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].

• Pain

  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. [symptoma.com]

  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]

  In the last seven years, he presented moderate pain in the neck, shoulders, deep pain in the thorax and the burning pain in the extremities was almost constant. A year before his admission, he began walking with staggering gait. [omicsonline.org]

  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]

• Nausea

  Symptoms of ependymoma are dependent on the location of the tumor, with intracranial tumors causing increased intracranial pressure, headache, ataxia, seizures, and nausea and vomiting, and spinal tumors causing weakness and back pain. [clinicaladvisor.com]

  Common symptoms may include: Nausea and vomiting Lethargy and irritability Headaches Clumsiness Difficulty with tasks like handwriting Gradual decline in school performance Changes in personality and behaviour Altered way of walking If the tumour spreads [royalmarsden.nhs.uk]

  This can lead to increased intracranial pressure (ICP, which is the pressure inside your head), which can cause headaches, nausea, vomiting, and dizziness. Symptoms may be sudden or they may start slowly and get worse over time. [saintlukeshealthsystem.org]

  People with an ependymoma in the brain may have headaches, nausea, vomiting and dizziness. [cern-foundation.org]

  It also can cause renal toxicity, increased risk of infection, nausea, and fatigue, requiring frequent clinic assessment and monitoring with twice-monthly labs,” says Murphy. [oncnursingnews.com]

• Vomiting

  Signs and symptoms [ edit ] Source: [1] severe headache visual loss (due to papilledema) vomiting bilateral Babinski sign drowsiness (after several hours of the above symptoms) gait change (rotation of feet when walking) impaction/constipation back flexibility [en.wikipedia.org]

  Symptoms of ependymoma are dependent on the location of the tumor, with intracranial tumors causing increased intracranial pressure, headache, ataxia, seizures, and nausea and vomiting, and spinal tumors causing weakness and back pain. [clinicaladvisor.com]

  Common symptoms may include: Nausea and vomiting Lethargy and irritability Headaches Clumsiness Difficulty with tasks like handwriting Gradual decline in school performance Changes in personality and behaviour Altered way of walking If the tumour spreads [royalmarsden.nhs.uk]

  This can lead to increased intracranial pressure (ICP, which is the pressure inside your head), which can cause headaches, nausea, vomiting, and dizziness. Symptoms may be sudden or they may start slowly and get worse over time. [saintlukeshealthsystem.org]

  As the tumor increases in its size, irritability, sleepiness, and vomiting may occur In older children and adults, vomiting and headache are often observed The symptoms also depend heavily on the location of the brain tumor: An Ependymoma near the brainstem [dovemed.com]

• Abdominal Pain

  These years I can barely withstand all the physical problems I face every minute: from double vision, headaches, balance trouble, legs that feel and respond like cooked pasta, trach pain, abdominal pains, swallowing difficulties to sheer exhaustion and [adultependymomabraintumor.com]

• 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]

  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]

  Non-mechanical back pain, especially in the middle or lower back, is the most frequent symptom of both benign and malignant spinal tumors. This back pain is not specifically attributed to injury, stress or physical activity. [aans.org]

• Headache

  With the recurrent tumor I had headaches, balance problems and severe double vision. In fact, I had so many physical complaints that I was overwhelmed. [adultependymomabraintumor.com]

  Signs and symptoms of Ependymomas could include: Headaches are one of the most common symptoms of Ependymomas, and are usually worse in the mornings In infants and younger children, one of the first symptoms is often head enlargement. [dovemed.com]

  Corticosteroids help lessen the swelling around the brain tumour and may relieve headaches and other symptoms. A commonly used corticosteroid is dexamethasone (Decadron, Dexasone). [cancer.ca]

  Signs and symptoms [ edit ] Source: [1] severe headache visual loss (due to papilledema) vomiting bilateral Babinski sign drowsiness (after several hours of the above symptoms) gait change (rotation of feet when walking) impaction/constipation back flexibility [en.wikipedia.org]

• Hyperreflexia

  He presented hypotonic brachial diparesis (grade 4), spastic paraparesis (grade 4-5), patellar hyperreflexia and clonus at the ankles, Babinski´s sign on the right foot. [omicsonline.org]

  Other signs evident upon physical examination may include spine tenderness, stiffening of gait, trophic changes of extremity, sensory loss, hyperreflexia, clonus, and scoliosis or torticollis (generally in children). [emedicine.medscape.com]

• Dizziness

  This can lead to increased intracranial pressure (ICP, which is the pressure inside your head), which can cause headaches, nausea, vomiting, and dizziness. Symptoms may be sudden or they may start slowly and get worse over time. [saintlukeshealthsystem.org]

  People with an ependymoma in the brain may have headaches, nausea, vomiting and dizziness. [cern-foundation.org]

• Vertigo

  Posterior fossa ependymoma presents in younger children and frequently includes symptoms and signs of increased intracranial pressure, such as headache, fatigue, diplopia, papilledema, nausea, vomiting, ataxia, vertigo, head tilt, or neck pain. [clinicaladvisor.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]

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].

Twenty-six patients who underwent treatment of spinal cord ependymoma at a single institution were retrospectively analysed. [ncbi.nlm.nih.gov]

Treatment Options for Childhood Ependymoma For information about the treatments listed below, see the Treatment Option Overview section. [cancer.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]

The main treatments for ependymoma are surgery and sometimes radiotherapy. Your treatment will depend on the size, grade and position of the tumour, and your general health. [macmillan.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]

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]

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]

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]

The etiology of intramedullary spinal tumors remains obscure but undoubtedly varies according to histology. [emedicine.medscape.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]

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]

Detailed Epidemiology Ependymoma occurs more commonly in children, in whom it is the third most common intracranial tumor. The incidence is slightly higher in male than in female patients. [clinicaladvisor.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. [en.wikipedia.org]

Pathophysiology The pathophysiology of intramedullary spinal cord tumors varies according to tumor type. Ependymomas are usually indolent, encapsulated tumors that are histologically benign. [emedicine.medscape.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]

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]

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