Spinal Cord Ependymoma in the Prime of Life
In January 2007, Erik Kratky was 21 and pursuing his undergraduate degree when he experienced bilateral leg weakness, which was later diagnosed as low-grade spinal cord ependymoma.
In January 2007, Erik Kratky was 21 and pursuing his undergraduate degree when he experienced bilateral leg weakness. Magnetic resonance imaging (MRI) revealed multiple spinal cord lesions in the T10 region and, after surgical resection, Erik was diagnosed with low-grade (II) spinal cord ependymoma.
He did well for 2 years and went on to graduate college, but in November 2009, he experienced lower-extremity weakness requiring confinement to a wheelchair. Imaging revealed tumor recurrence at the original site, as well as new tumor growth at T5, T6, and the upper level of T7, along with cord compression. T5/T6/T7 and T9/T10/T11 laminectomy was performed with resection of intradural extramedullary tumor, and pathology again revealed grade II disease.
Erik failed to improve postoperatively, and imaging revealed another recurrence, continued cord compression, and new tumor growth in the lumbar region (L1 and L4), consistent with dropped metastasis. He underwent resection of tumor in T9 through T12, and the ependymoma was upgraded to III-IV, with pathology suggestive of highly aggressive disease.
The following month, Erik was referred to the Cleveland Clinic. He underwent 4 1/2 weeks of whole-brain and spine radiation (5 days/week; 20 treatments with a planned boost), along with a total of three infusions of bevacizumab (Avastin) administered once every other week to help manage spinal cord edema and reduce further loss of leg function.
Erik did well for about 1 year, completing his graduate studies, teaching college-level math, and, with the help of physical and occupational therapy, walking with a walker. An MRI in June 2011 revealed diffuse progression of tumor in the lumbar region, and Erik began one 28-day cycle of oral lapatinib (INN; daily) and oral temozolomide (Temodar; days 1-7, repeated on days 15-21).
During a July hospitalization for a pain crisis, scanning demonstrated tumor progression in the cauda equina with spinal cord nerve irritation. Erik was discharged after single-dose emergent radiation and treatment with dexamethasone (Decadron), but within 1 month, repeat imaging demonstrated continued interval progression, necessitating a rethinking of options.
Erik was put back on intravenous (IV) bevacizumab (every 2 weeks), and a carboplatin (Paraplatin) infusion was added every 4 weeks. The carboplatin was discontinued in May 2012 due to myelosuppression, and today Erik remains on twicemonthly infusions of bevacizumab, which he tolerates well. While recent imaging shows an absence of tumor growth, Erik has sustained significant injury to his spinal cord and is currently confined to a wheelchair.
Tanya Tekautz, MD
Because ependymoma is not especially chemotherapy- sensitive, and residual malignant cells are likely after resection, tumor resection followed by radiotherapy is considered the most effective treatment for preventing recurrence. Tanya Tekautz, MD, oncologist at the Cleveland Clinic Rose Ella Burkhardt Brain Tumor and Neuro-Oncology Center and director of the Pediatric and Young Adult Brain Tumor Program, explains that despite the absence of brain metastasis, irradiation of Erik’s spine and brain was considered the standard of care in light of the likelihood of metastasis of small cancer cells not seen on imaging.
Burkhardt Center nurse clinician Mary Murphy, RN, BSN, notes that Erik tolerated the bevacizumab relatively well, and didn’t develop serious adverse effects (eg, brain hemorrhage, unilateral lower-extremity thrombi) despite his increased risk for clotting due to being nonambulatory. “We watch carefully for signs of brain hemorrhage, such as garbled speech and other cognitive deficits,” says Murphy, “and monitor the lower extremities for swelling, redness, and pain.” Because some of these symptoms can be insidious, Tekautz sees patients at every chemotherapy treatment, rather than every 2 months after scanning. Eric recalls the radiation-related esophagitis as the most difficult of the adverse effects he endured.
During Erik’s June 2011 disease progression, oral chemotherapy allowed him to be up and around to the extent possible. But with limited options after the 2012 progression, the change was made to IV bevacizumab and carboplatin, based primarily on data from small studies showing that the combination can help to control or slow tumor growth in ependymoma patients previously treated with surgery and radiation. “As was the case with Erik, carboplatin can cause severe myelosuppression. It also can cause renal toxicity, increased risk of infection, nausea, and fatigue, requiring frequent clinic assessment and monitoring with twice-monthly labs,” says Murphy.
Mary Murphy, RN, BSN
It was at this time, at age 25, that Erik was transferred from pediatric to adult care.
Moving from Pediatric to Adult Care
“Aging out” of the pediatric population and transitioning to adult care can pose unique challenges. For Erik, who had developed strong attachments to his pediatric nurses, the change was a difficult one. “The adult care staff is great, but the pediatric environment was incredibly nurturing for me and my parents,” says Eric.
For Eric, the balance between the desire for independence and the need for compassionate care has been difficult to achieve, especially in light of his disability, which necessitated a return to his parents’ home just as his adult life had begun. “It’s the disability that’s hardest to deal with. I had to relearn how to do everything,” he says. Erik credits his family, Tekautz, Murphy, and all of his nurses for helping him to maintain a positive attitude.
Each time it progresses, Erik’s disease becomes more difficult to treat. While reluctant to make any long-term predictions, Murphy and Tekautz note that the current goal is to control any tumor growth for as long as possible in a patient they describe as “magic to everyone who knows him.”
Laura Bruck is a freelance writer and editor based in Cleveland, Ohio. She has specialized in healthcare reporting since 1987.