young adults): loss supports poorly differentiated chordoma. Molecular testing can show SMARCB1 deletion. Modern PathologyScienceDirect

D) Electrodiagnostic

16. Visual evoked potentials (VEP): measures signal speed from eye to brain; delayed responses support optic-pathway compression.

17. Eye-movement recording / electro-oculography: documents abnormal saccades or gaze-holding in brainstem involvement.
    Why these matter: they quantify dysfunction when the exam is limited or when monitoring recovery. pubs.rsna.org

E) Imaging

18. MRI of brain, skull base, and orbits with contrast: first-line study; shows tumor size, relation to optic nerves/chiasm, cavernous sinus, and brainstem. Stanford Medicine

19. CT of skull base (bone windows): details clival bone destruction and surgical corridors. PMC

20. MR angiography or MR venography (or CTA): evaluates carotid encasement and venous sinus flow if pressure is high or surgery is planned. pubs.rsna.org

Non-pharmacological treatments

These do not shrink the tumor. They protect vision, reduce double vision, protect the cornea, and keep you safe while the oncology team treats the cancer. I’ve written each item with a brief description, purpose, and “how it helps” mechanism.

1. Education + symptom diary
   What: Keep a simple log of double vision times/angles, eye pain, headaches, and any visual “graying out.”
   Purpose: Helps your neuro-ophthalmologist fine-tune prisms or decide if a nerve palsy is stabilizing.
   Mechanism: Patterns show how eye misalignment changes during the day, guiding targeted aids and timing of surgery. (Clinical best practice.)

2. Temporary occlusion (patch or translucent “Bangerter” foil)
   What: Cover or dim one eye to stop double vision immediately.
   Purpose: Comfort and safety (reading, walking, driving restrictions as advised).
   Mechanism: Eliminates conflicting images when prisms are not yet set or palsy is changing. (Standard strabismus care in adults.)

3. Prism lenses (often stick-on Fresnel first)
   What: Prisms bend light so images from both eyes overlap again.
   Purpose: Reduce or eliminate double vision in primary gaze and reading.
   Mechanism: Optically realigns images without surgery; Fresnel prisms are easily adjustable while nerves recover or after radiation. canadianjournalofophthalmology.ca

4. Head-posture coaching
   What: Purposeful head turns/tilts that place eyes where double vision is minimal.
   Purpose: Functional single vision for daily tasks.
   Mechanism: Uses residual muscle ranges to line up the images (common compensation in nerve palsies). (Neuro-ophth practice.)

5. Low-vision rehabilitation
   What: Training + devices (illumination, high-contrast settings, magnifiers, CCTV/electronic readers).
   Purpose: Maintain reading, work, and independence when acuity or fields are reduced.
   Mechanism: Teaches efficient use of remaining vision and assistive tech. Evidence supports QOL gains, though RCTs are limited. PMCHealth.gov

6. Orientation & mobility (O&M) training
   What: Safe travel skills, white-cane use if needed, route planning with reduced fields or diplopia.
   Purpose: Prevent falls and increase confidence outdoors.
   Mechanism: Trains non-visual cues and scanning strategies. (Evidence base modest but widely used.) Cochrane

7. Work/learning accommodations
   What: Larger displays, text-to-speech, glare control, task lighting, breaks.
   Purpose: Reduce eyestrain and maintain productivity.
   Mechanism: Environmental adaptation keeps visual demand within comfortable limits. (Vision rehab standards.) PMC

8. Moisture-chamber glasses or nighttime eyelid taping
   What: Goggles or gentle tape at night to fully seal lids.
   Purpose: Prevent corneal drying if lids don’t close or corneal feeling is poor.
   Mechanism: Creates a humid micro-environment; studies suggest strong protection vs. lubrication alone. EyeWikiPMC

9. Humidifier and blink training
   What: Room humidity ~40–50%; conscious blinking during screens.
   Purpose: Reduce evaporation-related dryness.
   Mechanism: Preserves tear film and corneal clarity, lowering ulcer risk in exposure/neurotrophic keratopathy. NCBI

10. Protective eyewear
    What: Wraparound safety glasses for outdoor wind, dust, or low corneal sensation.
    Purpose: Prevent abrasions/infections on a vulnerable cornea.
    Mechanism: Physical barrier if the blink reflex is weak. Vagelos College

11. Lid hygiene and warm compresses
    What: Gentle lid cleaning and compresses.
    Purpose: Support the oily layer of tears to slow evaporation.
    Mechanism: Improves meibomian gland flow; complements moisture-chamber strategies. American Academy of Ophthalmology

12. Driving safety counseling
    What: Follow local regulations; avoid driving with active diplopia; reassess after prisms or surgery.
    Purpose: Public and personal safety.
    Mechanism: Matches legal/functional vision to task demands (discuss with your doctor). (Standard neuro-ophth counseling.)

13. Fall-proofing the home
    What: Better lighting, high-contrast stair edges, remove loose rugs.
    Purpose: Avoid injuries during periods of double vision or field loss.
    Mechanism: Environmental risk reduction (falls rise with visual impairment). Wiley Online Library

14. Mind-body therapies (CBT, mindfulness, support groups)
    What: Skills to manage anxiety, insomnia, and cancer-related stress.
    Purpose: Better treatment adherence, quality of life, and coping.
    Mechanism: Reduces sympathetic arousal and catastrophizing (helpful in chronic neurologic/oncologic care).

15. Physical therapy & balance work
    What: Gait and balance training when diplopia or brainstem compression caused unsteadiness.
    Purpose: Prevent falls; rebuild confidence.
    Mechanism: Proprioceptive and vestibular compensation strategies.

16. Sun and skin protection (especially on EGFR drugs)
    What: Hats, sunglasses, sunscreen.
    Purpose: EGFR inhibitors can cause rash; sun worsens it.
    Mechanism: UV avoidance reduces cutaneous side effects. MedlinePlus

17. Smoking cessation
    What: Stop tobacco entirely.
    Purpose: Protects healing, overall health, and medication effectiveness (notably EGFR inhibitors).
    Mechanism: Smoking induces enzymes that lower erlotinib levels and response. PMCFDA Access Data

18. Coordinate care in a skull-base center
    What: Multidisciplinary team (neurosurgery, ENT skull-base, radiation oncology, neuro-ophthalmology).
    Purpose: Better surgical access, safer resections, and expert radiotherapy planning around the optic apparatus.
    Mechanism: High-volume expertise improves local control and reduces complications. Chordoma FoundationJournal of Neurosurgery

19. Vision-first planning for radiation
    What: Radiation oncologists set strict dose limits to optic nerves/chiasm and retina; proton therapy often helps spare normal tissue.
    Purpose: Reduce radiation-induced optic neuropathy risk.
    Mechanism: Conformal plans and particle therapy concentrate dose on tumor and spare critical vision structures. FrontiersNature

20. Regular neuro-ophthalmic follow-up
    What: Scheduled checks of acuity, fields, ocular motility, and optic nerve status.
    Purpose: Early detection of improvement or new problems after surgery or radiation.
    Mechanism: Timely adjustments (prisms, drops, protective measures, or surgery) preserve function. EyeWiki

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Drug treatments

Doses below reflect common adult regimens used in related conditions and labels; chordoma use may be off-label and should be managed by oncology specialists. Always individualize dose for age, weight, kidney/liver function, and other medicines.

1. Dexamethasone (corticosteroid; edema control)
   Dose/timing: Often started 4–8 mg/day divided for mild tumor-related symptoms; up to 16 mg/day for severe mass-effect symptoms, then taper as quickly as safely possible.
   Purpose & mechanism: Shrinks peritumoral brain edema, reducing pressure on cranial and optic nerves; improves headaches, diplopia from mass effect, and nausea.
   Notable side effects: High blood sugar, mood changes, insomnia, infection risk, stomach irritation, bone loss; long tapers often needed. PMC+1Alberta Health Services

2. Imatinib (tyrosine kinase inhibitor targeting PDGFR; systemic therapy for unresectable/recurrent chordoma)
   Dose: Commonly 400–800 mg orally daily in studies.
   Purpose & mechanism: Many chordomas express PDGFR; imatinib blocks that signaling to slow growth; disease stabilization is more common than shrinkage.
   Side effects: Swelling, fatigue, rash, nausea; avoid St John’s wort (lowers drug levels). ScienceDirectResearchGatePubMed

3. Erlotinib (EGFR inhibitor; systemic therapy option)
   Dose: 150 mg orally daily.
   Purpose & mechanism: Some chordomas rely on EGFR signaling; blocking it can control growth in select cases or after other TKIs.
   Side effects & tips: Rash, diarrhea; smoking lowers drug exposure and effectiveness; sun protection advised. FrontiersFDA Access DataMedlinePlus

4. Afatinib (irreversible EGFR/HER2 inhibitor; systemic therapy under study)
   Dose: Often 40 mg orally daily (study protocols vary).
   Purpose & mechanism: Deeper EGFR pathway blockade; a recent prospective study reported disease control in a subset of chordoma patients.
   Side effects: Diarrhea, rash, mouth sores; hydration and skin care help. Chordoma FoundationAACR Journals

5. Sunitinib (VEGFR/PDGFR TKI; systemic option)
   Dose: 50 mg daily 4 weeks on / 2 weeks off, or 37.5 mg daily continuously (oncologist choice).
   Purpose & mechanism: Anti-angiogenic and anti-PDGFR effects may slow tumor growth.
   Side effects: Hypertension, fatigue, hand-foot syndrome, thyroid changes. Frontiers

6. Pazopanib (VEGFR TKI; systemic option)
   Dose: 800 mg orally once daily on an empty stomach.
   Purpose & mechanism: Starves tumor blood-vessel signaling; case reports/series suggest occasional responses.
   Side effects: Liver enzyme elevation, hypertension, fatigue; avoid grapefruit. PMCNature

7. Regorafenib (multi-kinase inhibitor; later-line systemic option)
   Dose: 160 mg daily for 3 weeks on / 1 week off (label schedule in other cancers).
   Purpose & mechanism: Broad anti-angiogenic/oncogenic kinase blockade; occasionally used when other TKIs fail.
   Side effects: Hand-foot reaction, fatigue, liver enzyme elevation. (Use per sarcoma/solid-tumor practice and trials.) SpringerLink

8. Pembrolizumab (PD-1 immune checkpoint inhibitor; selected cases)
   Dose: 200 mg IV every 3 weeks (or 400 mg q6w).
   Purpose & mechanism: Unleashes T-cells; can help in poorly differentiated or SMARCB1/INI1-deficient chordomas and occasional conventional cases, usually in trials.
   Side effects: Immune-related rash, colitis, thyroiditis; needs close monitoring. ASCO PublicationsResearch Communities by Springer NatureScienceDirect

9. Nivolumab (PD-1 inhibitor; selected cases)
   Dose: 240 mg IV q2w or 480 mg IV q4w.
   Purpose & mechanism: Similar to pembrolizumab; documented responses in INI1-deficient pediatric chordoma.
   Side effects: Immune-related events (skin, endocrine, liver, lung). ASCO Publications

10. Tazemetostat (EZH2 inhibitor; INI1-negative/poorly differentiated chordoma)
    Dose: 800 mg orally twice daily in existing protocols.
    Purpose & mechanism: Targets epigenetic driver when SMARCB1/INI1 is lost; emerging data suggest activity in this rare subtype.
    Side effects: Fatigue, nausea; contraception needed due to embryo-fetal risk. MDPIResearchGate

Important: Some of these medicines are off-label for chordoma or best given on clinical trials. Your oncology team will match drugs to tumor biology (e.g., PDGFR/EGFR activity, INI1 status) and your overall health. MDPIJournal of Neurosurgery

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Dietary “molecular” supplements

No supplement treats chordoma. These suggestions support eye surface health, bone health (especially if you use steroids), and overall resilience. Discuss each with your team to avoid drug interactions.

1. Vitamin D3 (e.g., 800–2000 IU/day, individualized)
   Function/mechanism: Bone and immune support; especially important if on steroids or low sun exposure. (Check blood levels.) American Cancer Society

2. Calcium (diet first; supplement to reach ~1000–1200 mg/day total)
   Function: Bone protection during/after steroids.
   Mechanism: Supports bone remodeling; combine with vitamin D. ASH Publications

3. Vitamin B12 (if low; typical 1000 µg/day oral or as prescribed)
   Function: Nerve health; can help prevent neuropathy from deficiency.
   Mechanism: Cofactor for myelin/nerve function. ASH Publications

4. Omega-3 fatty acids (EPA/DHA 1–2 g/day if approved)
   Function: Tear film support and general cardiometabolic health.
   Mechanism: Anti-inflammatory lipid mediators; evidence for dry eye is mixed—use as adjunct, not primary therapy. American Cancer Society

5. Lutein + Zeaxanthin (AREDS2 doses: 10 mg/2 mg daily)
   Function: Macular pigment support and glare reduction.
   Mechanism: Antioxidants concentrated in macula; useful for glare sensitivity though AREDS2 evidence is in AMD, not chordoma. American Cancer Society

6. Vitamin C (diet-based, or modest supplement)
   Function: Antioxidant support; wound healing.
   Mechanism: Collagen cross-linking, scavenges free radicals. (ACS nutrition guidance.) American Cancer Society

7. Vitamin E (diet emphasis; avoid high-dose unless advised)
   Function: Antioxidant support for cell membranes.
   Mechanism: Synergizes with vitamin C; caution with anticoagulants. (ACS guidance.) American Cancer Society

8. Zinc (diet first; avoid high-dose long-term unless directed)
   Function: Immune and wound healing roles.
   Mechanism: Enzymatic cofactor; excess can lower copper.

9. Probiotics (product-specific dosing)
   Function: GI support during stress or antibiotics.
   Mechanism: Gut microbiome balance; choose strains with oncology team approval (immunosuppression considerations). American Cancer Society

10. Magnesium (if low; dose individualized)
    Function: Muscle/nerve function, sleep quality.
    Mechanism: Cofactor in neuromuscular signaling.

Interaction alerts: St John’s wort lowers levels of many TKIs (notably imatinib); smoking reduces erlotinib levels; grapefruit can raise TKI levels unpredictably. Always clear supplements with your oncologist/pharmacist. PubMedFDA Access DataPMC

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Regenerative / stem-cell” drugs

• Immune checkpoint inhibitors (e.g., nivolumab, pembrolizumab) can help selected chordoma patients, especially those with INI1-deficient (poorly differentiated) chordoma, but results vary and treatment is typically pursued in expert centers or trials. ASCO PublicationsScienceDirect

• EZH2 inhibitor (tazemetostat) is experimental but promising for INI1-negative chordoma. MDPI

• Cancer vaccines (brachyury-targeted) and cell therapies are being explored; they remain investigational. MDPI

• Unapproved “stem-cell” injections or exosomes offered outside clinical trials should be avoided. The U.S. FDA explicitly warns that many marketed regenerative products are not approved and have caused serious harms. Do not pursue stem-cell therapies for chordoma outside regulated trials. PMCAAO Journal

If you read headlines about “regenerating nerves,” remember: for eye-surface nerve loss (neurotrophic keratopathy), approved options are topical therapies and specialized surgeries—not systemic stem cells. PubMed

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Surgeries

1. Endoscopic endonasal resection of clival chordoma
   What: A team of neurosurgeons and ENT skull-base surgeons removes as much tumor as safely possible through the nose with an endoscope.
   Why: To relieve pressure on cranial/optic nerves and enable effective postoperative radiation. This approach often offers direct access to the clivus with less brain retraction. PMC+1

2. Transcranial skull-base resection
   What: Open approaches (e.g., extended skull-base corridors) when tumor extends laterally or inferiorly beyond safe endonasal reach.
   Why: To achieve maximal safe resection tailored to tumor extent and anatomy. RSNA Publications

3. Strabismus (eye-muscle) surgery with adjustable sutures
   What: Repositioning one or more eye muscles; sutures can be adjusted after surgery while you’re awake to fine-tune alignment.
   Why: For persistent, stable double vision after nerve palsy and once tumor/radiation effects are stable; goal is comfortable single vision in primary gaze/reading. canadianjournalofophthalmology.ca

4. Tarsorrhaphy or eyelid weight placement (oculoplastics)
   What: Partially sewing lids together (temporary or permanent) or implanting a small gold/platinum weight in the upper lid.
   Why: To protect the cornea when eyelids can’t close (exposure keratopathy) and lubrication isn’t enough. Moisture-chamber strategies can also help and may postpone surgery. EyeWikiAmerican Academy of Ophthalmology

5. CSF shunt or endoscopic third ventriculostomy (ETV)
   What: Diversion procedures when hydrocephalus (fluid build-up) raises intracranial pressure.
   Why: To protect vision and brain function when medical measures aren’t enough. (Neuro-oncology standard practice.)

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Prevention tips

1. Protect the cornea: use preservative-free tears/ointment, moisture-chamber glasses, and nighttime taping if lids don’t close fully. EyeWiki

2. Don’t ignore new double vision or visual dimming: prompt neuro-ophthalmic review can prevent accidents and catch compression early. EyeWiki

3. Follow steroid plans carefully: use the lowest effective dose and taper under supervision to limit side effects. Alberta Health Services

4. Plan radiation with vision in mind: choose experienced centers; ask about optic pathway dose constraints and proton therapy suitability. Frontiers

5. Avoid risky “stem-cell cures”: stick to regulated trials only. PMC

6. Quit smoking: improves medication effectiveness (notably EGFR inhibitors) and general healing. FDA Access Data

7. Manage dry eye triggers: screens, air vents, and low humidity; use blinking breaks and a humidifier. NCBI

8. Keep nutrition strong during treatment: prioritize plant-forward meals and adequate protein; see an oncology dietitian early. American Cancer Society

9. Prevent falls: brighter lighting, high-contrast stair tape, no loose rugs; use O&M training if fields are reduced. Cochrane

10. Maintain regular follow-ups: neuro-ophthalmology plus skull-base oncology/radiation, since issues can emerge months to years later. Journal of Neurosurgery

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When to see a doctor urgently

• Sudden or rapidly worsening double vision, new droopy eyelid, or new eye movement problem.

• New visual loss, curtain, or graying out, especially with headache or nausea/vomiting.

• Eye pain, light sensitivity, or a red, watering eye in someone with poor lid closure or reduced corneal feeling (could be a corneal ulcer).

• Severe headache on waking, transient vision blackouts, or pulsatile tinnitus (possible raised intracranial pressure).
  These can be vision- or life-threatening emergencies—seek urgent neuro-ophthalmic or emergency care. (Neuro-oncology and cornea practice standards.)

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What to eat and what to avoid

What to eat (helpful habits):

1. Plenty of colorful vegetables and fruits daily (aim for a variety across the week).
   Supports antioxidants and fiber; aligns with American Cancer Society nutrition advice. American Cancer Society+1

2. Adequate protein (eggs, fish, poultry, dairy, legumes, tofu).
   Supports healing after surgery/radiation and maintains strength. American Cancer Society

3. Calcium + vitamin D sources (dairy/fortified plant milks, small fish with bones, sunlight as appropriate, vitamin D supplement if needed).
   Helps offset steroid-related bone loss. ASH Publications

4. Whole grains and legumes
   Stabilize energy and support gut health during treatment. American Cancer Society

5. Hydration
   Keeps mucus membranes (including the eye surface) comfortable; aim for regular fluids unless restricted.

What to limit/avoid (safety and interactions):

6. Grapefruit/grapefruit juice with TKIs (imatinib/pazopanib/regorafenib).
   May alter drug levels—avoid unless your team says otherwise. PMC

7. St John’s wort and other enzyme-inducing herbals.
   Can lower imatinib and other TKI levels and reduce effectiveness. PubMed

8. Smoking
   Directly reduces erlotinib exposure; also worsens healing. FDA Access Data

9. Very salty ultra-processed foods
   May worsen steroid-related fluid retention and blood pressure; focus on fresh foods when possible. American Cancer Society

10. Excess alcohol
    Can interact with meds and inflame the GI tract/immune system; ask your team what’s safe for you. American Cancer Society

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Frequently asked questions

1) Can double vision from chordoma get better on its own?
Sometimes. If the cause is temporary nerve irritation or post-operative swelling, it may improve over weeks to months. While waiting, prisms or occlusion help you function safely. Persistent, stable misalignment can be fixed with strabismus surgery. canadianjournalofophthalmology.ca

2) Why is the sixth nerve (abducens) palsy so common?
The sixth nerve has a long, vulnerable course (including through Dorello’s canal near the clivus). A clival tumor easily presses it, causing inward eye turn and horizontal diplopia. EyeWiki

3) My MRI shows the tumor near the optic chiasm. What visual changes should I watch for?
Report subtle side-vision loss (bitemporal field defects), color desaturation, dimming, or transient “grayouts.” Early detection helps protect function. PubMed

4) Could radiation hurt my vision?
Radiation is essential for control, but very high doses to the optic nerves/chiasm can rarely cause radiation-induced optic neuropathy. Modern planning keeps doses within safe limits; proton therapy can further spare healthy tissue. NatureFrontiers

5) Are there medications that actually shrink chordoma?
Some targeted drugs (e.g., imatinib, EGFR/VEGFR inhibitors) and immunotherapies can stabilize or occasionally shrink tumors in selected patients, often within clinical trials. Results are variable, and surgery + high-dose radiation remain the mainstays. MDPIFrontiers

6) I’ve heard of “brachyury vaccines.” Are they available?
Brachyury (TBXT) is a hallmark protein in chordoma; vaccines targeting it are experimental and accessible mainly in research settings. MDPI

7) Should I try stem-cell therapy for nerve healing?
No, not outside regulated trials. The FDA warns against unapproved stem-cell/exosome products due to safety issues and lack of proof. PMC

8) If my lids don’t close, will “just drops” protect my eye?
Drops help, but moisture-chamber protection (and sometimes temporary tarsorrhaphy) is often needed to truly protect the cornea. EyeWiki

9) How long should I wait before considering strabismus surgery?
Typically once alignment is stable (often several months after surgery or radiation), your surgeon can plan adjustable-suture surgery for comfortable single vision. canadianjournalofophthalmology.ca

10) Can low-vision rehab help even if my visual acuity is “okay”?
Yes. It helps with contrast, glare, reading endurance, and field adaptation, improving overall quality of life. PMC

11) Do prisms work for every double-vision angle?
They work best for small-to-moderate, relatively stable deviations—often with stick-on Fresnel prisms first. Very large or varying deviations may need occlusion or surgery. canadianjournalofophthalmology.ca

12) Are there warning signs of dangerous intracranial pressure?
Headaches on waking, transient vision loss with position change, pulsatile noise in ears, vomiting, or rapidly worsening vision can indicate pressure—seek urgent care. (Neuro-ophthalmology standards.)

13) Which supplements are must-haves?
There’s no “must-have” for tumor control. Vitamin D and calcium are common if you use steroids; others depend on your labs and diet; always check interactions first. ASH Publications

14) Does smoking really make some cancer drugs less effective?
Yes. Erlotinib levels are significantly lower in smokers; quitting matters. FDA Access Data

15) Where should I be treated?
At a skull-base center with chordoma experience and integrated neuro-ophthalmology. Outcomes are better when surgery and radiation are planned by teams who do this often. Chordoma Foundation

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The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: August 14, 2025.

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