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Spinal Cord Ependymoma

Spinal cord ependymomas (SCEs) represent the larger proportion of ependymomas, which are rare tumors of the central nervous system. SCEs tend to occur in adulthood, with a male preponderance, and symptoms depend on the level of the lesion.


Presentation

A spinal cord ependymoma is a rare benign central nervous system (CNS) tumor that is mostly found in the spinal cord. SCEs constitute a minority of both CNS and spinal cord tumors [1]. They are slow-growing and usually present in the fourth decade of life [2]. The typical features arise from proliferation of abnormal tumor cells into the normal cord cells, as well as from the compressive effects of the mass [3]. These commonly include back and neck pain, in addition to motor and sensory neurological fall out [2]. Less often seen are bladder and gastrointestinal tract symptoms.

SCE can be classified according to the World Health Organisation (WHO) classification system, as, subependymomas, myxopapillary ependymomas, classic ependymomas, and anaplastic ependymomas [4] [5]. Males are more affected than females. Furthermore, SCEs are mostly found in adults, the frequent sub-types being myxopapillary and classic [4]. Classic SCEs affect the cervical and thoracic spine, while myxopapillary SCE develops lower down [5]. The latter are also usually encapsulated. SCEs cause less morbidity and mortality than ependymomas that occur in the brain. SCEs consist of ependymal cells, which are glial cells of the CNS that regulate the fluid exchange between cerebrospinal fluid (CSF) and adjacent nerve cells. Their presence can further cause CSF flow obstruction. Moreover, in a few cases, metastasis through the CSF occurs [6].

The location of pain is dependent on the level at which the SCE forms. Tumors in the lumbar spine may produce signs such as lower back and leg pain and may be very similar to disc pathology [7]. For most patients, pain precedes neurological complaints, sometimes by over a year, and is usually more pronounced at night [8]. Neurological symptoms include paraesthesia, radicular pain, dysesthesia, ataxia, and numbness which may start distally and move proximally, in the lower limbs [3]. Because of the slowly progressive nature of the tumors, presentation and diagnosis are usually at a late stage [8].

Aspiration
  • Except in patients with myxopapillary tumours and anaplastic tumours, cytomorphologic features of ependymoma have been described only in case reports of intraoperative imprinting or fine needle aspiration biopsies (FNABs) and not in CSF cytology.[ncbi.nlm.nih.gov]
  • The tumor was carefully excised using Rhoton micro-instruments; Cavitron Ultrasonic Surgical Aspirator ( CUSA ) and bipolar. We first internally debulked the tumor with the CUSA.[omicsonline.org]
Hiccup
  • These symptoms had been accompanied by continual hiccupping; urinary frequency and two episodes of syncope.[omicsonline.org]
Back Pain
  • Spinal cord tumors commonly present with back pain, sciatica, weakness in the legs and bladder symptoms. A case is reported in which an isolated flaccid neurogenic bladder was the only presenting symptom of a spinal cord ependymoma.[ncbi.nlm.nih.gov]
  • The patient presented at the University Hospital of Heraklion, Crete, suffering from a chest to back pain.[ncbi.nlm.nih.gov]
  • Follow up CT head detected no residual tumor until he complained of back pain and pain in right thigh 6 years after surgery. MRI revealed multiple mass at D10-12 and L2-3. He underwent laminectomy and excision of tumor.[nepjol.info]
  • The mean age at presentation for ependymoma is 38.8 years. 1 However, they remain a fairly rare entity, with approximately 227 diagnosed in theUnited States each year. 2 Patients most often present with neck or back pain.[appliedradiology.com]
  • When I landed, I had some lower back pain, but I didn’t think anything of it. I went to my hotel, had dinner and went to sleep. Around 4 a.m., I woke up in a cold sweat with excruciating pain.[mdanderson.org]
Bilateral Leg Weakness
  • Eric Kratky In January 2007, Erik Kratky was 21 and pursuing his undergraduate degree when he experienced bilateral leg weakness.[oncnursingnews.com]
Suggestibility
  • The finding of a very extensive tumor (from T5 to L5) after only 14 months of life suggests that in our case the tumor was present since birth. Magnetic resonance well shows the real extent of the tumor in height and its different components.[ncbi.nlm.nih.gov]
  • This suggests that radiotherapy may achieve long-term tumor control in over half of those patients with residual spinal ependymoma.[ncbi.nlm.nih.gov]
  • Thus, all the above might suggest that in patients with spinal ependymomas the genome in some way is altered.[jmedicalcasereports.biomedcentral.com]
  • 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.[oncnursingnews.com]
  • This hemosiderin cap is fairly suggestive of ependymomas. With filum ependymomas, hemorrhagic products may be seen within the filum.[ependymomas.com]
Anger
  • Angers 1994. Neurochirurgie 1994;40(suppl 1):1–108. Rezai AR, Woo HH, Lee M, Cohen H, Zagzag D, Epstein FJ: Disseminated ependymomas of the central nervous system. J Neurosurg 1996;85:618–624. Article / Publication Details First-Page Preview[karger.com]
Urinary Incontinence
  • The most common presenting symptoms were local back pain (69.5%), radicular pain (70.5%) and urinary incontinence (19%). Mean time from symptom occurrence to diagnosis was 18.7 months (range, 1 month to 20 years).[jmedicalcasereports.biomedcentral.com]

Workup

Cervical lesions may result in weakness and atrophy of the muscles in the arms and hands [9]. This can occur unilaterally or bilaterally. Features of Brown-Sequard syndrome may be seen. There are a number of imaging modalities employed in the diagnosis and assessment of SCEs:

  • Magnetic resonance imaging (MRI) - This is the preferred imaging study. T1 and T2 weighted imaging typically demonstrate hypo or isointense and hyperintense lesions respectively [7] [10].
  • Plain X-rays of the spine also showcase characteristic features of spinal cord ependymoma, such as posterior vertebral body erosion, thinning of the lamina and scoliosis.
  • Computerized tomography (CT) or MRI myelogram.
  • CT scan with or without contrast.
  • Surgery is both diagnostic and therapeutic, as SCEs can be resected [11].

Treatment

  • Twenty-six patients who underwent treatment of spinal cord ependymoma at a single institution were retrospectively analysed.[ncbi.nlm.nih.gov]
  • Complete surgical resection has been established as first-line treatment and can be curative. However, SCEs tend to recur when complete tumor resection is not possible.[ncbi.nlm.nih.gov]
  • Abstract Surgical outcome of intramedullary spinal cord ependymoma was investigated in order to define the treatment strategy. We have operated on 18 cases of intramedullary spinal cord ependymoma based on the principle of radical resection.[ncbi.nlm.nih.gov]
  • Treatment Considerations 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[oncnursingnews.com]

Prognosis

  • Key message: Ependymomas are a group of CNS tumours with moderately good prognosis, though higher grade tumours have slightly poorer survival. The prognosis for spinal ependymomas is better than that for cerebral tumours.[ncin.org.uk]
  • However, there are different opinions among doctors about importance of grade in determining the prognosis for children diagnosed with grade II ependymoma or anaplastic ependymoma (WHO grade III).[cancer.net]
  • Microscopic examination is unreliable in determining the long-term prognosis.[childhoodbraintumor.org]
  • Ependymoma prognosis The prognosis for ependymoma is very closely related to whether the neurosurgeon can remove all of the tumour, and the spread and aggressiveness of the tumour.[royalmarsden.nhs.uk]
  • […] affected (1 month - 81 years) Infratentorial ependymomas more common in children; cause hydrocephalus from obstruction if in posterior fossa Spinal tumours more common in adults (30 - 40 years); better able to excise completely and generally better prognosis[pathologyoutlines.com]

Etiology

  • The third cyst type, dilation of the central canal, is likely reactive in etiology as well, from partial canal obstruction by the tumor.[appliedradiology.com]
  • The etiology is uncertain, with disparate reports of polyomavirus SV40 being complicit and/or altogether unrelated. An increased incidence is noted in type 2 neurofibromatosis.[healio.com]
  • (Etiology) The exact cause of development of Ependymoma is unknown However, it has been observed that often the affected individuals has certain chromosomal defects, which include: Having monosomy 22, which means that a cell has only one 22 nd chromosome[dovemed.com]

Epidemiology

  • J Neurosurg Pediatr 4: 254–261 PubMed CrossRef Google Scholar Schellinger KA et al. (2008) Descriptive epidemiology of primary spinal cord tumors.[link.springer.com]
  • Currently, the Surveillance Epidemiology and the End Results (SEER) Program and the Central Brain Tumor Registry (CBTRUS) group all grades of ependymomas together for reporting purposes.[cern-foundation.org]
  • Definition / general Slowly growing tumor of ependymal cells arising from walls of ventricles or spinal canal Exhibit glial (GFAP ) and epithelial (EMA ) differentiation Epidemiology 3 - 9% of primary CNS tumors More common in children / young adults[pathologyoutlines.com]
  • "Descriptive epidemiology of malignant and nonmalignant primary spinal cord, spinal meninges, and cauda equina tumors, United States, 2004-2007". Cancer. 118 (17): 4220–7. doi : 10.1002/cncr.27390. PMC 4484585. PMID 22907705.[en.wikipedia.org]
  • Jump to navigation Jump to search Epidemiology [ edit ] 5-10% of pediatric brain tumors Primarily in infants and children Approximately 200 pediatric cases per year in USA Location [ edit ] Glial tumors that arise from ependymal lining of the ventricular[en.wikibooks.org]
Sex distribution
Age distribution

Prevention

  • Treatment Considerations 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[oncnursingnews.com]
  • Clinical trials look at new ways to prevent, find and treat cancer. Find out more about clinical trials .[cancer.ca]
  • The drugs kill cancer cells, preventing them from dividing and spreading further. Read more Meet our doctors Everything we do is focused on designing better care for our patients.[genesiscare.co.uk]
  • NIH: National Cancer Institute Cancer (Medical Encyclopedia) Cancer and lymph nodes (Medical Encyclopedia) Cancer treatment -- early menopause (Medical Encyclopedia) Cancer treatment: preventing infection (Medical Encyclopedia) Cancer treatments (Medical[icdlist.com]
  • Anticonvulsants If you have seizures (fits), you may be given drugs called anticonvulsants to help prevent them.[macmillan.org.uk]

References

Article

  1. Gilbert MR, Ruda R, Soffietti R. Ependymomas in adults. Curr Neurol Neurosci Rep. 2010;10(3):240-247.
  2. Hanbali F, Fourney DR, Marmor E, et al. Spinal cord ependymoma: radical surgical resection and outcome. Neurosurgery. 2002;51(5):1162–1174.
  3. Schwartz TH, McCormick PC. Intramedullary ependymomas: clinical presentation, surgical treatment strategies and prognosis. J Neurooncol. 2000;47(3):211-218.
  4. Rudà R, Gilbert M, Soffietti R. Ependymomas of the adult. molecular biology and treatment. Curr Opin Neurol. 2008;21(6):754–761.
  5. Sakai Y, Matsuyama Y, Katayama Y, et al. Spinal myxopapillary ependymoma: neurological deterioration in patients treated with surgery. Spine (Phila Pa 1976). 2009;34(15):1619-1624.
  6. Peschel RE, Kapp DS, Cardinale F, Manuelidis EE. Ependymomas of the spinal cord. Int J Radiat Oncol Biol Phys. Int J Radiat Oncol Biol Phys. 1983;9(7):1093-1096.
  7. Schweitzer JS, Batzdorf U. Ependymoma of the cauda equina region : diagnosis, treatment, and outcome in 15 patients. Neurosurgery. 1992;30(2):202–207.
  8. Boström A, von Lehe M, Hartmann W, et al. Surgery for spinal cord ependymomas: outcome and prognostic factors. Neurosurgery. 2011;68(2):302-308. discussion 309.
  9. McCormick PC, Torres R, Post KD, Stein BM. Intramedullary ependymoma of the spinal cord. J Neurosurg. 1990;72(4):523-532.
  10. Choi JY, Chang KH, Yu IK, et al. Intracranial and Spinal Ependymomas: Review of MR Images in 61 Patients. Korean J Radiol. 2002;3(4):219-228.
  11. Hoshimaru M, Koyama T, Hashimoto N, Kikuchi H. Results of microsurgical treatment for intramedullary spinal cord ependymomas: analysis of 36 cases. Neurosurgery. 1999;44(2):264-269.

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Last updated: 2019-06-28 10:45