Resection of disseminated recurrent myxopapillary ependymoma with more than 4-year follow-up: operative nuance for prolonged prone position. Illustrative case

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  • 1 University of Minnesota Medical School, Minneapolis, Minnesota;
  • | 2 Departments of Neurological Surgery
  • | 3 Radiation Oncology, and
  • | 4 Bioinformatics and Computational Biology, University of Minnesota, Minneapolis, Minnesota; and
  • | 5 Surgical Services, Minneapolis VA Health Care System, Minneapolis, Minnesota
Open access

BACKGROUND

Symptomatic disseminated myxopapillary ependymoma (MPE) in a young person presents a daunting challenge because the risks of prolonged prone positioning and spinal cord injury may outweigh the likelihood of attaining the benefit of gross total resection.

OBSERVATIONS

The authors reported the case of a 15-year-old girl with five discrete recurrent spinal cord ependymomas. The patient received a 25-hour surgical procedure for gross total resection of the tumors and fusion over an approximately 33-hour period. She experienced complete resolution of all preoperative neurological symptoms and subsequently received adjuvant radiation therapy. At 52 months after surgery, she was still experiencing neurologically intact, progression-free survival. This case illustrated one of the most extensive recurrent tumor resections for MPE with prolonged disease-free survival reported to date. It may also represent the longest prone position spinal case reported and was notable for a lack of any of the complications commonly associated with the prolonged prone position.

LESSONS

The authors discussed the complexity of surgical decision-making in a symptomatic patient with multiple disseminated metastases, technical considerations for resection of intradural and intramedullary spinal cord tumors, and considerations for avoiding complications during prolonged positioning necessary for spinal surgery.

ABBREVIATIONS

EBL = estimated blood loss; FFP = fresh frozen plasma; GTR = gross total resection; LR = lactated Ringer’s; MPE = myxopapillary ependymoma; MRI = magnetic resonance imaging; POVL = perioperative vision loss; RBC = red blood cell; STR = subtotal resection; RT = radiation therapy; WHO = World Health Organization

BACKGROUND

Symptomatic disseminated myxopapillary ependymoma (MPE) in a young person presents a daunting challenge because the risks of prolonged prone positioning and spinal cord injury may outweigh the likelihood of attaining the benefit of gross total resection.

OBSERVATIONS

The authors reported the case of a 15-year-old girl with five discrete recurrent spinal cord ependymomas. The patient received a 25-hour surgical procedure for gross total resection of the tumors and fusion over an approximately 33-hour period. She experienced complete resolution of all preoperative neurological symptoms and subsequently received adjuvant radiation therapy. At 52 months after surgery, she was still experiencing neurologically intact, progression-free survival. This case illustrated one of the most extensive recurrent tumor resections for MPE with prolonged disease-free survival reported to date. It may also represent the longest prone position spinal case reported and was notable for a lack of any of the complications commonly associated with the prolonged prone position.

LESSONS

The authors discussed the complexity of surgical decision-making in a symptomatic patient with multiple disseminated metastases, technical considerations for resection of intradural and intramedullary spinal cord tumors, and considerations for avoiding complications during prolonged positioning necessary for spinal surgery.

ABBREVIATIONS

EBL = estimated blood loss; FFP = fresh frozen plasma; GTR = gross total resection; LR = lactated Ringer’s; MPE = myxopapillary ependymoma; MRI = magnetic resonance imaging; POVL = perioperative vision loss; RBC = red blood cell; STR = subtotal resection; RT = radiation therapy; WHO = World Health Organization

BACKGROUND

Symptomatic disseminated myxopapillary ependymoma (MPE) in a young person presents a daunting challenge because the risks of prolonged prone positioning and spinal cord injury may outweigh the likelihood of attaining the benefit of gross total resection.

OBSERVATIONS

The authors reported the case of a 15-year-old girl with five discrete recurrent spinal cord ependymomas. The patient received a 25-hour surgical procedure for gross total resection of the tumors and fusion over an approximately 33-hour period. She experienced complete resolution of all preoperative neurological symptoms and subsequently received adjuvant radiation therapy. At 52 months after surgery, she was still experiencing neurologically intact, progression-free survival. This case illustrated one of the most extensive recurrent tumor resections for MPE with prolonged disease-free survival reported to date. It may also represent the longest prone position spinal case reported and was notable for a lack of any of the complications commonly associated with the prolonged prone position.

LESSONS

The authors discussed the complexity of surgical decision-making in a symptomatic patient with multiple disseminated metastases, technical considerations for resection of intradural and intramedullary spinal cord tumors, and considerations for avoiding complications during prolonged positioning necessary for spinal surgery.

Myxopapillary ependymomas (MPEs) are rare neoplasms comprising 0.5% of all ependymomas1 or approximately 13% of ependymomas arising in the spinal cord. MPEs most frequently develop in the lumbosacral region at the filum terminale and conus medullaris and have an annual incidence of 1 per 1 million people in the United States.2

MPEs were formerly classified as World Health Organization (WHO) grade I, but further appreciation for recurrence potential and capacity for dissemination has resulted in reclassification of MPEs as WHO grade II in the most recent WHO Classification of Tumors of the Central Nervous System.3 Most cases of MPE are localized at initial presentation. However, there are reports of disseminated disease at first diagnosis.4 When dissemination is present at the time of initial diagnosis without previous surgery, the mechanism for tumor spread is thought to be due to spontaneous capsule rupture and bleeding.5,6 Dissemination is also a recognized potential complication after resection.7,8

Despite absence of definitive guidelines, resection is the first-line treatment for MPE.9 Because of the risk of dissemination after resection, gross total resection (GTR) is most desirable and portends the best prognosis.10 Ideally, GTR is achieved through an en bloc resection,11 but often piecemeal GTR or even subtotal resection (STR) is performed due to inability to safely mobilize tumor from neural structures. Although overall survival does not appear to be impacted by extent of resection,11,12 Kraetzig et al.10 showed a 2.5-fold decrease in recurrence when GTR is achieved compared to STR. If residual tumor cannot be resected, the use of adjuvant radiation therapy (RT) may be considered. Mixed results associated with adjuvant RT may be due to differential outcomes based on patient age13 and dose of RT.14 Agbahiwe et al.15 found that adjuvant RT yields improved local control compared to surgery alone in a retrospective study of 16 pediatric patients. Several other reports indicate a benefit of adjuvant RT even in adult patients.14,16,17 Understanding the potential benefits and limitations of RT is important because this therapy has well-recognized risks. Patients with MPE are likely to achieve long-term survival, especially in the pediatric population, so potential late effects of RT must be considered. Compared to traditional photon radiation, proton radiation may serve as an effective alternative with an improved safety profile in the pediatric population with spinal ependymoma.18

Recurrent and disseminated MPE further complicates management, and there is a paucity of evidence to guide treatment planning. Here we present a case of recurrent MPE in the form of disseminated disease along the neuraxis and discuss the complex decision-making involved prior to attempting GTR as well as the keys to avoiding complications of prolonged positioning.

Illustrative Case

Initial Presentation

A patient initially presented at 12 years old with symptoms of L2 and L3 radiculopathy. Magnetic resonance imaging (MRI) of the spine demonstrated a single gadolinium-enhancing intradural lesion that was radiographically consistent with an ependymoma. The patient received an uncomplicated piecemeal tumor resection and laminoplasty at the L1–3 levels with a different surgeon. The diagnosis of MPE was confirmed on analysis of specimens obtained at the time of surgery. No further adjuvant treatment was used at that time.

Disseminated Recurrence

Three years after the patient’s initial surgical procedure, when she was 15 years old, she returned to our clinic with reports of back pain. The patient endorsed worsening of her back pain at night and during flexion of the spine while tying her shoes. Furthermore, she noted bilateral lower extremity paresthesias. She also reported difficulty with ascending stairs. Physical examination demonstrated full strength in the bilateral lower extremities. The patient had patchy paresthesia throughout the lower extremities and diminished reflexes at the patella and Achilles tendon. Standing radiographs demonstrated mild thoracolumbar kyphosis. Gadolinium-enhanced MRI demonstrated five intradural tumors at T7, T10, L2, L3, and S1–3, suggestive of disseminated recurrence of her prior MPE (Fig. 1, right). We conducted extensive discussion with the patient and her parents regarding surgical and nonsurgical options. The low likelihood of prolonged disease-free survival was discussed. The patient and her family elected to proceed with a staged attempt at resection followed by fusion to address the thoracolumbar kyphosis.

FIG. 1.
FIG. 1.

Preoperative MRI with gadolinium contrast demonstrating five contrast-enhancing lesions (arrows) at the levels of T7 (left), T10, L2, L3, and S1–3 (right) in a 15-year-old girl with a history of MPE resection 3 years earlier who is now presenting with back pain and bilateral lower-extremity paresthesia.

Procedure

Extensive preoperative planning was carried out to facilitate GTR of the lesions and reduce the risk of complications, especially given the length of the procedure and known risks of prolonged prone positioning.19,20 The patient was placed prone on a Jackson table with Wilson attachment on the first day and without the Wilson frame to attain improved lordosis on the second day. Her head was maintained in a Mayfield to avoid pressure on the cranium. Neuromonitoring of both upper and lower extremities was performed to avoid neurapraxia. GTR of all five tumors and fusion from T7 to the pelvis was accomplished in a 2-day staged procedure. Removal of all tumors was accomplished with laminectomy, opening of the dura at the midline, and GTR in a piecemeal fashion. Direct stimulation of nerve roots was used during resection of tumor that had infiltrated the nerve roots, particularly in the S1–3 area, where the nerve sheaths had to be opened to remove tumor. Sharp dissection without cautery was performed to minimize thermal injury. An MRI was performed intraoperatively after tumor resection on day 1 (Fig. 2, left) and prior to fusion (Fig. 2, right). Lastly, neuronavigation was used to perform an instrumented fusion from T7 to the sacrum (Fig. 3). In total, the patient was in the prone position from 8:45 am on operative day 1 until 11:15 pm and then from 7:30 am to 6:00 pm for day 2 of the staged procedure (a cumulative operative time of 25 hours in a 33.25-hour period). The total time for anesthesia was 28 hours.

FIG. 2.
FIG. 2.

Postoperative MRI demonstrating complete resection of the five intradural lesions immediately after resection on operative day 1 (left) and prior to starting fusion on operative day 2 (right).

FIG. 3.
FIG. 3.

Two postoperative radiographs showing spinal fusion with instrumentation from T7 to the sacrum and improvement of thoracolumbar kyphosis compared to preoperative imaging. Imaging captures the thoracic levels (left) and the lumbar to sacral levels (right).

Outcome and Adjuvant Therapy

Postoperatively, the patient experienced full resolution of her neurological deficits and retained full bowel and bladder function as well as lower extremity strength and sensation. MRI was performed to monitor for indications of adjuvant RT. A residual, stable 2-mm nodular focus of intradural/extramedullary enhancement at the level of T4–5 was identified on MRI (performed with metal artifact reduction protocol).

She received subsequent adjuvant radiation several weeks postoperatively. For radiation planning, the postoperative MRIs were fused to the planning software, and the clinical treatment volume was defined as the entire spinal canal and nerve roots extending from C2 to the terminus of the thecal sac. She was treated on TomoTherapy with 6 MV photons using an intensity modulated radiotherapy technique using daily image guidance. The field extended from C2 to the bottom of the thecal sac and was treated at 150 cGy/day to a total cumulative dose of 4,200 cGy. Given the MRI evidence of a 2-mm nodular focus of intradural/extramedullary enhancement at the level of T4–5, using a concomitant boost technique, the area at T4–5 and below L2 received a higher dose of 180 cGy/day for a total cumulative dose of 5,040 cGY.

The patient is currently 59 months out from her staged tumor resection and fusion and remains neurologically intact. On the most recent follow-up contrasted MRI, at 52 months after the recurrent tumor resection, there is no evidence of tumor progression. Her thoracolumbar rods have broken bilaterally, but because she is not overtly symptomatic from this, we are managing that finding nonoperatively.

Discussion

Observations

Our case demonstrates prolonged asymptomatic, disease-free survival after GTR and RT of symptomatic, disseminated, recurrent MPE. We reviewed the literature for cases of disseminated MPE with a PubMed database search of “disseminated myxopapillary ependymoma,” which yielded 191 results. After applying inclusion criteria of confirmed MPE; dissemination, which was defined as multiple neuraxis lesions, drop metastases, or extraneural dissemination at initial presentation and/or at time of recurrence; and treatment data, we identified 24 reports of 75 distinct patients (Table 1).4–45 In the setting of disseminated recurrence, there were five reported cases of GTR in four different patients. RT was also used in one of these cases. Abdallah et al.21 reported GTR of one drop metastasis, whereas Lee et al.22 reported GTR of a maximum of two masses at the time of surgery. Lastly, Abdu et al.23 described a case in which one mass was removed by GTR. Thus, to the best of our knowledge, our illustrative case with GTR of five lesions is the greatest number of lesions removed by GTR in the setting of disseminated MPE at the time of recurrence. Our case also demonstrates the longest period of asymptomatic, disease-free survival after reoperation for symptomatic, recurrent disseminated MPE among reported cases of GTR of recurrent disseminated MPE.

TABLE 1.

Patient and treatment information for cases of disseminated MPE at initial presentation or as recurrent disease

Authors & YearAge, SexInitial PresentationRecurrence
Local/ Disseminated1° Treatment2° TreatmentTime to RecurrenceLocal/ Disseminated1° Treatment2° Treatment
Vongsfak et al., 20202913, MDisseminatedSTRRTNo recurrence at 2-mo follow-up
Straus et al., 2014463, MDisseminatedNTRRTNo recurrence at 6-mo follow-up
Mishra et al., 20213018, MDisseminatedBiopsyRTNo recurrence at 18-mo follow-up
Toktaş et al., 20153126, MDisseminatedSTRNone, no follow-up data provided
Abdallah et al., 20202111, MLocalGTR (2 segments)75 mos laterDisseminatedGTR
13, FLocalGTR (2 segments)45 mos laterDisseminatedRT
12, FLocalSTRRT12 mos laterDisseminatedBiopsyRT
Looi et al., 20213215, MLocalGTR16.3 mos median time to recurrenceDisseminatedSTR
8, MLocalSTRDisseminatedSTR
12, MDisseminatedGTRDisseminatedSTR
8, FLocalGTRDisseminatedRT
8, FDisseminatedSTRRTResidual LM diseaseNone
10, FLocalGTRDisseminatedRT
14, FLocalSTRDisseminatedSTR
13, MDisseminatedNTRRTSmall residualNone
7, FDisseminatedNTRRTSmall residualNone
16, FDisseminatedGTRDisseminatedSTR
21, MDisseminatedNTRRTSmall residualNone
18, MDisseminatedGTRRT No recurrenceNone
Abdulaziz et al., 20151159, MLocalSTR12 mosDisseminatedChemoRT
Khalatbari et al., 20133316, MDisseminatedSTRRTNo recurrence at 5-yr follow-up
Fujiwara et al., 20183426, FLocalGTR2 yrsLocalGTRRT
4 yrsDisseminatedRT
6 yrsLocalGTRTMZ
Fonseca et al., 20193521, MLocalSTRRT21 yrsDisseminatedSTRRT
Awaya et al., 2021522, MDisseminatedGTR of dominant lesionNo recurrence at 12 mos
Zhu et al., 20193623, MDisseminatedSTRRTNo recurrence at 18 mos
Lee et al., 20192213, MDisseminatedSTRRT & AvastinStable residual disease at 4 yrsNone
16, FDisseminatedGTR4 yrsDisseminatedResection NFSRT
31, MDisseminatedSTRChemo RT9 mosLocalSTR
31, FDisseminatedResection NFSRT14 yrsDisseminatedGTRChemo
40, FDisseminatedSTRProton therapyNone at 2.5 yrs
45, MDisseminatedSTRRT12 yrsDisseminatedGTR
15 yrsDisseminatedGTRRT
Fassett et al., 20053713, FDisseminatedGTRRT3 yrsDisseminatedRT
14, MDisseminatedGTRRTNo recurrence at 5 yrs
14, MDisseminatedSTRRTNo recurrence at 3.5 yrs
8, MDisseminatedSTRRTStable residual, local disease at 4.5 yrsNone
Macedo et al., 20113833, MDisseminatedGTR of cerebral lesionBiopsy of lumbar lesion & RTStable disease at 2 mos from completion of RTNone
Deniel et al., 20193930, FLocalGTR3 recurrences over 10 yrsLocalGTR each time
11 yrsLocalRT
13 yrsDisseminatedTMZPatient died within a few weeks of initiating TMZ
Rege et al., 20164023, MLocalSTRRT8 mos after RTDisseminatedSTRRT
Abdu et al., 20212347, FLocalGTRRT28 mosDisseminatedGTR 44 mos after initial GTR
48 mosDisseminatedBiopsy
Plans et al., 20064123, MLocalSTRRT3 yrsDisseminatedWatchful waiting for 4 yrsBiopsy & RT 7 yrs after
Al-Halabi et al., 20104214, MDisseminatedSTRRT4 mosLocalSTRRT
14, MLocalGTR2 mosDisseminatedRT
18, MLocalGTR36 mosDisseminatedRT
18, MDisseminatedGTRRTNo recurrence at 78 mos
Bandopadhayay et al., 20164317, FDisseminatedGTRNADisseminatedNone
8, MLocalSTRRTDisseminatedNone
21, FDisseminatedSTRDisseminatedSTRRT
8, MDisseminatedGTRDisseminatedSTRChemo, RT
15, FDisseminatedSTRDisseminatedSTRChemo, RT
19, MDisseminatedSTRRTDisseminated (asymptomatic)None
12, MLocalGTRDisseminated (asymptomatic)None
10, FDisseminatedGTRLocalResection NFS
14, FDisseminatedSTRNone
17, FDisseminatedSTRNone
11, MDisseminatedGTRLocal (asymptomatic)None
Pencovich et al., 20144436, MDisseminatedSTRNone at 9 mos
35, MDisseminatedSTR19 mosLocal
28, FDisseminatedSTRNone at 1.5 yrs
37, FDisseminatedGTRRTNone at 6 yrs
18, MDisseminatedSTRNone at 4.5 yrs
14, MDisseminatedSTRRTNone 2 yrs
12, MDisseminatedGTRRTNone at 3 yrs
15, MDisseminatedSTRNone at 7 yrs
17, MDisseminatedSTRNone at 7.5 yrs
Higgins et al., 20054536, FDisseminatedBiopsyRT6 mos after RTDisseminatedDexamethasone
Kraetzig et al., 20181021, MLocalSTR4 mosDisseminatedResection NFSChemo, RT
12 mosLocalResection NFS
27, MLocalGTR2 mosDisseminated (asymptomatic)None
9, MLocalGTRRT20 yrsDisseminatedResection NFSChemo, RT
30, MLocalSTRRT20 mosDisseminated (asymptomatic)None
43, MDisseminatedGTRNone at 36 mos
20, FDisseminatedGTRNone at 24 mos
18, FDisseminatedGTRNone at 6 mos
32, MLocalGTR3 mosDisseminated (asymptomatic)None
15, MLocalGTRRT30 yrsDisseminatedResection NFS
48, MDisseminatedSTRRT42 mosDisseminated (asymptomatic)None

Chemo = chemotherapy; LM = leptomeningeal; NA = not applicable; NFS = not further specified; NTR = near total resection; TMZ = temozolomide.

Lessons

Surgical Decision-Making

The reported overall survival for MPE is high, with rates of 90% to 100% at 5 years.10,14,16,24 However, the concern with this condition is a loss of spinal cord function at or below the spinal level with the greatest tumor burden. Surgical decision-making should be carefully considered for treatment of MPE, and patients ought to be counseled that surgical and/or adjuvant RT has not demonstrated improvement in overall survival.14,16,24

Consideration must be given to the constellation of symptoms a patient is experiencing. Ultimately, the risks of surgery must be weighed against the likelihood of tumor progression. As in our case, metastatic seeding of the thecal sac can occur after resection of WHO grade II MPE.8,10 Treatment failure rates have been reported in approximately 30% of patients.14,16 Kraetzig et al.10 reported distant metastases in 57.9% (11/19) of patients with MPE; 36% (4/11) of cases of disseminated MPE were identified at the time of initial diagnosis, whereas 64% (7/11) were identified during the follow-up period with a median time to recurrence of 20 months. Interestingly, 72.7% (8/11) of these patients remained asymptomatic with no evidence of progression over a median follow-up period of 32 months. The authors concluded that close follow-up is an appropriate option for asymptomatic patients with metastatic disease.10 The ultimate concern for these patients is that at some point they may become symptomatic and surgery will not be an option if the tumor spread is too diffuse. Symptomatic recurrence can impact quality of life, and for patients who are symptomatic, surgery appears to offer the greatest benefit.10,12,24

Technical Considerations for Achieving GTR with Disseminated MPE at Recurrence

GTR appears to offer the highest likelihood of progression-free survival.14,24,25 The surgical goal is to resect each MPE lesion en bloc without disruption of the capsule. However, the natural tendency for these lesions to be adherent to neurological structures makes en bloc or even piecemeal GTR challenging; therefore, it is often not achieved. In our review of the literature, we identified 50 patients with disseminated disease at initial diagnosis, with 18 (36%) of them receiving GTR of any of their lesions whereas 30 (60%) had subtotal tumor resection. Among the 40 patients with disseminated lesions at the time of recurrence, 5 (12.5%) received GTR of at least one tumor, and STR was achieved in 14 (35%) of the patients. Moreover, among patients with disseminated disease, microscopic spread is assumed to have occurred and GTR is not achieved beyond the primary tumor targeted for resection.

In our case, preoperative contrasted MRI highlighted five discrete intradural tumor foci at T7, T10, L2, L3, and S1–3. The initial surgical exposure extended from T10 to the sacrum. We performed wide laminectomies at each affected level to maximize the likelihood of safe GTR of lesions adherent to the spinal cord and/or nerve roots.26 We began with the S1–3 lesion, which was resected along with its encapsulating dura while preserving the exiting nerve roots and temporarily occluding the caudal thecal sac to avoid cerebrospinal fluid leakage, although no fluid was noted until the L2 and L3 lesions were resected. We then moved rostrally in a stepwise manner, performing additional wide laminectomies and opening the dura along the midline and resecting the lesions at the corresponding levels individually. We then extended our initial exposure to T7, allowing resection of the lesion at this level.

We used intraoperative neurophysiological monitoring because it has proven to be an effective tool to guide intraoperative surgical and medical adjustments to reduce the likelihood of neurological injury. Identification of the tumor-nerve/spinal cord interface is paramount, particularly when involving encapsulated lesions such as MPE.27 Neuromonitoring capabilities were essential for dissection of the lesions at the S1–2 level, L2, and L3 because these lesions were adherent to nerve roots. We were able to identify the tumor-nerve interface surrounding the S1–2 lesion. We were unable to identify a plane circumferentially around the lesions at L2 and L3, and they were removed in a piecemeal fashion with periodic nerve stimulation for tumor-nerve plane confirmation. Furthermore, neuromonitoring enabled us to identify a lesion within the nerve sheath of the S1 nerve root after this root failed to respond to stimulation.

Considerations for Prolonged Prone Positioning

Based on our literature review, this may be the longest reported prone position case completed without complication. Our patient was in the prone position for 14.5 hours on operative day 1. On the subsequent day, she was placed prone for an additional 10.5 hours, for a total of 25 hours prone in less than a 34-hour period. Various complications are associated with prolonged prone positioning with or without intraoperative hypotension, including perioperative vision loss (POVL).19,20 Specific thresholds for vital signs, lab values, and positioning are difficult to ascertain. However, there are practice advisories with recommendations for reducing the risks of POVL during spine surgery (see Table 2 for a summary of considerations to minimize POVL).28

TABLE 2.

Recommendations for POVL prevention for spine surgery

Preoperative
During the informed consent, inform the patient of risks of prolonged* prone positioning, including POVL, and substantial blood loss

Debrief members of the surgical team regarding the importance of monitoring labs and pressure points and adequately compensating for any fluid/blood losses
Intraoperative Monitoring and Management
Blood pressure

MAP maintained between 75 and 90 mm Hg

Blood/fluid compensation

Hemoglobin, hematocrit, and sodium levels consistently monitored throughout the duration of the procedures

Combination of blood products, crystalloids, and colloids administered to offset losses

Vasopressors

Phenylephrine administered to maintain MAP in desired range

Positioning

Jackson table and Wilson frame utilized to avoid direct pressure over the eyes and face with continued monitoring for pressure points during the case

Maintained head above the level of the heart to reduce risk of increased intracranial and intraocular pressure

Staging of procedures

Tumor resections performed on operative day 1 and fusion with instrumentation is carried out on the subsequent day
Postoperative Evaluation and Management
Visual acuity examined during the postoperative evaluation

MAP = mean arterial pressure.

Prolonged procedures are defined as spine procedures >4 hours.28

Substantial blood loss is defined as blood loss >800 mL.28

We used a Jackson table and Mayfield head-holder for our procedure to avoid any direct contact with the face or eyes, and neuromonitoring was used for identifying neurological injury secondary to the procedure itself or prolonged positioning. All potential pressure points were padded appropriately, radial arterial pressure was monitored via catheter, and a left subclavian triple lumen vascular access was placed.

During the case, avoidance of hypotension, fluid maintenance, and offset of blood loss were strictly observed during rotation of two anesthesiologists and seven certified registered nurse anesthetists. Despite the potential for exacerbation of surgical blood loss, the patient’s head was maintained higher than the level of the heart throughout the procedure to avoid elevation of intracranial and intraocular pressures. The patient’s mean arterial pressure was maintained between 75 and 90 mm Hg with phenylephrine throughout the procedure on operative days 1 and 2. The following data were recorded for operative day 1: 1.85 L of estimated blood loss (EBL), urine output of 11.325 L, hemoglobin (N = 10) of 8.3 to 11.6, hematocrit (N = 16) of 21 to 33.2, and sodium (N = 11) between 143 and 150. Blood and fluid losses were corrected with 1.5 L of packed red blood cells (RBCs), 120 mL of autologous RBCs, 500 mL of fresh frozen plasma (FFP), 6 L of lactated Ringer’s (LR) solution, 5 L of 0.45% NaCl, 6.6 L of Plasmalyte, and 250 mL of albumin.

On operative day 2 there was an EBL of 1.5 L and a urine output of 8.9 L. Monitoring values included hemoglobin (N = 6) of 9.4 to 12, hematocrit (N = 13) of 22 to 33.7, and sodium (N = 9) of 140 to 149. Blood and fluid losses from operative day 2 were compensated with 1.05 L of packed RBCs, 450 mL of autologous RBCs, 784 mL of FFP, 243 mL of platelets, 6.9 L of LR solution, 1.55 L of 0.45% NaCl, 6.9 L of Plasmalyte, and 250 mL of albumin.

Limitations

Limitations to the generalizability of lessons of this case may preclude extrapolation to other cases of disseminated MPE. In the absence of randomized, controlled trials and evidence-based guidelines, clinical decision-making in the setting of disseminated MPE remains a matter for discussion between patients and physicians. Because each patient brings their own risks into surgery and because of variability in surgical skills, one should not generalize that resection of disseminated spinal cord tumors followed by radiation outweighs the risks of other management strategies.

Furthermore, our experience of uncomplicated prolonged prone positioning may not be generalizable because our patient may not be representative of the larger population of patients with disseminated MPE or those being considered for procedures necessitating prolonged prone positioning. It is important to acknowledge that our patient was in her teens and had a BMI of 22.1. Both factors likely played a protective role against complications with prolonged prone positioning and may not be present in other patients presenting with disseminated MPE. Thus, our experience should be considered cautiously when planning the positioning of other patients with different risk factor profiles.

Conclusions

MPE is a rare, WHO grade II neoplasm with the potential to recur as disseminated disease after primary surgical intervention. Here we demonstrate that in an appropriately selected patient with disseminated lesions, resection can be a safe and effective treatment modality to achieve prolonged asymptomatic progression-free survival. Additionally, we report on potentially the longest duration of prone positioning for spine surgery without complication. Further studies are needed to establish evidenced-based treatment guidelines for focal and disseminated MPE.

Disclosures

Dr. Samadani has equity in Oculogica and has received personal fees from Integra. Medtronic has made a donation to the J Aron Allen Legacy Foundation, which has donated research money to the laboratory of Dr. Samadani. No other disclosures were reported.

Author Contributions

Conception and design: Samadani, Johnson, Cramer. Acquisition of data: all authors. Analysis and interpretation of data: Samadani, Johnson. Drafting the article: Samadani, Johnson, Cramer. Critically revising the article: all authors. Reviewed submitted version of manuscript: all authors. Approved the final version of the manuscript on behalf of all authors: Samadani. Statistical analysis: Johnson. Administrative/technical/material support: Johnson, Cramer. Study supervision: Cramer.

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    • Export Citation
  • 13

    Feldman WB, Clark AJ, Safaee M, Ames CP, Parsa AT. Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children: a systematic review. J Neurosurg Spine. 2013;19(4):471476.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 14

    Pica A, Miller R, Villà S, et al. The results of surgery, with or without radiotherapy, for primary spinal myxopapillary ependymoma: a retrospective study from the Rare Cancer Network. Int J Radiat Oncol Biol Phys. 2009;74(4):11141120.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 15

    Agbahiwe HC, Wharam M, Batra S, Cohen K, Terezakis SA. Management of pediatric myxopapillary ependymoma: the role of adjuvant radiation. Int J Radiat Oncol Biol Phys. 2013;85(2):421427.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 16

    Akyurek S, Chang EL, Yu TK, et al. Spinal myxopapillary ependymoma outcomes in patients treated with surgery and radiotherapy at M.D. Anderson Cancer Center. J Neurooncol. 2006;80(2):177183.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 17

    Chao ST, Kobayashi T, Benzel E, et al. The role of adjuvant radiation therapy in the treatment of spinal myxopapillary ependymomas. J Neurosurg Spine. 2011;14(1):5964.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 18

    Amsbaugh MJ, Grosshans DR, McAleer MF, et al. Proton therapy for spinal ependymomas: planning, acute toxicities, and preliminary outcomes. Int J Radiat Oncol Biol Phys. 2012;83(5):14191424.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 19

    DePasse JM, Palumbo MA, Haque M, Eberson CP, Daniels AH. Complications associated with prone positioning in elective spinal surgery. World J Orthop. 2015;6(3):351359.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 20

    Shriver MF, Zeer V, Alentado VJ, Mroz TE, Benzel EC, Steinmetz MP. Lumbar spine surgery positioning complications: a systematic review. Neurosurg Focus. 2015;39(4):E16.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 21

    Abdallah A, Emel E, Gündüz HB, Sofuoğlu ÖE, Asiltürk M, Abdallah BG. Long-term surgical resection outcomes of pediatric myxopapillary ependymoma: experience of two centers and brief literature review. World Neurosurg. 2020;136:e245e261.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 22

    Lee JC, Sharifai N, Dahiya S, et al. Clinicopathologic features of anaplastic myxopapillary ependymomas. Brain Pathol. 2019;29(1):7584.

  • 23

    Abdu AM, Alshoabi SA, Alshoaibi AM, Hamid AM, Jhaveri MD. Myxopapillary ependymoma with interval postoperative CSF seeding: a report of an unusual case. Radiol Case Rep. 2021;16(12):38383843.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 24

    Weber DC, Wang Y, Miller R, et al. Long-term outcome of patients with spinal myxopapillary ependymoma: treatment results from the MD Anderson Cancer Center and institutions from the Rare Cancer Network. Neuro Oncol. 2015;17(4):588595.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 25

    Lucchesi KM, Grant R, Kahle KT, Marks AM, DiLuna ML. Primary spinal myxopapillary ependymoma in the pediatric population: a study from the Surveillance, Epidemiology, and End Results (SEER) database. J Neurooncol. 2016;130(1):133140.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 26

    Angevine PD, Kellner C, Haque RM, McCormick PC. Surgical management of ventral intradural spinal lesions. J Neurosurg Spine. 2011;15(1):2837.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 27

    Takami T, Naito K, Yamagata T, Ohata K. Surgical management of spinal intramedullary tumors: radical and safe strategy for benign tumors. Neurol Med Chir (Tokyo). 2015;55(4):317327.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 28

    Practice Advisory for Perioperative Visual Loss Associated with Spine Surgery. Practice advisory for perioperative visual loss associated with spine surgery 2019: an updated report by the American Society of Anesthesiologists Task Force on Perioperative Visual Loss, the North American Neuro-Ophthalmology Society, and the Society for Neuroscience in Anesthesiology and Critical Care. Anesthesiology. 2019;130(1):1230.

    • Search Google Scholar
    • Export Citation
  • 29

    Balser D, Farooq S, Mehmood T, Reyes M, Samadani U. Actual and projected incidence rates for chronic subdural hematomas in United States Veterans Administration and civilian populations. J Neurosurg. 2015;123(5):12091215.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 30

    Mishra A, Sadashiva N, Krishna U, et al. Disseminated craniospinal myxopapillary ependymoma treated with biopsy and adjuvant radiation therapy: a case report and review of literature. Neurol India. 2021;69(2):500504.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 31

    Toktaş ZO, Demir MK, Yapıcıer Ö, Akakın A, Yılmaz B, Konya D. Disseminated adult spinal extramedullary myxopapillary ependymoma. Spine J. 2015;15(12):e69e70.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 32

    Looi WS, Indelicato DJ, Mailhot Vega RB, et al. Outcomes following limited-volume proton therapy for multifocal spinal myxopapillary ependymoma. Pediatr Blood Cancer. 2021;68(3):e28820.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 33

    Khalatbari MR, Jalaeikhoo H, Hamidi M, Moharamzad Y. Craniospinal dissemination of filum myxopapillary ependymoma following spinal trauma: case report and literature review. Childs Nerv Syst. 2013;29(1):149152.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 34

    Fujiwara Y, Manabe H, Izumi B, Shima T, Adachi N. Remarkable efficacy of temozolomide for relapsed spinal myxopapillary ependymoma with multiple recurrence and cerebrospinal dissemination: a case report and literature review. Eur Spine J. 2018;27(suppl 3):421425.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 35

    Fonseca L, Cicuendez M, Martínez-Ricarte F, Martínez-Saez E, Cordero E, Bescos A. A rare case of an intramedullary metastasis of a myxopapillary ependymoma. Surg Neurol Int. 2019;10(83):83.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 36

    Zhu F, Ding J, Li Y, et al. Benign ependymoma with extensive intracranial and spinal cerebrospinal fluid dissemination: case report and literature review. Br J Neurosurg. 2019;33(3):290293.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 37

    Fassett DR, Pingree J, Kestle JRW. The high incidence of tumor dissemination in myxopapillary ependymoma in pediatric patients. Report of five cases and review of the literature. J Neurosurg. 2005;102(suppl 1):5964.

    • Search Google Scholar
    • Export Citation
  • 38

    Macedo LT, Rogerio F, Pereira EB, de Souza Queiroz L, Carvalheira JBC. Cerebrospinal tumor dissemination in a patient with myxopapillary ependymoma. J Clin Oncol. 2011;29(32):e795e798.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 39

    Deniel A, Marguet F, Beaussire L, et al. TERTp mutation detection in plasma by droplet-digital polymerase chain reaction in spinal myxopapillary ependymoma with lung metastases. World Neurosurg. 2019;130:405409.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 40

    Rege SV, Narayan S, Patil H, Songara A. Spinal myxopapillary ependymoma with interval drop metastasis presenting as cauda equina syndrome: case report and review of literature. J Spine Surg. 2016;2(3):216221.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 41

    Plans G, Brell M, Cabiol J, Villà S, Torres A, Acebes JJ. Intracranial retrograde dissemination in filum terminale myxopapillary ependymomas. Acta Neurochir (Wien). 2006;148(3):343346.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 42

    Al-Halabi H, Montes JL, Atkinson J, Farmer JP, Freeman CR. Adjuvant radiotherapy in the treatment of pediatric myxopapillary ependymomas. Pediatr Blood Cancer. 2010;55(4):639643.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 43

    Bandopadhayay P, Silvera VM, Ciarlini PDSC, et al. Myxopapillary ependymomas in children: imaging, treatment and outcomes. J Neurooncol. 2016;126(1):165174.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 44

    Pencovich N, Bot G, Lidar Z, et al. Spinal ependymoma with regional metastasis at presentation. Acta Neurochir (Wien). 2014;156(6):12151222.

  • 45

    Higgins GS, Smith C, Summers DM, Statham PXF, Erridge SC. Myxopapillary ependymoma with intracranial metastases. Br J Neurosurg. 2005;19(4):356358.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • View in gallery

    Preoperative MRI with gadolinium contrast demonstrating five contrast-enhancing lesions (arrows) at the levels of T7 (left), T10, L2, L3, and S1–3 (right) in a 15-year-old girl with a history of MPE resection 3 years earlier who is now presenting with back pain and bilateral lower-extremity paresthesia.

  • View in gallery

    Postoperative MRI demonstrating complete resection of the five intradural lesions immediately after resection on operative day 1 (left) and prior to starting fusion on operative day 2 (right).

  • View in gallery

    Two postoperative radiographs showing spinal fusion with instrumentation from T7 to the sacrum and improvement of thoracolumbar kyphosis compared to preoperative imaging. Imaging captures the thoracic levels (left) and the lumbar to sacral levels (right).

  • 1

    McGuire CS, Sainani KL, Fisher PG. Incidence patterns for ependymoma: a surveillance, epidemiology, and end results study. J Neurosurg. 2009;110(4):725729.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 2

    Limaiem F, Das JM. Myxopapillary Ependymoma. In: StatPearls. StatPearls Publishing; 2021.

  • 3

    Louis DN, Perry A, Wesseling P, et al. The 2021 WHO classification of tumors of the central nervous system: a summary. Neuro Oncol. 2021;23(8):12311251.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 4

    Straus D, Tan LA, Takagi I, O’Toole JE. Disseminated spinal myxopapillary ependymoma in an adult at initial presentation: a case report and review of the literature. Br J Neurosurg. 2014;28(5):691693.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 5

    Awaya T, Nishimura Y, Eguchi K, et al. Preoperative intracranial dissemination of spinal myxopapillary ependymoma attributed to tumor hemorrhage. World Neurosurg. 2021;145:1318.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 6

    Tonogai I, Sakai T, Tezuka F, et al. Spontaneous rupture and hemorrhage of myxopapillary ependymoma of the filum terminale: a case report and literature review. J Med Invest. 2014;61(3–4):430435.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 7

    Rezai AR, Woo HH, Lee M, Cohen H, Zagzag D, Epstein FJ. Disseminated ependymomas of the central nervous system. J Neurosurg. 1996;85(4):618624.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 8

    Ellison DW, Aldape KD, Capper D, et al. cIMPACT-NOW update 7: advancing the molecular classification of ependymal tumors. Brain Pathol. 2020;30(5):863866.

    • Search Google Scholar
    • Export Citation
  • 9

    Bates JE, Choi G, Milano MT. Myxopapillary ependymoma: a SEER analysis of epidemiology and outcomes. J Neurooncol. 2016;129(2):251258.

  • 10

    Kraetzig T, McLaughlin L, Bilsky MH, Laufer I. Metastases of spinal myxopapillary ependymoma: unique characteristics and clinical management. J Neurosurg Spine. 2018;28(2):201208.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 11

    Abdulaziz M, Mallory GW, Bydon M, et al. Outcomes following myxopapillary ependymoma resection: the importance of capsule integrity. Neurosurg Focus. 2015;39(2):E8.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 12

    Sonneland PR, Scheithauer BW, Onofrio BM. Myxopapillary ependymoma. A clinicopathologic and immunocytochemical study of 77 cases. Cancer. 1985;56(4):883893.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 13

    Feldman WB, Clark AJ, Safaee M, Ames CP, Parsa AT. Tumor control after surgery for spinal myxopapillary ependymomas: distinct outcomes in adults versus children: a systematic review. J Neurosurg Spine. 2013;19(4):471476.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 14

    Pica A, Miller R, Villà S, et al. The results of surgery, with or without radiotherapy, for primary spinal myxopapillary ependymoma: a retrospective study from the Rare Cancer Network. Int J Radiat Oncol Biol Phys. 2009;74(4):11141120.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 15

    Agbahiwe HC, Wharam M, Batra S, Cohen K, Terezakis SA. Management of pediatric myxopapillary ependymoma: the role of adjuvant radiation. Int J Radiat Oncol Biol Phys. 2013;85(2):421427.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 16

    Akyurek S, Chang EL, Yu TK, et al. Spinal myxopapillary ependymoma outcomes in patients treated with surgery and radiotherapy at M.D. Anderson Cancer Center. J Neurooncol. 2006;80(2):177183.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 17

    Chao ST, Kobayashi T, Benzel E, et al. The role of adjuvant radiation therapy in the treatment of spinal myxopapillary ependymomas. J Neurosurg Spine. 2011;14(1):5964.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 18

    Amsbaugh MJ, Grosshans DR, McAleer MF, et al. Proton therapy for spinal ependymomas: planning, acute toxicities, and preliminary outcomes. Int J Radiat Oncol Biol Phys. 2012;83(5):14191424.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 19

    DePasse JM, Palumbo MA, Haque M, Eberson CP, Daniels AH. Complications associated with prone positioning in elective spinal surgery. World J Orthop. 2015;6(3):351359.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 20

    Shriver MF, Zeer V, Alentado VJ, Mroz TE, Benzel EC, Steinmetz MP. Lumbar spine surgery positioning complications: a systematic review. Neurosurg Focus. 2015;39(4):E16.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 21

    Abdallah A, Emel E, Gündüz HB, Sofuoğlu ÖE, Asiltürk M, Abdallah BG. Long-term surgical resection outcomes of pediatric myxopapillary ependymoma: experience of two centers and brief literature review. World Neurosurg. 2020;136:e245e261.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 22

    Lee JC, Sharifai N, Dahiya S, et al. Clinicopathologic features of anaplastic myxopapillary ependymomas. Brain Pathol. 2019;29(1):7584.

  • 23

    Abdu AM, Alshoabi SA, Alshoaibi AM, Hamid AM, Jhaveri MD. Myxopapillary ependymoma with interval postoperative CSF seeding: a report of an unusual case. Radiol Case Rep. 2021;16(12):38383843.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 24

    Weber DC, Wang Y, Miller R, et al. Long-term outcome of patients with spinal myxopapillary ependymoma: treatment results from the MD Anderson Cancer Center and institutions from the Rare Cancer Network. Neuro Oncol. 2015;17(4):588595.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 25

    Lucchesi KM, Grant R, Kahle KT, Marks AM, DiLuna ML. Primary spinal myxopapillary ependymoma in the pediatric population: a study from the Surveillance, Epidemiology, and End Results (SEER) database. J Neurooncol. 2016;130(1):133140.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 26

    Angevine PD, Kellner C, Haque RM, McCormick PC. Surgical management of ventral intradural spinal lesions. J Neurosurg Spine. 2011;15(1):2837.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 27

    Takami T, Naito K, Yamagata T, Ohata K. Surgical management of spinal intramedullary tumors: radical and safe strategy for benign tumors. Neurol Med Chir (Tokyo). 2015;55(4):317327.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 28

    Practice Advisory for Perioperative Visual Loss Associated with Spine Surgery. Practice advisory for perioperative visual loss associated with spine surgery 2019: an updated report by the American Society of Anesthesiologists Task Force on Perioperative Visual Loss, the North American Neuro-Ophthalmology Society, and the Society for Neuroscience in Anesthesiology and Critical Care. Anesthesiology. 2019;130(1):1230.

    • Search Google Scholar
    • Export Citation
  • 29

    Balser D, Farooq S, Mehmood T, Reyes M, Samadani U. Actual and projected incidence rates for chronic subdural hematomas in United States Veterans Administration and civilian populations. J Neurosurg. 2015;123(5):12091215.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 30

    Mishra A, Sadashiva N, Krishna U, et al. Disseminated craniospinal myxopapillary ependymoma treated with biopsy and adjuvant radiation therapy: a case report and review of literature. Neurol India. 2021;69(2):500504.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 31

    Toktaş ZO, Demir MK, Yapıcıer Ö, Akakın A, Yılmaz B, Konya D. Disseminated adult spinal extramedullary myxopapillary ependymoma. Spine J. 2015;15(12):e69e70.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 32

    Looi WS, Indelicato DJ, Mailhot Vega RB, et al. Outcomes following limited-volume proton therapy for multifocal spinal myxopapillary ependymoma. Pediatr Blood Cancer. 2021;68(3):e28820.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 33

    Khalatbari MR, Jalaeikhoo H, Hamidi M, Moharamzad Y. Craniospinal dissemination of filum myxopapillary ependymoma following spinal trauma: case report and literature review. Childs Nerv Syst. 2013;29(1):149152.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 34

    Fujiwara Y, Manabe H, Izumi B, Shima T, Adachi N. Remarkable efficacy of temozolomide for relapsed spinal myxopapillary ependymoma with multiple recurrence and cerebrospinal dissemination: a case report and literature review. Eur Spine J. 2018;27(suppl 3):421425.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 35

    Fonseca L, Cicuendez M, Martínez-Ricarte F, Martínez-Saez E, Cordero E, Bescos A. A rare case of an intramedullary metastasis of a myxopapillary ependymoma. Surg Neurol Int. 2019;10(83):83.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 36

    Zhu F, Ding J, Li Y, et al. Benign ependymoma with extensive intracranial and spinal cerebrospinal fluid dissemination: case report and literature review. Br J Neurosurg. 2019;33(3):290293.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 37

    Fassett DR, Pingree J, Kestle JRW. The high incidence of tumor dissemination in myxopapillary ependymoma in pediatric patients. Report of five cases and review of the literature. J Neurosurg. 2005;102(suppl 1):5964.

    • Search Google Scholar
    • Export Citation
  • 38

    Macedo LT, Rogerio F, Pereira EB, de Souza Queiroz L, Carvalheira JBC. Cerebrospinal tumor dissemination in a patient with myxopapillary ependymoma. J Clin Oncol. 2011;29(32):e795e798.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 39

    Deniel A, Marguet F, Beaussire L, et al. TERTp mutation detection in plasma by droplet-digital polymerase chain reaction in spinal myxopapillary ependymoma with lung metastases. World Neurosurg. 2019;130:405409.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 40

    Rege SV, Narayan S, Patil H, Songara A. Spinal myxopapillary ependymoma with interval drop metastasis presenting as cauda equina syndrome: case report and review of literature. J Spine Surg. 2016;2(3):216221.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 41

    Plans G, Brell M, Cabiol J, Villà S, Torres A, Acebes JJ. Intracranial retrograde dissemination in filum terminale myxopapillary ependymomas. Acta Neurochir (Wien). 2006;148(3):343346.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 42

    Al-Halabi H, Montes JL, Atkinson J, Farmer JP, Freeman CR. Adjuvant radiotherapy in the treatment of pediatric myxopapillary ependymomas. Pediatr Blood Cancer. 2010;55(4):639643.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 43

    Bandopadhayay P, Silvera VM, Ciarlini PDSC, et al. Myxopapillary ependymomas in children: imaging, treatment and outcomes. J Neurooncol. 2016;126(1):165174.

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 44

    Pencovich N, Bot G, Lidar Z, et al. Spinal ependymoma with regional metastasis at presentation. Acta Neurochir (Wien). 2014;156(6):12151222.

  • 45

    Higgins GS, Smith C, Summers DM, Statham PXF, Erridge SC. Myxopapillary ependymoma with intracranial metastases. Br J Neurosurg. 2005;19(4):356358.

    • Crossref
    • Search Google Scholar
    • Export Citation

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