"Ependymoma". Product of a real clinical case

in steemstem •  3 months ago 

Hello everyone ... In this new article I want to present you with a clinical entity that is not frequent, but that by chance was diagnostic in the Internal Medicine service. Although in spite of the fact that brain tumors are pathologies affecting the CNS and that clinical manifestations are determined according to the location and characteristics of it.

human brain on white background. Public domain image source Flickr

Every year the malignant tumors of the Central Nervous System (CNS) represent a high mortality rate with a high risk of metastatic lesions, with an index of six per 100,000 individuals.

The frequency of diagnosis of benign tumors is similar, but with a much lower mortality rate.

According to its characteristics and incidence, glial tumors represent approximately 60% of all primary brain tumors, while meningioma accounts for 25%, neurinomas with figures of 10%, and the remaining 5% for other CNS tumors.

Lesions metastases in any other system of the body and even in the vertebral bodies have higher prevalence than primary tumors of the CNS. About 15% of patients who suffer or die from oncological pathologies have symptomatic brain metastasis lesions in more than 50% of cases and an additional 5% suffer from spinal cord involvement.


Anatomically the Nervous System (SN) is divided into two sub-groups or systems, which are:

Central Nervous System (CNS) conformed by the encephalon (constituting cerebellum, brain, and brainstem) and the spinal cord. These structures represent the source of our thoughts, emotions, memory among others.
Peripheral Nervous System (SNP) that constitutes nervous tissue or better said cranial nerves, which connect the CNS with other systems of the organism.

Cortical and subcortical brain regions involved in the overt estimation of elapsed time (explicit timing). The functional role of different areas in the diverse information processing stages is also specified. Public domain image licensed CC BY 3.0

Brain Cells:

Our nervous system is composed of two types of cells:

1.- The neuron functional and basic unit of the nervous system, which allows the passage of the nerve impulse and in turn, perform functions such as muscle activity. It is composed by;

  • Body or neuronal soma.
  • The dendrites.
  • The Axon.
  • 2. Neuroglia are all those cells whose sole function is to support neurons in the nervous system. These cells are;

  • Astrocytes.
  • Oligodendrocytes.
  • Microglia.
  • Ependymal cells.
  • Schwann cells.
  • Satellite cells.
  • Cerebrospinal Fluid and Ventricles:

    Crystalline liquid, like rock water, colorless, whose objective is to surround the entire nervous system, protect it from external agents and serve as a means of transport of substances such as glucose, oxygen, among others, vital for the proper functioning of the Central Nervous System.

    This originates in a structure called Choroid Plexus, which in turn is composed of ependymal cells.

    This liquid is distributed throughout the central nervous system by means of structures called ventricles, which are only small cavities.

  • Lateral ventricles, located in the latero-medial portion in each hemisphere of the brain.
  • III Ventricle.
  • IV Ventricle.

  • Ventricles of the Brain. Author: BruceBlaus. Public domain image licensed CC BY 3.0

    Brain tumors

    ¿How are they Clinically Manifested? Signs and symptoms

    In general, brain tumors are manifested by one of three syndromes:

  • The focal neurological deficit, expressed, as previously described, in hemiparesis or what is equal loss of muscle strength to predominance either right or left, which will depend on the location of the space-occupying lesion (LOE), as well as alteration of cranial pairs, dysarthria, aphasia among others.
  • Convulsions, which are no more than generalized clonic tonic movements, which can coexist with ocular retroversion, and loss of sphincter control.
  • Non-focal neurological disorder; where the patient can simply tell us that he has several weeks or even months of headache (headache), dementia, personality changes, gait disorder, vertigo, vomiting, neurological deterioration.
  • In addition to what has already been described, it may present with what are known as general symptoms, such as discomfort, weight loss, anorexia, hyporexia.

    In relation to fever, it is usually a negative symptom as long as it is not related to an infectious process, since it is usually products of terminal metastatic lesions compared to a primary brain tumor, where fever is not usually common.

    Sometimes these lesions at the brain level can be confused with a cerebral vascular event due to the similarity of the clinical presentation of the patient, but which are later discarded at the time of the realization of Neuroimaging, either Tomography of the Skull, or simple Brain Resonance that are the studies that are requested at first instance.

    It is important, always during the realization of the current disease of a patient that we suspect some space-occupying lesion at the brain level those Non-Focal Manifestations such as vomiting, especially that referred in the form of a projectile, nausea, blurred vision, drowsiness, since the establishment of the same tells us that we are probably under the context of ENDOCRANEAN HYPERTENSION SYNDROME.

    This is due to an increase in the pressures proper, but at a cerebral level, triggered by the space-occupying lesion.

    Those tumors that originate in certain areas of the brain, such as the FRONTAL LOBE can produce usually manifested by personality changes, dementia or depression.

    As for the headache that is nothing more than the headache that the patient comes to refer to, it has a characteristic typical of itself, since these are also secondary to intracranial hypertension, they are usually holocephalic and episodic and occur several times a day.

    Once the headache is established has a period of approximately 20 to 40 minutes, when the episodes are headache accompanied by vomiting it is very likely that there is already an increase in severe intracranial pressure and that in turn we can observe in this patient's what is called papilledema after performing an eye fundus.

    Diagnostic tests

    The techniques of positron emission tomography (Cerebral CT) is one of the techniques used to determine the presence of a space-occupying lesion, which allows us to observe the location, its characteristics and behavior.

    In relation to more specific studies once we determine the presence of LOE at the cerebral level, it is very useful, the cerebral NMR with spectroscopy and gadolinium.

    The biopsy of the lesion is what allows us to determine the histology of the tumor and therefore is the gold standard method.


    The treatment in most of these patients is symptomatic, in case we are under the presence of an endocranial hypertension syndrome secondary to occupant injury of the cerebral space, the use of steroids, type Dexamethasone EV is recommended.

    In case of convulsive episodes, the use of antiepileptic drugs, type Epamin (sodium phenytoin), carbamazepine, valproic acid, is even recommended as prophylactic use, and even more if the location of the lesion is sub-cortical, in frontal lobe.

    Acetazolamine, as a prophylactic and for the treatment of endocranial hypertension, this drug has the function of being Diuretic, whose mechanism of action is to inhibit carbonic anhydrase. It is used at a dose of 250 mg orally every 12 hours.

    In relation to surgical treatment, it will be at the neurosurgeon's discretion and the location and association to adjacent structures in the central nervous system.


    As well, it had been described, Ependymomas are those that originate from cells of the ependyma, which are located in the cerebral ventricles where the CSF runs and in the spinal cord.

    Generally this type of tumors are usually one of the most common at the cerebral level, and with a better prognosis, with a degree of severity II, but nevertheless the presence of Anaplastic Ependymoma tumors that are high-grade (III) proliferative have been determined, with accelerated growth, they are usually located in the posterior cranial fossa and IV ventricle.

    Up to now, 4 types of ependymomas have been described:

  • Myxopapillary ependymomas.
  • Subependymomas.
  • Simple ependymomas.
  • Anaplastic ependymomas.

    Although ependymomas are tumors of the central nervous system, which come from the neuroectoderm from ependymal cells proper, which line the walls of the cerebral ventricles and the spinal cord, although, of course, this type of lesions They are more common in pediatric age if cases have been described in a small amount in adults, more frequently in males.

    Real clinical case report

    A 32-year-old female patient with no known medical history who reported onset of illness approximately 4 weeks ago characterized by insipidus-dominant occipital headache, which partially resolves with common analgesics, for which a previous week presents nausea and persistent vomiting, associated to vertigo and instability for walking, (ataxia), with progressive deterioration of the neurological state, expressed in drowsiness, which is why it is assessed.

    At the physical examination;

  • Blood pressure: 110/60 mmHg.
  • Heart rate: 89 Lpm.
  • Respiratory frequency 19Rpm.
  • Oxygen saturation: 96% ambient air.
  • Regular clinical conditions, afebrile to the touch, dehydrated, eupneic, skin and mucosa normocoloreada. The positive to the neurological examination, somnolent patient, oriented in person, disoriented in time and space, without alteration of cranial pairs, decreased muscle strength in upper limbs, III / V preserved sensitivity, normoreflexia with ataxic gait.

    A patient with no personal or family history who has a neurological clinic, paraclinical and imaging studies are requested.

    Magnetic Resonance with spectroscopy and gadolinium.


    Space-occupying lesion at the floor level of the IV Ventricle and inferior extension to the canal through the foramen, with neoplastic behavior, approximately 52x24 mm on its anterior-posterior longitudinal axis. with morphological and spectroscopic modifications suggestive of probable ependymoma.

    Final case discussion

    Although it has been described that this type of lesion is located more frequently at pediatric ages with an estimate of under 3 years, where some literature establishes that the highest incidence rate is found in the male sex, but nevertheless there are literatures and studies where they do not establish a significant predilection in any sex.

    The tumors located in the floor of the IV cerebral ventricle trigger a particular clinical situation, and this is the presence of vertigo, ataxia, and this is as a result of stimulation of the centers of vomiting located in said ventricles and by its proximity to the cerebellum whose mass it can compress the patient and produce cerebellar symptoms. In cases of large tumors, clinical signs of intracranial hypertension can be seen, as described above.

    The ideal treatment in this type of brain injuries, would be the total recession of the same but this will depend on its location and extension to neighboring structures, it is estimated that only 50% of diagnosed cases can have a satisfactory recession of injury, combined this is associated with the use of chemotherapy and radiotherapy.

    Sources of support in the publication

  • Brain Tumors
  • Epidemiology of Malignant and Non-Malignant Primary Brain Tumors in Jordan
  • The worldwide incidence and prevalence of primary brain tumors: a systematic review and meta-analysis
  • Epidemiology and etiology of meningioma
  • The biology of brain metastases—translation to new therapies
  • Neuroglia
  • Glia
  • Protection of the Brain
  • Lateral and third ventricles, underside of the cerebrum
  • Rapidly progressive dementia and ataxia in an elderly man
  • Headache with focal neurological signs or symptoms: a complicated differential diagnosis.
  • Brain Imaging in Stroke: Insight Beyond Diagnosis
  • What causes blurred vision and a headache?
  • Intracranial space occupying lesions
  • Diagnosis of frontal meningioma presenting with psychiatric symptoms
  • Space-occupying Lesions of the Brain
  • Advances in the understanding of headache in idiopathic intracranial hypertension
  • acetazolamide - Drug Summary
  • Ependymoma
  • Intramedullary Spinal Cord Tumors: Part I—Epidemiology, Pathophysiology, and Diagnosis
  • Ependymomas
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