Published Nov 20, 2024

47-Year-Old Woman Likened Her Headaches To Stress, But It Turned Out To Be a Highly Aggressive Brain Tumor— Understanding Gioblastoma Multiforme (GBM)

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Understanding Glioblastoma Multiforme (GBM)

Amy Wareham first thought she was suffering from “stress” headaches. However, she ultimately was diagnosed with a highly aggressive tumor called glioblastoma multiforme (GBM), a grade 4 glioma brain tumor that arises from glial cells in the brain and spinal cord.
Glioblastoma (GBM), a grade 4 glioma, presents significant challenges for treatment due to its highly invasive nature, rapid growth, and resistance to most conventional therapies.
While new treatment approaches are continually being explored through clinical trials, the standard of care for glioblastoma has remained largely consistent since 2005. It combines maximal safe surgical resection (surgery to remove as much of the cancer as possible) followed by chemotherapy and radiation.
The typical course after surgery is chemotherapy and radiation therapy together, followed by additional chemotherapy. More recently, a clinical trial found that the addition of tumor treating fields (Optune Gio) to the chemotherapy following combined chemoradiation improved survival further with minimal increase in toxicity.
Tumor Treating Fields, or TTF therapy, is delivered via a wearable device called Optune Gio [which Wareham uses]. It uses alternating electric fields to inhibit tumor growth.

Amy Wareham initially dismissed her infrequent headaches to stress from work, but she later discovered she had a highly aggressive tumor called glioblastoma multiforme (GBM), a grade 4 glioma brain tumor that arises from glial cells in the brain and spinal cord.

The 47-year-old woman, who has since set up The Amy Wareham Fund to help fund research on high grade brain tumors, didn’t learn her head pain was due to cancer until she suffered from a seizure last May, something which led her to have a CT scan and an MRI scan, revealing two lesions on her brain. Two weeks later she underwent emergency neurosurgery. Then, in July 2023, she was diagnosed with Glioblastoma Grade 4 (GBM).

Wareham, who resides in London, England, recounted her frightening diagnosis in a recent interview with SWNS, according to Yahoo! Life, explaining, “At the moment, I am relatively very well. I have come a long way since my first and last seizure in May last year. Then, I was very scared and had a whirlwind of appointments and scans. Now, things have calmed down and I am taking it day by day.

“My last MRI was stable. Aside from quarterly MRI scans, I have Avastin infusions fortnightly and chemotherapy monthly. The chemotherapy thankfully is oral and I have been tolerating it well.”

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A post shared by Amy Wareham (@amybrainy)

As for her diagnosis, she admits, “It is an understatement to say I was shocked at being told I have glioblastoma grade 4, and that it has an average 12 to 18 month prognosis.

“I was very surprised that I hadn’t heard of it, it being the biggest cancer killer of under 40-year-olds, including children, and tenth biggest overall cancer killer. That’s why I set up my own Fund with The Brain Tumor Charity. I immediately knew that I needed to raise money and awareness.”

Wareham, who married her now-husband months after her diagnosis, also noted that there’s a lack in funding for brain tumor research, something she says is “urgently needed.”

The resilience lawyer, noted in a recent social media post that she also has breast cancer [early stage breast cancer called Ductal carcinoma in situ (DCIS)], informing her followers, “Here I am waiting for my 6 month check up. Good news, it’s shrinking and hasn’t spread to my lymph nodes.

“Bad news, I’ve still got the more aggressive Glioblastoma stage 4. I’m still taking it day by day which is all I can really do.”

Earlier this summer she shared a selfie on Instagram, writing, “Back in Guy’s for my fortnightly Avastin infusion. Also back on Optune, had a welcome break during my radiotherapy but now it’s back to work. MRI a week Friday, send me positive vibes!”

This past spring, she announced a third new spot was found on a part of her brain, but she was focused on keeping a positive mindset throughout her cancer journey.

She also added, “I have started Avastin supported by my private healthcare. FYI Avastin is only available in the USA under specific circumstances. I am grateful to my medical team and employer for their support getting my application over the line. It certainly wasn’t guaranteed. The downside is that my employer is funding but I’m not focusing on that.

“In breast cancer news I’m also focusing on the positives. Last month I booked myself a follow up medical appointment with my breast cancer specialists through my private healthcare. You may recall that I started tamoxifen to inhibit the growth of my existing breast lump. The good news is that it seems to be working. It was confirmed at that appointment that the lump hadn’t grown and in fact was shrinking. They put a “marker” into my breast lump in the form of a staple. This is to monitor my breast cancer, we have high hopes the lump will disappear.”

Wareham concluded, “Rest assured that I’m facing the road ahead positively. Good things come to those who wait patiently.”

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A post shared by Amy Wareham (@amybrainy)

Understanding Gliomas & How They Are Diagnosed

Glioblastoma (GBM) is the most aggressive and lethal form of primary brain tumor.

A glioma is a tumor originating in the central nervous system (CNS), specifically in the brain or spinal cord. A glioma originates in glial cells. Glial cells are supportive cells in the brain that protect and maintain the neurons. These types of tumors can either be benign (non-cancerous) or malignant (cancerous).

Glioblastomas present doctors with a significant challenge for treatment due to their highly invasive nature, rapid growth, and resistance to most conventional therapies.

WATCH: Understanding Gliomas

Diagnosing gliomas involves a multi-step approach that involves clinical assessment, imaging studies, histopathological examination, and molecular testing.

As patients are diagnosed, they’re observed for typical glioma symptoms. These may include:

Headaches
Seizures
Cognitive or Behavior Changes
Visual or Speech Changes and Impairments
Loss of Body Weight and Deconditioning
Changes in mental function, mood, or personality
Changes in speech
Sensory changes in hearing, smell, and sight
Loss of balance
Changes in your pulse and breathing rate

Patients then undergo a neurological exam that tests cranial nerve function, motor strength and coordination, sensory function, and cognitive abilities.

Next, patients undergo an MRI, which provides doctors with a visualization of the tumor.

“If you’re suspected of having a tumor on imaging and our neurosurgeons think that tumor can come out, they will take a piece of that tumor out first and confirm in the operating room and with our pathologists that, in fact, what they are looking at is a tumor,” Dr. Alexandra Miller, Director of the Neuro-Oncologist Division at NYU Langone Health, tells SurvivorNet. “And if it is, they resect it at that time. It’s not usually a two-step procedure.”

If surgery cannot be performed due to tumor location or patient-specific factors, a less invasive stereotactic biopsy can be obtained. Once the tissue sample is obtained, it’s examined under a microscope for molecular testing. At this stage, the tumor is given a grade, which determines how aggressive it is.

Grade I-II gliomas – These are considered low-grade and tend to grow slowly. “The grade one is a very indolent, benign tumor that can be cured with surgery alone,” Dr. Henry Friedman, Deputy Director of the Preston Robert Tisch Brain Tumor Center at Duke, tells SurvivorNet.
Grade III gliomas – These are considered high-grade and tend to grow more rapidly. They are typically classified as malignant and require more aggressive treatment, which can include surgery, radiation, and chemotherapy.
Grade IV gliomas – These are the most aggressive gliomas, which are locally aggressive and require treatment intensification. Glioblastomas are the most common grade IV glioma, “which is by far the most well-known and feared tumor in the lay population and, quite frankly, the medical population,” Dr. Friedman explains.

What Are Treatment Options for Glioblastoma Patients?

Surgery is often the first line of treatment for patients with a brain tumor. However, other treatment options may be explored if the patient cannot withstand surgery.

WATCH: Treatment Path for Glioma Patients

The main treatment options are:

Observation—Some benign, small, and without symptoms of gliomas or tumors located in inoperable locations can be recommended for observation.
Surgery is often the first-line treatment for gliomas. The goal is maximal safe resection, where the largest amount of tumor is removed without causing significant neurologic deficits. For low-grade gliomas, surgery alone can sometimes be curative.

“We maximize the amount of tumor that can be removed through surgical intervention,” Dr. Friedman explains.

After surgery, pathologists examine the tumor tissue to understand its features and molecular makeup, a process part of molecular testing. This test helps doctors better understand the tumor’s genetic makeup and offers insight into an effective treatment.

One common mutation appreciated through molecular testing in gliomas is the IDH (isocitrate dehydrogenase) mutation, which can now be targeted with new treatments like Vorasidenib.

Radiation and chemotherapy are often needed after surgery because removing the tumor completely is usually not possible due to the tumor’s ability to spread into surrounding brain tissue.

Radiation therapy uses high–energy X–rays to target and kill tumor cells. It is often used after surgery to target residual tumor cells.
Chemotherapy – These medications kill or slow the growth of cancer cells. Chemotherapy can be used alongside radiation or following radiation and is often used in higher-grade tumors.

“There’s always going to be microscopic disease left behind even after what appears to be a gross total resection (when a surgeon removes most of the visible tumor), and the tumor can certainly evolve,” Dr. Friedman explains. Chemotherapy is used to help control this residual disease.

Together, surgery, chemotherapy, and radiation form the core standard of care for glioma patients, providing a comprehensive approach to tackling the disease from multiple angles.

The Food and Drug Administration (FDA) has approved some drug treatments, including temozolomide (Temodar), to help patients with this aggressive disease. Temozolomide is a chemotherapy drug patients can take after surgery and radiation therapy.

Targeted therapy and immunotherapy are newer treatments designed to target specific genetic mutations in the tumor or to stimulate the immune system to fight the cancer. Their role in the treatment of gliomas is continuing to evolve.

Other FDA-approved drugs for treating glioblastoma include lomustine (Gleostine), intravenous carmustine (Bicnu), carmustine wafer implants, and Avastin (bevacizumab).

Avastin, which Wareham is now taking, is a targeted drug therapy that blocks glioblastoma cells from requesting new blood vessels that feed and allow the tumor to grow.

All About Glioblastoma Multiforme (GBM)

Glioblastoma (GBM) is the most aggressive and lethal form of primary brain tumor. Classified as a Grade 4 glioma by the World Health Organization (WHO), glioblastoma presents significant challenges for treatment due to its highly invasive nature, rapid growth, and resistance to most conventional therapies.

Despite advances in neurosurgery, chemotherapy, radiation therapy, and supportive care, glioblastoma remains a highly fatal disease, with median survival rates of approximately 20 months and a five-year survival rate of approximately 10%.

While new treatment approaches are continually being explored through ongoing clinical trials, the standard of care for glioblastoma has remained largely consistent since 2005, following a landmark trial that established the current multimodality treatment which combines maximal safe surgical resection (surgery to remove as much of the cancer as possible) followed by chemotherapy and radiation.

“What we attempt to do to achieve that control of the disease is we often follow surgery with some form of chemotherapy as well as radiation to help control the microscopic disease of infiltration,” Dr. Ganish Shankar, a neurosurgeon at Massachusetts General Hospital, tells SurvivorNet. The typical course after surgery is chemotherapy and radiation therapy together, followed by additional chemotherapy.

More recently, a clinical trial published in 2017 found that the addition of tumor treating fields (Optune Gio) to the chemotherapy following combined chemoradiation improved survival further with minimal increase in toxicity.

Wareham often takes to her Instagram page to mention that she uses Optune Gio to treat her cancer. Tumor Treating Fields (Optune Gio) represent a relatively new addition to the standard treatment for glioblastoma. TTF therapy, delivered via a wearable device called Optune Gio, uses alternating electric fields to disrupt cell division, thereby inhibiting tumor growth.

The Optune device uses adhesive pads with electrodes that are applied directly to the skin. These are called transducer arrays and typically four of them are applied to the scalp.

In order to be applied, you need to shave your head prior to the using Optune. The Optune pads are worn continuously throughout the day for a long as possible, with minimal interruptions or breaks. For Optune to be maximally effective, studies have shown the pads need to be worn and activated for at least 18 hours each day, seven days a week.

The Optune device can be used for up to 24 months or until the disease progresses twice.

The pivotal trial for tumor treating fields demonstrated that adding to maintenance Temozolomide therapy extended both progression-free survival and overall survival compared to Temozolomide alone.

The use of Optune does not increase the rate of side effects from chemotherapy. In regards to Optune specifically, it is common to experience mild to moderate skin irritation on the scalp when using the device, including itching and rash. It is extremely uncommon to experience a severe skin reaction.

Supportive and Palliative Care

In addition to the active treatment of surgery, chemoradiation, and chemotherapy with tumor treating fields, supportive care is essential for managing the symptoms and side effects associated with glioblastoma and its treatments.

This can include:

Corticosteroids: Used to reduce brain swelling (edema) and alleviate symptoms such as headaches or neurological deficits.
Antiepileptic drugs: Seizures are common in glioblastoma patients, and antiepileptic medications are used to prevent or control them.
Physical, occupational, and speech therapy: To help patients regain function or adapt to neurological impairments.
Palliative care: Focuses on improving quality of life and managing symptoms such as pain, fatigue, and depression. Palliative care is integrated into the treatment plan from the time of diagnosis and becomes more prominent as the disease progresses.

Ongoing Research & Hope for the Future

Research is continuously evolving, and scientists are learning more about how to target these difficult cancer stem cells. Dr. Reid Thompson, Chair of Neurosurgery at Vanderbilt University Medical Center, previously told SurvivorNet that one promising area of research involves understanding the unique “fingerprint” of each patient’s tumor at a molecular level. By studying these fingerprints, or molecular profiles, doctors hope to find new treatments that specifically target the cells most likely to cause recurrence.

Dr. Thompson expressed optimism for the future: “There’s a lot of cool insight happening on the research front, and it gives those of us who take care of patients with gliomas and glioblastomas a lot of hope because it gives us hope for the future.”

In summary, gliomas are challenging to treat because they spread into nearby healthy brain tissue and often contain cancer stem cells. These characteristics make it difficult to completely resect them and resistant to standard treatments. Surgery, radiation, and chemotherapy each play a role in managing the tumor, but even with these approaches, gliomas can return.

Researchers like Dr. Thompson are continually searching for better ways to target these stubborn cells and prevent recurrence, bringing hope for new, more effective treatments in the future.

Dr. Thompson emphasized to SurvivorNet that each glioma is unique, and understanding these differences is key to advancing treatment options. “No two gliomas are exactly the same,” he says, highlighting the importance of individually tailored treatment approaches.

With the right information, patients and families can feel more empowered and better prepared for the challenges that may lie ahead in the journey of glioma treatment.