Clinical Course of Adult Patients With Ependymoma

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1 Clinical Course of Adult Patients With Ependymoma Results of the Adult Ependymoma Outcomes Project Terri S. Armstrong, PhD 1,2 ; Elizabeth Vera-Bolanos, MS 2 ; and Mark R. Gilbert, MD 2 BACKGROUND: Ependymomas are a rare tumor in adults, and there are limited reports of the clinical course, treatment, and current health status of patients. METHODS: Patients with ependymoma completed an online survey regarding their diagnosis, treatment course, and current health status. Descriptive statistics were used to report the characteristics and degree of symptom severity. Correlations between demographic and clinical characteristics were explored with univariate analysis. RESULTS: One hundred eighteen adults participated, and there were more women (n ¼ 68) than men (n ¼ 50). The median age was 48 years (range, years). Fifty-nine participants (48%) reported that they had not been able to work since their diagnosis, and 31% reported receiving disability benefits. Patients who had been treated for spine tumors had symptoms longer than those who had been treated for brain lesions (chi-square statistic, 7.294; P ¼.026), and the majority had 3 symptoms before diagnosis. Most patients reported undergoing complete resection (brain lesions, 62%; spine tumors, 58%), and patients with spine tumors were less likely to have received additional treatment (chi-square statistic, 9.687; P ¼.008). The majority had not had a recurrence and reported consulting a neurosurgeon for surveillance. Despite having stable disease and not receiving active treatment, most patients described moderate to severe symptoms, including fatigue (44%), numbness/tingling (39%), pain (36%), and disturbed sleep (34%) overall. Brain lesions were associated with altered vision (25%), difficulty concentrating (25%), weakness (19%), irritability (19%), difficulty speaking (19%), and understanding (17%); and spine lesions were associated with extremity weakness (55%), sexual dysfunction (48%), radiating pain (37%), and change in bowel pattern (35%). CONCLUSIONS: Treatment for adult patients with ependymoma is not standardized. Despite the low recurrence rate, patients reported significant symptoms and disability. Cancer 2011;117: VC 2011 American Cancer Society. KEYWORDS: central nervous system, tumor, symptoms, cancer treatment, quality of life. Ependymomas are rare central nervous system (CNS) tumors in adults, comprising 2% to 8% of all primary adult CNS tumors diagnosed in the United States each year. 1 According to the World Health Organization classification system, neoplasms are classified as either very low grade (grade 1 [myxopapillary]), low grade (grade 2), or high grade (grade 3 or anaplastic). Ependymomas can occur throughout the CNS. In adults, ependymomas appear most commonly in the spinal cord and as grade 1 or2 tumors. 2 Although they are less common overall, anaplastic ependymomas (World Health Organization grade 3) tend to occur in the brain and are rare in the spine. The approach to treatment in adults is not standardized; however, patients typically undergo maximal safe tumor resection. Subsequent treatment at initial diagnosis and recurrence with radiation and/or chemotherapy varies based on the extent of resection, the recurrence pattern, and the degree of anaplasia and is reserved for patients who have more malignant anaplastic ependymomas. 3,4 Because of the relative rarity of ependymoma, there are few reports of the pathologic characteristics, tumor location, and clinical course in adult patients, and most include small numbers of patients. 5-8 There are even fewer reports of symptomatology at presentation and the impact of the disease and its treatment on the health status of patients, including those with long-term tumor control Because these tumors are rare and patients often have extended periods of remission, Corresponding author: Terri S. Armstrong, PhD, ANP-BC, FAANP, 6901 Bertner Avenue, Room 791, Houston, TX 77030; Fax: (713) ; terri.s. armstrong@uth.tmc.edu 1 University of Texas Health Science Center-Houston School of Nursing, Houston, Texas; 2 Neuro-Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas. DOI: /cncr.26181, Received: January 7, 2011; Revised: February 22, 2011; Accepted: March 23, 2011, Published online April 28, 2011 in Wiley Online Library (wileyonlinelibrary.com) Cancer November 15,

2 a wide variety of medical disciplines, including neurosurgeons, family practitioners, internists, neurologists, and radiation oncologists, among others, are involved in the treatment and follow-up of these patients. The Ependymoma Outcome (EO) Project was designed by investigators in the Collaborative Ependymoma Research Network (CERN) as an online questionnaire for patients and their caregivers with the goal of gathering information on outcomes for adult patients with ependymoma. The key objectives of the EO Project are to gather information on the treatment strategies and health status of adult patients with ependymoma; to disseminate information to health care providers on the symptoms, treatment, and outcomes of patients with ependymoma with the goal of improving patient management; to publish objective data on trends and regional differences in the care of adult patients who have ependymoma with the goal of improving and standardizing care; and to develop ideas based on these data for future clinical trials with the goal of improving patient survival and outcome. MATERIALS AND METHODS The EO Project opened for enrollment in January 2009 through the CERN Foundation website ( accessed April 11, 2011). The questionnaire can be viewed in its entirety on the CERN Foundation website ( accessed April 11, 2011). Participants initially completed an enrollment form and were then sent a unique participant identification number and were given access to the electronic EO survey. Two groups participated in the survey. The first group consisted of patients with ependymoma or an ependymoma variant who had attended a consultation at The University of Texas MD Anderson Cancer Center in Houston, Texas between 1995 and 2010, and the second group consisted of patients who registered through the website without solicitation. The survey consists of a series of 84 questions related to demographic characteristics, clinical presentation, tumor location, treatments received, and current health status. In addition, the questionnaire includes the MD Anderson Symptom Inventory-Brain Tumor Module (MDASI-BT) and the MD Anderson Symptom Inventory-Spine Tumor Module (MDASI-SP), which allow patients to report current symptoms and the interference of symptoms with daily life. The MDASI-BT and MDASI-SP consist of symptoms (23 in the MDASI-BT and 18 in the MDASI-SP) and 6 interference items rated on an 11-point scale from 0 to 10 to indicate the presence and severity of each symptom in the last 24 hours, with 0 indicating not present and 10 indicating as bad as you can imagine. 11,12 The symptoms included on the instrument are those commonly associated with cancer therapies and those symptoms common in the brain and spine tumor patient population. The MDASI-BT has evidence of content and construct validity, discriminant validity by performance status and disease progression, and internal consistency (Cronbach alpha ¼.92). 11 The internal consistency (reliability) of the MDASI-SP is 0.946, and the instrument reportedly was sensitive to disease severity based on Karnofsky performance status (KPS) in a group of patients with both primary and metastatic spinal cord tumors. 12 The statistical analysis was performed using the SPSS software package (version 17.0; SPSS Inc., Chicago, Ill). Descriptive statistics were used to report demographic and clinical characteristics. For symptom data, symptom severity and interference were categorized into clinically meaningful groups based on work by Serlin and colleagues 13 for pain severity cutoff scores and using the methods of Mendoza and colleagues 14 for fatigue severity cutoff scores. We dichotomized mean symptom severity into none/mild (scores from 0 to 3) and moderate/severe (scores from 4 to 10). Correlations between demographic and clinical characteristics were explored with univariate analysis using chi-square tests for categorical variables and independent-sample t tests for continuous variables. RESULTS Participation In total, 118 patients had completed the survey by August 2010 and were included in the current analysis. One hundred thirty-six invitations were sent out to the MD Anderson Cancer Center group, and 59 invited patients (43%) completed the EO survey. One hundred five adults registered without solicitation through the CERN website, and 59 (56%) of those patients completed the survey in that group. Most respondents completed the survey without assistance. Eight patients required help from a family or friend, 10 surveys were completed by a family member, and 1 patient required help from a health care provider to complete the survey. Patient Characteristics Demographic characteristics of the sample are presented in Table 1. Among the participants, there were more 5134 Cancer November 15, 2011

3 EO Project/Armstrong et al Table 1. Demographic Characteristics Characteristic Overall BT ST BT and ST Sex Men 50 (42) 22 (42) 26 (43) 2 (33) Women 68 (58) 30 (58) 34 (57) 4 (67) Current age, y Median Mean Range Age at diagnosis, y Median Mean Range Marital status Never married 16 (14) 11 (21) 4 (7) 1 (17) Married 88 (75) 37 (71) 46 (77) 5 (83) Divorced 7 (6) 0 (0) 7 (12) 0 (0) Widowed 1 (1) 1 (2) 0 (0) 0 (0) Separated 1 (1) 0 (0) 1 (2) 0 (0) NA 5 (4) 3 (6) 2 (3) 0 (0) Education Some high school 2 (2) 2 (4) 0 (0) 0 (0) High school graduate 8 (7) 3 (6) 5 (8) 0 (0) Some college 25 (21) 11 (21) 12 (20) 2 (33) College graduate 40 (34) 20 (39) 20 (33) 0 (0) Postgraduate 39 (33) 14 (27) 21 (35) 4 (67) NA 4 (3) 2 (4) 2 (3) 0 (0) Income <$20,000 5 (4) 4 (8) 1 (2) 0 (0) $20-$59, (22) 11 (21) 14 (23) 0 (0) $60-$99, (17) 12 (23) 6 (10) 2 (33) $100, (39) 17 (33) 25 (42) 4 (67) Prefer not to say 16 (14) 4 (8) 12 (20) 0 (0) NA 6 (5) 4 (8) 2 (3) 0 (0) Currently employed No 60 (51) 31 (60) 28 (47) 1 (17) Full time 42 (36) 15 (29) 24 (40) 3 (50) Part time 9 (8) 3 (6) 4 (7) 2 (33) NA 7 (6) 3 (6) 4 (7) 0 (0) Changed jobs since diagnosis Different job 21 (18) 4 (8) 16 (27) 1 (17) Same job 35 (30) 15 (29) 16 (27) 4 (67) Not working 56 (48) 29 (56) 26 (43) 1 (17) NA 6 (5) 4 (8) 2 (3) 0 (0) Receiving disability benefits No 76 (64) 30 (58) 41 (68) 5 (83) Yes 37 (31) 19 (37) 17 (28) 1 (17) NA 5 (4) 3 (6) 2 (3) 0 (0) Abbreviations: BT, brain tumor; NA, not available; ST, spinal tumor. women (n ¼ 68) than men (n ¼ 50), and the median age at the time the survey was completed was 48 years (range, years). The majority of patients had a college education, and >50% reported a household income $100,000. Sixty patients (51%) were not employed outside the home. Cancer November 15,

4 Table 2. Clinical Characteristics Characteristic Overall BT ST BT and ST Tumor location Brain 52 (44) Spine 60 (51) Brain and spine 6 (5) Tumor type Ependymoma 69 (59) 30 (58) 35 (58) 4 (67) Anaplastic ependymoma 14 (12) 12 (23) 2 (3) 0 (0) Myxopapillary ependymoma 16 (14) 0 (0) 15 (25) 1 (17) Subependymoma 4 (3) 3 (6) 1 (2) 0 (0) Tancytic ependymoma 2 (2) 2 (4) 0 (0) 0 (0) Giant cell ependymoma 2 (2) 0 (0) 1 (2) 1 (17) Did not know name 10 (9) 5 (10) 5 (8) 0 (0) NA 1 (1) 0 (0) 1 (2) 0 (0) Location Brain Left 8 (15) 0 (0) Right 13 (25) 2 (33) Midline 4 (8) 0 (0) NA 30 (58) 4 (67) Supratentorial 16 (31) 0 (0) Infratentorial 27 (52) 5 (83) Both 2 (4) 0 (0) NA 7 (13) 1 (17) Spine Cervical 25 (42) 4 (67) Thoracic 19 (32) 1 (17) Lumbar 21 (35) 1 (17) Sacral 9 (15) 1 (17) NA 2 (3) 1 (17) No. of tumor sites at diagnosis Single 98 (83) 41 (79) 52 (87) 5 (83) Multiple 11 (9) 4 (8) 6 (10) 1 (17) Did not know 6 (5) 5 (10) 1 (2) 0 (0) NA 3 (3) 2 (4) 1 (2) 0 (0) Abbreviations: BT, brain tumor; NA, not available; ST, spinal tumor. Tumor Information Most patients who participated in the survey had been diagnosed for several years before completing the survey (brain tumor: median, 50 months; range, months; spine tumor: median, 74 months; range months). The median age at diagnosis was 40 years (range years). Most patients had grade 2 ependymomas; however, for grade 3 (anaplastic) ependymomas, there was a strong association with brain location (chi-square statistic, ; P ¼.004). Patients with brain lesions most commonly had the tumor located infratentorially along the brainstem (52%), whereas those with spine tumors most commonly had tumors located in the cervical spine (44%). Table 2 outlines information related to diagnosis. Clinical Presentation The majority of patients had 3 symptoms before diagnosis (range, 0-10 symptoms). Common symptoms leading to diagnosis for those with brain tumors included headache (52%), visual problems (46%), nausea/vomiting (39%), weakness (33%), and numbness/tingling (33%); and 37% reported that they had symptoms for >6 months. In patients with spine tumors, numbness/tingling (58%), weakness (45%), back pain (35%), and radiating back pain (27%) were the most common symptoms; and 64% had experienced symptoms for >6 months before diagnosis. There was a significant difference in the length of time between presenting symptoms for patients who had spine tumors compared with patients who had brain tumors (chi-square statistic, 7.294; P ¼.026) Cancer November 15, 2011

5 EO Project/Armstrong et al Table 3. Treatment Course Treatment Overall BT ST BT and ST Initial surgery Biopsy 2 (2) 2 (4) 0 (0) Partial resection 19 (16) 18 (35) 1 (17) Gross total resection 35 (30) 32 (62) 3 (50) Spine surgery for biopsy 5 (4) 5 (8) 0 (0) Spine surgery with partial resection 13 (11) 13 (22) 0 (0) Spine surgery with gross total resection 37 (31) 35 (58) 2 (33) No surgery performed 2 (2) 2 (3) 0 (0) NA 5 (4) 0 5 (8) 0 (0) Condition after first surgery Better 61 (52) 34 (65) 24 (40) 3 (50) Worse 38 (32) 12 (23) 25 (42) 1 (17) Same 12 (10) 2 (4) 9 (15) 1 (17) NA 7 (6) 4 (8) 2 (3) 1 (17) Complications after first surgery Infection 12 (10) 6 (12) 4 (7) 2 (33) Blood clot 6 (5) 4 (8) 2 (3) 0 (0) Bleeding in brain 3 (3) 2 (4) 0 (0) 1 (17) Seizure 7 (6) 6 (12) 0 (0) 1 (17) Diabetes 5 (4) 2 (4) 2 (3) 1 (17) Drug allergy 6 (5) 2 (4) 3 (5) 1 (17) Weakness 45 (38) 17 (33) 24 (40) 4 (67) Paralysis 23 (20) 6 (12) 16 (27) 1 (17) Inability to urinate 10 (9) 2 (4) 7 (12) 1 (17) Incontinence 14 (12) 1 (2) 11 (18) 2 (33) Sexual dysfunction 22 (19) 5 (10) 17 (28) 0 (0) Additional treatment after surgery Radiation therapy 30 (25) 19 (37) 10 (17) 1 (17) Chemotherapy and radiation therapy 13 (11) 8 (15) 4 (7) 1 (17) Chemotherapy, radiation therapy, and radiosurgery 2 (2) 2 (4) 0 (0) 0 (0) None 64 (54) 22 (42) 38 (63) 4 (67) NA 9 (8) 1 (2) 8 (13) 0 (0) Abbreviations: BT, brain tumor; NA, not available; ST, spinal tumor. Treatment Course Most commonly, patients reported that surgery resulted in a complete resection for both brain tumors (62%) and spine tumors (58%). Patients who had spine tumors were more likely to report receiving no additional treatment after surgery compared with patients who had brain tumors (chi-square statistic, 9.687; P ¼.008). Sixty-five percent of patients with brain lesions and 40% of those with spine lesions reported improvement in their overall condition after surgery. Common complications after surgery for those with brain lesions included weakness (33%), seizures (12%), and infection (12%); whereas, for patients who underwent surgery for spine tumors, the most common postsurgical complications included weakness (40%), sexual dysfunction (28%), and incontinence (18%) (Table 3). Tumor Recurrence At the time the survey was completed, 31% of patients with brain lesions and 27% of patients with spine tumors reported having experienced a recurrence of their tumor. It is noteworthy that the majority of those with spine tumors had experienced >2 recurrences (56%), whereas most patients with brain tumors experienced only 1 recurrence (50%). The most common symptoms at recurrence included numbness or tingling in the arms, legs, or body (brain tumors, 25%; spine tumors, 50%); headache (brain tumors, 25%); and back pain (spine tumors, 44%). Most recurrences occurred in the location of the original tumor, and a variety of treatment approaches were employed, including surgery, radiation, and chemotherapy. Six percent of patients reported that they received chemotherapy alone (27% received it alone or in combination with Cancer November 15,

6 Table 4. Recurrence Information Variable Overall BT ST BT and ST Has the ependymoma recurred? No 80 (68) 35 (67) 40 (67) 5 (83) Yes 33 (28) 16 (31) 16 (27) 1 (17) NA 5 (4) 1 (2) 4 (7) 0 (0) What was the type of ependymoma at recurrence? Ependymoma 10 (30) 6 (38) 3 (19) 1 (100) Myxopapillary ependymoma 9 (27) 0 (0) 9 (56) 0 (0) Anaplastic ependymoma 7 (21) 5 (31) 2 (13) 0 (0) Did not know name 5 (15) 3 (19) 2 (13) 0 (0) NA 2 (6) 2 (13) 0 (0) 0 (0) How many times has your ependymoma recurred? 1 13 (39) 8 (50) 4 (25) 1 (100) 2 4 (12) 3 (19) 1 (6) 0 (0) >2 11 (33) 2 (13) 9 (56) 0 (0) NA 5 (15) 3 (19) 2 (13) 0 (0) Did it recur in the same location? No 7 (21) 5 (31) 2 (13) 0 (0) Yes 25 (76) 10 (63) 14 (87) 1 (100) NA 1 (3) 1 (6) 0 (0) 0 (0) Symptoms at recurrence Numbness/tingling of legs, arms, or body 13 (39) 4 (25) 8 (50) 1 (100) Headache 6 (18) 4 (25) 2 (13) 0 (0) Weakness 7 (21) 0 (0) 7 (44) 0 (0) Back pain 8 (24) 1 (6) 7 (44) 0 (0) Radiating back pain 6 (18) 0 (0) 6 (38) 0 (0) Treatment at recurrence Surgery only 8 (24) 2 (13) 6 (38) 0 (0) Radiation only 9 (27) 3 (19) 6 (38) 0 (0) Chemotherapy only 2 (6) 2 (13) 0 (0) 0 (0) Radiation and chemotherapy 1 (3) 1 (6) 0 (0) 0 (0) Surgery and radiation 4 (12) 2 (13) 1 (6) 1 (100) Surgery and chemotherapy 3 (9) 3 (19) 0 (0) 0 (0) Surgery, radiation, and chemotherapy 3 (9) 0 (0) 3 (19) 0 (0) NA 3 (9) 3 (19) 0 (0) 0 (0) Abbreviations: BT, brain tumor; NA, not available; ST, spinal tumor. radiation or surgery plus radiation), and 51% received chemotherapy with radiation therapy for tumor recurrence. Table 4 outlines these results. Follow-Up The majority of patients reported consulting a neurosurgeon for follow-up of their ependymoma (brain tumors, 35%; spine tumors, 55%), but the other patients also reported being followed with a variety of other disciplines, including family physicians or internists (see Table 5). Surprisingly, >66 separate providers, the majority of which were community-based clinics or hospitals, were listed by the 118 participants in this survey, supporting the contention that patient care is not centralized and that patients are not being treated at major academic centers. The majority of patients reported having a magnetic resonance image (MRI) to evaluate disease status on a yearly basis, and 70% had an MRI to evaluate their disease status within the last year. Fifty-three percent reported that there was no evidence of disease at the time of their last MRI. Although the survey participants reported the use of a wide variety of medications, commonly prescribed medications included anticonvulsants for those with brain tumors (21%) and narcotic analgesics for those with spine tumors (47%). Thirty percent also reported using complementary and alternative medicine therapy, including high-dose vitamins, acupuncture, and a variety of herbal preparations Cancer November 15, 2011

7 EO Project/Armstrong et al Table 5. Type of Physician Managing Patients Type of Physician Overall BT ST BT and ST Neurosurgeon 52 (44) 18 (35) 33 (55) 1 (17) Neuro-oncologist 35 (30) 19 (37) 12 (20) 4 (67) Family physician or internal medicine 21 (18) 5 (10) 16 (27) 0 (0) Radiation oncologist 18 (15) 11 (21) 6 (10) 1 (17) Oncologist 8 (7) 4 (8) 4 (7) 0 (0) Abbreviations: BT, brain tumor; ST, spinal tumor. Symptom Burden and Current Health Status The patient s current symptoms and the impact of these symptoms on daily life also were evaluated. Despite the low percentage of patients who had recurrent tumors or who were receiving active treatment, most patients described moderate-to-severe symptoms. For the group overall, the most severe general symptoms included fatigue (44%), numbness/tingling (39%), pain (36%), and disturbed sleep (34%). Patients with tumors involving the brain reported moderate-to-severe problems with vision (25%), concentration (25%), weakness (19%), irritability (19%), difficulty speaking (19%), and understanding (17%). Patients with spine ependymomas reported moderate-to-severe extremity weakness (55%), sexual dysfunction (48%), radiating pain (37%), and change in bowel pattern (35%). Participants were asked to describe their current employment and disability status in addition to current health insurance coverage. Overall, 59 patients (48%) reported that they had not been able to work since their diagnosis, and 37 patients (31%) reported that they were receiving disability benefits. The majority (53%) reported having a commercial insurance (n ¼ 62), and the next most common medical coverage was Medicare (n ¼ 32). Eighty-one percent of participants reported that they believed they had access to all the health care services that they needed (n ¼ 96), and 73% reported that their insurance was very helpful in providing payments for these services (n ¼ 86). Participants were also asked to report on their quality of life and activities. Only a few patients (n ¼ 19) reported that they were not at all content with the quality of their life and were unable to enjoy leisure pursuits (n ¼ 17). However, 26 patients (22%) reported they were unable to drive, and nearly all patients (n ¼ 98) reported that their health prevented them from participating in vigorous activities, such as running or lifting heavy objects. Seventy-two patients (61%) reported that even moderate activities (such as vacuuming) were impacted by their current health status. Participants also reported that their current health status impacted their ability to walk 1 block (n ¼ 41; 35%) or climb a flight of stairs (n ¼ 44; 37%). One-third of patients (n ¼ 33) reported that they needed assistance with bathing or dressing on a daily basis. DISCUSSION The EO Project survey was completed by approximately half of the patients who were identified as eligible for participation. It is unclear why some patients chose not to participate, and we cannot determine the impact of the responses these patients would have had on the overall survey results. The majority of patients, as expected, were diagnosed with low-grade ependymoma, and anaplastic ependymomas were much less frequent. Consistent with disease incidence, anaplastic ependymomas occur more commonly in the brain than in the spinal cord, and most patients had a solitary lesion at the time of diagnosis. A significant limitation of this study s approach, however, is that the treatment and diagnosis information provided was not validated by a review of the medical record. In addition, the length of time from diagnosis (median, 50 months) may have resulted in recall bias of self-reported presentation, treatment, and complication data. Patients reported that they experienced an average of 3 symptoms often for several months before diagnosis, and some patients reported having symptoms for several years before their diagnosis was made. It is unclear when evaluation by a health care provider was sought or whether patients were treated for other conditions in response to the patient s report of symptoms. It is noteworthy that, on average, patients who had tumors involving the spine had symptoms for a significantly longer time than patients who had Cancer November 15,

8 ependymomas involving the brain. A recent report from Kucia and colleagues also indicated a long duration of symptoms (average, 22.2 months) in a small group of patients with spinal cord ependymoma, including bowel/bladder dysfunction and weakness. 10 These data may have clinical significance, because early recognition of the diagnosis may impact therapeutic outcomes, as demonstrated in a recent report that baseline McCormick status was a predictor of postoperative neurologic dysfunction. 9 Future studies to improve earlier diagnosis should explore whether patients self-manage their symptoms or seek out evaluation in the health care system. Our survey indicated that the majority of patients underwent surgery as their only treatment. However, variations in treatment approach were identified: Twentyfive percent of patients received radiation only, and others received a variety of regimens that combined radiation and chemotherapy. These findings lend further support to the lack of standardization in treatment of these patients. Consequently, it is difficult to evaluate the impact of therapies on the ultimate clinical outcome either from the time of initial diagnosis or in patients with recurrent tumors. Although the majority of patients reported that they had not had a recurrence of their tumor, nearly 40% of patients reported that they were unable to work from the time of diagnosis and reported significant symptoms, such as pain, weakness, cognitive changes, and sexual dysfunction. In addition, the majority reported that their current health status limited their daily functions and leisure activities. There are several important implications of these findings. Ependymomas typically are considered a more benign tumor, and in this context, along with the relative rarity of the disease, the impact on patient function, ability to work, and cost of care has not been explored. The findings from this survey suggest that significant numbers of patients are impacted by their disease even when the tumor has not recurred. Nearly half of the patients with spine tumors reported needing narcotic analgesics to manage pain, and >33% had significant issues with weakness, bowel function, and sexual function. Future studies should explore the relation of symptoms to diagnosis and treatment approach and should prompt improvements in symptom management strategies. The EO Project provides a unique perspective on the presentation, clinical management, and current health status of adult patients with ependymoma from the perspective of the patient. The benefits of this analysis include sampling from a wide region rather than a single institution and the number of respondents, considering the relative rarity of the diagnosis. This information would have been difficult to obtain otherwise because of the large number and wide variety of physician specialties that are currently providing care and follow-up of these patients, as indicated by the survey. These preliminary results suggest that that the impact of this more benign tumor may be significant in terms of disability and ongoing symptomatology. Future studies are underway to further explore the treatments patients undergo as well as the economic and health impact of ependymoma in adult patients. FUNDING SOURCES This work was supported by the Collaborative Ependymoma Research Network (CERN) Foundation. CONFLICT OF INTEREST DISCLOSURES The authors made no disclosures. REFERENCES 1. Central Brain Tumor Registry of the United States (CBTRUS). CBTRUS Statistical Report: Primary Brain and Central Nervous System Tumors Diagnosed in the United States in Hinsdale, IL: CBTRUS; Verstegen MJ, Bosch DA, Troost D. Treatment of ependymomas. Clinical and non-clinical factors influencing prognosis: a review. Br J Neurosurg. 1997;11: Armstrong TS, Vera-Bolanos E, Bekele BN, Aldape K, Gilbert MR. Adult ependymal tumors: prognosis and the M.D. Anderson Cancer Center experience. Neuro Oncol. 2010; 12: Vitanovics D, Balint K, Hanzely Z, Banczerowski P, Afra D. Ependymoma in adults: surgery, reoperation and radiotherapy for survival. Pathol Oncol Res. 2010;16: Reni M, Brandes AA. Current management and prognostic factors for adult ependymoma. Expert Rev Anticancer Ther. 2002;2: Donahue B, Steinfeld A. Intracranial ependymoma in the adult patient: successful treatment with surgery and radiotherapy. J Neurooncol. 1998;37: Shaw EG, Evans RG, Scheithauer BW, Ilstrup DM, Earle JD. Postoperative radiotherapy of intracranial ependymoma in pediatric and adult patients. Int J Radiat Oncol Biol Phys. 1987;13: Morris K, Lye RH. Ependymoma of the fourth ventricle: an unusual presentation. Br J Neurosurg. 1988;2: Kucia EJ, Bambakidis NC, Chang SW, Spetzler RF. Surgical technique and outcomes in the treatment of spinal cord ependymomas part I. Intramedullary ependymomas 5140 Cancer November 15, 2011

9 EO Project/Armstrong et al [published online ahead of print December 30, 2010]. Neurosurgery Kucia EJ, Maughan PH, Kakarla UK, Bambakidis NC, Spetzler RF. Surgical technique and outcomes in the treatment of spinal cord ependymomas: part II: myxopapillary ependymoma. Neurosurgery. 2011;68(1 suppl operative):90-94; discussion Armstrong TS, Mendoza T, Gning I, et al. Validation of the M.D. Anderson Symptom Inventory Brain Tumor Module (MDASI-BT). J Neurooncol. 2006;80: Armstrong TS, Gning I, Mendoza TR, et al. Reliability and validity of the M.D. Anderson Symptom Inventory-Spine Tumor Module. J Neurosurg Spine. 2010;12: Serlin RC, Mendoza TR, Nakamura Y, Edwards KR, Cleeland CS. When is cancer pain mild, moderate or severe? Grading pain severity by its interference with function. Pain. 1995;61: Mendoza TR, Wang XS, Cleeland CS, et al. The rapid assessment of fatigue severity in cancer patients: use of the Brief Fatigue Inventory. Cancer. 1999;85: Cancer November 15,