How Temozolomide Works
Temozolomide is a prodrug, meaning it's inactive when you take it but converts to an active form in your body. Here's how it works:
The Mechanism
- Conversion to active form: After absorption, temozolomide spontaneously converts to MTIC (monomethyl triazeno imidazole carboxamide) at physiological pH
- DNA methylation: MTIC methylates (adds methyl groups to) DNA at multiple sites, most importantly the O6 position of guanine and the N7 position of guanine
- DNA damage: These methyl adducts cause DNA damage and trigger the cell's DNA repair mechanisms
- Cell death: If the damage is too extensive to repair, the cell dies (apoptosis)
The Role of MGMT
One of the most important factors in how well temozolomide works is a protein called MGMT (O6-methylguanine-DNA methyltransferase):
- MGMT's job: This protein repairs the exact type of DNA damage that temozolomide causes (removes methyl groups from O6-guanine)
- MGMT methylation status: About 45% of glioblastomas have "methylated" MGMT, meaning the MGMT gene is turned off
- Why it matters: If MGMT is methylated (turned off), tumor cells can't repair temozolomide damage → much better treatment response
- Clinical impact: Patients with MGMT-methylated tumors have median survival of ~20-24 months with treatment vs. ~12-15 months with unmethylated MGMT
What is Temozolomide Used For?
FDA-Approved Uses
- Newly diagnosed glioblastoma: Given with radiation therapy, then continued as maintenance treatment (the Stupp protocol)
- Recurrent anaplastic astrocytoma: Brain tumor that has come back after initial treatment
Common Off-Label Uses
- Anaplastic oligodendroglioma
- Low-grade gliomas (in select cases)
- Brain metastases (from melanoma or other cancers)
- Medulloblastoma (recurrent)
- CNS lymphoma (recurrent)
- Pituitary tumors (aggressive cases)
- Neuroendocrine tumors (advanced)
How is Temozolomide Given?
The Stupp Protocol (Standard for Newly Diagnosed Glioblastoma)
This landmark regimen, published in 2005, dramatically improved survival for glioblastoma patients:
- Temozolomide dose: 75 mg/m² daily, 7 days/week
- Radiation: 60 Gy total, given in 30 fractions (2 Gy/day, Monday-Friday)
- Duration: 42 days (6 weeks)
- Timing: Take temozolomide 1 hour before radiation
- PCP prophylaxis: Required (usually Bactrim 3 times/week)
- Break: 4 weeks off treatment after completing chemoradiation
- Cycle 1 dose: 150 mg/m² daily for 5 days, every 28 days
- Cycles 2-12 dose: 200 mg/m² daily for 5 days, every 28 days (if cycle 1 tolerated well)
- Total cycles: Up to 12 cycles (some oncologists continue longer if tolerated)
- Timing: Days 1-5 of each 28-day cycle
Dosing Schedule
| Phase | Dose | Schedule | Duration |
|---|---|---|---|
| Concurrent with radiation | 75 mg/m² daily | Every day during radiation | 6 weeks |
| Maintenance - Cycle 1 | 150 mg/m² daily | Days 1-5 of 28-day cycle | 1 cycle |
| Maintenance - Cycles 2-12 | 200 mg/m² daily | Days 1-5 of 28-day cycle | Up to 11 cycles |
| Recurrent disease | 150-200 mg/m² daily | Days 1-5 of 28-day cycle | Until progression |
Alternative Dosing Schedules
Some oncologists use different schedules for recurrent disease or specific situations:
- Metronomic dosing: 50 mg/m² daily, continuous (every day). May have anti-angiogenic effects with less bone marrow toxicity
- 7 days on/7 days off: 150 mg/m² daily for 1 week, then 1 week off. More dose-dense approach
- 21 days on/7 days off: 75-100 mg/m² daily for 21 days, then 1 week break
Important Administration Guidelines
- Empty stomach: Take on an empty stomach (at least 1 hour before or 2-3 hours after eating) to reduce nausea and improve absorption
- Bedtime dosing: Many patients take it at bedtime to "sleep through" nausea
- Swallow whole: Don't open, crush, or chew capsules. If capsule breaks, avoid inhaling powder or getting it on skin
- Anti-nausea medicine: Take ondansetron (Zofran) or other anti-emetic 30 minutes BEFORE temozolomide
- Consistent timing: Take at the same time each day during the 5-day treatment period
Side Effects and Management
Most Common Side Effects (>30% of patients)
- Fatigue (60-80%): Usually mild to moderate. Rest when needed, short naps helpful
- Nausea and vomiting (40-60%): Usually manageable with anti-nausea medications
- Constipation (30-40%): Often related to anti-nausea medications. Increase fluids and fiber
- Headache (30-40%): Can be tumor-related or treatment-related
- Hair loss (55%): Usually mild thinning, not complete hair loss like traditional chemotherapy
Serious Side Effects Requiring Monitoring
- Fever ≥100.4°F (38°C) - may indicate infection when blood counts are low
- Unusual bleeding or bruising - easy bruising, nosebleeds, blood in urine/stool
- Severe fatigue or weakness - may indicate anemia or very low blood counts
- Shortness of breath, dry cough - could be PCP pneumonia or other lung infection
- Severe headache, vision changes, seizures - may indicate tumor progression or complications
- Persistent nausea/vomiting despite anti-nausea medications
1. Bone Marrow Suppression (Myelosuppression)
This is the MOST SERIOUS side effect of temozolomide:
- Timing: Blood counts typically drop 21-28 days after starting each cycle (nadir)
- Incidence:
- Low white blood cells (lymphopenia): 40-55%
- Low platelets (thrombocytopenia): 15-20%
- Low neutrophils (neutropenia): 8-14%
- Anemia: 15-20%
- Monitoring: Complete blood count (CBC) checked weekly during concurrent phase, then before each maintenance cycle
- Management:
- Dose reduction if severe (ANC <1,000 or platelets <50,000)
- Growth factors (G-CSF) if needed for neutropenia
- Platelet transfusions if severe thrombocytopenia
- Blood transfusions if severe anemia
2. Pneumocystis jirovecii Pneumonia (PCP) Risk
Temozolomide causes severe lymphocyte depletion, increasing risk of this opportunistic infection:
- When: During the entire concurrent chemoradiation phase (6 weeks) and continue until lymphocyte recovery
- Standard prophylaxis: Trimethoprim-sulfamethoxazole (Bactrim DS) 1 tablet 3 times per week
- If sulfa allergy: Dapsone 100 mg daily or atovaquone (Mepron) 1,500 mg daily
- Duration: Continue for at least 4 weeks after completing chemoradiation, or longer if lymphocyte count remains low
3. Nausea and Vomiting
Temozolomide is classified as moderate emetogenic risk:
- Prevention is key: Don't wait for nausea to start
- Anti-nausea medications:
- Ondansetron (Zofran) 8 mg: Take 30 minutes before temozolomide
- Metoclopramide (Reglan) 10 mg: Alternative or additional option
- Prochlorperazine (Compazine) 10 mg: For breakthrough nausea
- Olanzapine (Zyprexa) 5 mg: Very effective for refractory nausea
- Non-medication strategies:
- Take at bedtime to "sleep through" peak nausea
- Eat small, frequent meals (avoid large meals)
- Avoid greasy, spicy, or strong-smelling foods
- Ginger tea or ginger candies may help
- Stay hydrated - sip fluids throughout day
4. Fatigue
Very common, especially during concurrent chemoradiation:
- Multifactorial causes: Chemotherapy effects, radiation effects, anemia, brain tumor itself, steroids (if taking dexamethasone)
- Management:
- Balance activity and rest - short naps okay but avoid excessive daytime sleep
- Light exercise if possible (short walks)
- Treat underlying anemia if present
- Optimize thyroid function (radiation can affect pituitary gland)
- Consider stimulants (modafinil, methylphenidate) for severe fatigue
5. Hair Loss (Alopecia)
- Pattern: Usually mild to moderate hair thinning, not complete baldness
- Timing: Typically starts 3-4 weeks after beginning treatment
- Note: Radiation to the brain causes permanent hair loss in the radiation field. Temozolomide adds mild additional thinning
- Recovery: Hair typically regrows after treatment ends
6. Other Side Effects
- Liver enzyme elevation: Usually mild, monitored with regular blood tests
- Constipation: Very common (30-40%), especially with anti-nausea medications. Use stool softeners, increase fluids and fiber
- Loss of appetite: Common. Try small, frequent, high-calorie meals. Nutrition shakes if needed
- Rash: Uncommon (8-10%), usually mild
- Cognitive effects: Difficult to separate from tumor effects and radiation effects
Monitoring During Treatment
Required Blood Tests
| Test | Frequency | Purpose |
|---|---|---|
| Complete blood count (CBC) | Weekly during concurrent phase; before each maintenance cycle and on day 21-22 of cycle | Monitor bone marrow function |
| Comprehensive metabolic panel (CMP) | Before each cycle | Liver and kidney function |
| Lymphocyte count | Weekly during concurrent phase | Determine need for PCP prophylaxis |
Imaging During Treatment
- Brain MRI with contrast: Every 2-3 months during treatment
- Challenge: "Pseudoprogression" - inflammation from treatment can mimic tumor growth on MRI (occurs in 20-30% of patients, usually 2-3 months after completing radiation)
- Advanced imaging: MRI perfusion, MR spectroscopy, or PET scan may help distinguish pseudoprogression from true progression
Dose Modifications
Temozolomide dose is reduced or held based on blood count nadirs:
| Toxicity | ANC | Platelet Count | Dose Modification |
|---|---|---|---|
| Grade 0-2 | ≥1,500/µL | ≥100,000/µL | Increase to/maintain 200 mg/m² |
| Grade 3 | 1,000-1,499/µL | 50,000-99,999/µL | Reduce by 50 mg/m² (or maintain 150 mg/m²) |
| Grade 4 | <1,000/µL | <50,000/µL | Hold temozolomide; reduce by 50 mg/m² when resume |
How Well Does Temozolomide Work?
The Stupp Trial: A Landmark Study
The 2005 EORTC-NCIC trial (led by Dr. Roger Stupp) revolutionized glioblastoma treatment:
- Median survival: 14.6 months with radiation + temozolomide vs. 12.1 months with radiation alone
- 2-year survival: 27% vs. 11%
- 5-year survival: 9.8% vs. 1.9% (nearly 5-fold improvement!)
- Impact: This combination became the standard of care worldwide and remains so today
MGMT Status and Outcomes
The benefit of temozolomide varies significantly based on MGMT methylation:
| MGMT Status | Median Survival with TMZ+RT | 2-Year Survival | Temozolomide Benefit |
|---|---|---|---|
| Methylated (~45% of patients) | 21-24 months | ~48% | Substantial benefit |
| Unmethylated (~55% of patients) | 12-15 months | ~15% | Modest benefit |
Long-Term Survivors
While glioblastoma remains a very difficult cancer to treat, there are long-term survivors:
- 5-year survival: ~10% overall, up to 15-20% with favorable factors (young age, good performance status, MGMT methylation, gross total resection)
- 10-year survival: 2-5% in some series
- Favorable factors: MGMT methylation, younger age (<50), good performance status, IDH mutation (secondary glioblastoma), complete or near-complete surgical resection
How Long is Treatment?
Standard Duration
- Concurrent phase: 6 weeks (42 days of daily temozolomide with radiation)
- Break: 4 weeks off treatment
- Maintenance phase: 6-12 cycles of temozolomide (each cycle is 28 days)
- Total duration: Approximately 12-14 months from start to finish
Extended Treatment
Some evidence suggests benefit from continuing beyond 12 cycles:
- Practice variation: Some oncologists continue maintenance temozolomide for 18-24 cycles if well-tolerated
- Data: Retrospective studies suggest possible survival benefit with longer duration
- Challenge: Cumulative bone marrow toxicity often limits ability to continue beyond 12 cycles
- Decision factors: Individual tolerance, disease control, patient preference
Stopping Treatment
Treatment is typically stopped if:
- Tumor progression on MRI (confirmed, not pseudoprogression)
- Unacceptable toxicity (especially bone marrow suppression)
- Patient completes planned 12 cycles
- Patient preference after discussion with oncologist
Drug Interactions and Precautions
Important Drug Interactions
- Valproic acid (Depakote): May decrease temozolomide clearance. Often used together for seizure management; monitor closely
- Other myelosuppressive drugs: Increased risk of severe bone marrow suppression
- Live vaccines: AVOID during treatment (e.g., MMR, varicella, yellow fever, nasal flu vaccine). Inactivated vaccines okay but may be less effective
- CYP enzyme effects: Temozolomide is not metabolized by CYP enzymes, so fewer drug interactions than many medications
Medications to Support Treatment
Most patients on temozolomide take several supportive medications:
- Anti-seizure medication: Levetiracetam (Keppra), valproic acid (Depakote), or others - most brain tumor patients need seizure prophylaxis
- Corticosteroids: Dexamethasone for brain swelling (edema) - tapered as tolerated
- PCP prophylaxis: Bactrim, dapsone, or atovaquone (required during concurrent phase)
- Anti-nausea medications: Ondansetron (Zofran), metoclopramide (Reglan), or others
- Proton pump inhibitor: Omeprazole or similar (if taking dexamethasone to protect stomach)
- Stool softener: Docusate (Colace) to prevent constipation
Special Populations
- Pregnancy: Category D - can cause fetal harm. Effective contraception required during treatment and for 6 months after
- Breastfeeding: Do not breastfeed during treatment
- Elderly patients: May have increased toxicity, especially bone marrow suppression. Often need dose reductions
- Kidney disease: Use with caution; may need dose adjustment
- Liver disease: Use with caution; monitor liver enzymes closely
Storage and Handling
- Store capsules: Room temperature (68-77°F), away from moisture and light
- Keep in original bottle: Don't transfer to pill organizers (capsules can degrade)
- If capsule breaks: Don't touch powder. Clean up with wet paper towels and dispose in sealed plastic bag. Wash hands thoroughly
- Safe handling: Some oncologists recommend wearing gloves when handling capsules if you're sensitive
- Disposal: Return unused capsules to pharmacy for proper disposal. Don't flush down toilet
- Keep away from children and pets
Cost and Insurance Coverage
Medication Cost
- Brand name (Temodar): $30,000-50,000+ per cycle at 200 mg/m² dose (5-day cycle)
- Generic temozolomide: Much less expensive, typically $2,000-5,000 per cycle (prices vary widely)
- Total treatment cost: $25,000-100,000+ for complete 12-month treatment (generic to brand)
Insurance Coverage
- Coverage: Usually well-covered by insurance for FDA-approved indications (glioblastoma, anaplastic astrocytoma)
- Prior authorization: May be required
- Co-pays: Vary widely depending on insurance plan; can be several hundred to several thousand dollars per cycle
Financial Assistance
- Manufacturer copay assistance: Available from Merck (Temodar manufacturer) for eligible patients with commercial insurance
- Patient assistance programs: Free medication for uninsured or underinsured patients who qualify
- Generic assistance: Various programs through pharmacies and foundations
- Contact information:
- Merck Patient Assistance Program: 1-800-727-5400
- CancerCare Co-Payment Assistance: 1-866-552-6729
- Patient Advocate Foundation: 1-800-532-5274
Alternatives and Comparisons
Other Chemotherapy Options for Glioblastoma
- Lomustine (CCNU): Another alkylating agent. Sometimes used at recurrence. Not superior to temozolomide
- Bevacizumab (Avastin): Anti-angiogenic therapy. FDA-approved for recurrent glioblastoma. Improves progression-free survival but not overall survival. Often combined with temozolomide or lomustine
- Carmustine wafers (Gliadel): Chemotherapy wafers placed in tumor bed during surgery. Provides local chemotherapy. Modest survival benefit (2-3 months)
- PCV (procarbazine, lomustine, vincristine): Used for oligodendroglioma, not glioblastoma
Emerging Therapies
- Tumor Treating Fields (Optune): Wearable device that delivers alternating electric fields. Added to maintenance temozolomide, improves survival by 5 months (median survival 20.9 vs. 16 months)
- Clinical trials: Immunotherapy (checkpoint inhibitors, vaccines), targeted therapy, novel drug delivery methods
Recent Advances and Ongoing Research
Combination Approaches
- Temozolomide + Tumor Treating Fields: Now FDA-approved based on EF-14 trial showing survival benefit
- Temozolomide + targeted therapy: Studies combining with VEGF inhibitors, EGFR inhibitors, others
- Temozolomide + immunotherapy: Checkpoint inhibitors being tested in combination
Understanding Resistance
- MGMT-mediated resistance: Strategies to overcome, including MGMT inhibitors
- Mismatch repair deficiency: Rare in glioblastoma but when present, may predict resistance to temozolomide
- Stem cell populations: Glioblastoma stem cells may be more resistant to temozolomide
Novel Dosing Strategies
- Dose-dense temozolomide: 21/28 day regimen or metronomic daily dosing - still being studied
- Personalized dosing: Based on MGMT status and early response
Frequently Asked Questions
Living with Temozolomide Treatment
Practical Tips for Daily Life
- Set a daily alarm: To remember to take temozolomide at the same time each day
- Bedtime dosing: Most patients find taking it at bedtime with anti-nausea medicine 30 minutes before helps "sleep through" nausea
- Keep a symptom diary: Track side effects, energy levels, symptoms to discuss with your team
- Infection prevention: Wash hands frequently, avoid sick people, call doctor for fever ≥100.4°F
- Bleeding precautions: Use soft toothbrush, electric razor, be careful with sharp objects if platelets are low
- Stay hydrated: Drink plenty of fluids, especially during the 5 treatment days
When to Call Your Doctor
- Fever ≥100.4°F (38°C)
- New or worsening neurologic symptoms (weakness, numbness, vision changes, speech problems, seizures)
- Severe headache different from usual
- Unusual bleeding or bruising
- Shortness of breath or new cough
- Persistent nausea/vomiting despite medications
- Signs of infection (cough, burning with urination, skin redness/warmth)
Support Resources
- National Brain Tumor Society: 800-770-8287, www.braintumor.org
- American Brain Tumor Association: 800-886-2282, www.abta.org
- Brain Tumor Support Groups: In-person and online communities
- CancerCare: Free counseling and support, 800-813-4673
- Musella Foundation: Virtual Trial database and patient support, www.virtualtrials.com