The 20th Congress of RSCANP,

Băile Felix, 18-21.09.2019

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The 42st National Conference of Child and Adolescent Neurology and Psychiatry and Allied Professions with international participation


SPINAL MENINGIOMA IN CHILDREN

Autor: Svetlana Hadjiu Cornelia Călcîi Anatolie Litovcenco Anastasia Railean Carolina Golovcenco Tatiana Lozan Ana Antohi Maria Goțonoagă
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Spinal tumors are benign or malignant formations that occur within or near the spinal cord or vertebrae. Neuroepithelial meningioma is a tumor that develops in the brain or spinal meninges. On rare occasions, there are malign forms of spinal tumors.Spinal meningiomas are expansive processes that can remain misdiagnosed  for a long period of time, growing  slowly and manifesting subtle and insidious symptoms. They predominate in women of middle age. The frequency of pediatric studies ranges from 1-17%. Relationship between intracranial and spinal meningiomas in adults is 4:1, in children the rate is 20:1.

Clinical case

A 3-year-old child was admitted to the Neurological Clinic of the Institute of Mother and Child Healthcare in September 2012 accusing very pronounced abdomen pains, mostly at night, marked irritability and nervousness, muscle weakness, difficulty in standing and walking. The pain was very pronounced and the child could not move, or better said, when the child was put to stand, he began to cry and become extremely agitated.

History of the disease: The child had been ill for 2 weeks when on a background of an acute viral illness the child started experiencing pain in the abdomen. The child was hospitalized in the district hospital, where the surgeon was originally consulted to rule out acute pathology. Subsequently, the child was consulted by the gastroenterology physician, who prescribed the child to undergo the USG of the internal organs, blood examination for evaluating biochemical tests and blood count. All the examinations have not reported any changes, therefore the child was transferred to the Neurological Clinic of the Institute of Mother and Child Healthcare in Chisinau.

Life History: The child was born in term weighing 3400 g, Apgar score 8/9 balls. Psycho-motor and verbal development was according to the age. Physical examination revealed a very sick child face, ringed eyes, pale skin. Any changes in the lung and heart auscultation were not noted. Neurological examination of the child revealed a diminished muscle strength in the legs, lower tendon reflexes overactive bilateral, Babinski and Oppenheim signs were present , and the elongation reflexes (Lasseque, Wassermann, Maţchievici) were present. A bilateral clonus was revealed. The patient had no sphincter disturbance. Sensorial disturbances could not be evaluated because of marked agitation.

Analyses carried out: Laboratory tests revealed normal values. The spinal MRI 3 Tesla has reported the presence of a spinal process (suggestive of meningioma) with compression of spinal roots.

 

Discussions

Modern imaging methods have revolutionized the diagnosis of spinal cancer in the last 30 years. Widespread availability of magnetic resonance imaging methods (MRI) decreased delayed diagnosis. Peak incidence is in the fifth or sixth decade of life, although they have been reported in all age groups. This disease is most commonly seen in women, with a sex ratio of 3:1 – 9:1. The tumor is found most commonly in the thoracic spine. Meningiomas can have an intradural – extramedullar location (83%) or an extradural component (5-14%). Usually, spinal meningiomas occur localized posterolaterally in th spinal cord, while cervecale Meningiomas are usually encountered in an anterolateral location. Intracranial and spinal ratio is about 8:1.7. Extramedullary spinal meningiomas in children occur most commonly in the thoracic spine. In general the number of cases are very few in the studies conducted on children, so it is difficult to establish certain epedimiological data on this pathology.

Histological subtypes of meningioma include meningoteliale, fibroblastic, transitional, angiomatoase, psamomatoase meningiomas. Although malignant meningiomas in the spine are encountered, they are quite rare and are pathologically marked by the invasion of adjacent tissues, nuclear pleomorphism and mitosis.

A progesterone receptor activation was found in meningiomas, and this explains the predominance of meningiomas in association with female breast cancer. A translocation involving chromosome 22 has been described as predictive factors.

Multiple spinal meningiomas are rare and are seen in patients with neurofibromatosis in patients receiving radiation for other reasons. Extradural meningiomas are a different subtype of meningioma which behaves in a more aggressive manner. For extradural meningiomas reported incidence ranges from 2.7-10%, and because of their location these can be mistaken for metastases. Very rarely other malignant tumors in other parts of the body can metastasize directly into the dura mater and therefore may be confused radiologically with meningiomas. The predominant clinical signs are progressive weakness and pain. It is not uncommon for patients to go for a while without a diagnosis because of the insidious nature of the onset of symptoms. The pain often is located in the back – 80% of the cases.

Radicular symptoms are observed. Because of its location myelopathy signs may precede radicular symtoms. Since most meningiomas occur in a location somewhat side ipsilateral weakness and symptoms can be observed sensorially. Examination of a patient with a spinal meningioma is characterized by typical myelopathy stigmas. Changes in reflexes are hallmarks of myelopathy and are present in almost 80% of cases.

Weakness in the extremities are characteristic and presented in two thirds of patients. Sensorial dysfunctions are seen in approximately one third of patients, including numbness, paresthesia. Disruption of body temperature, pain in the chest may also be present. Bowel and bladder dysfunction are encountered later and are reported in approximately 40% of cases.

It should be borne in mind that any disease which causes dysfunction of the spinal cord can produce symptoms and signs characteristic for meningiomas. Entities that can mimic these clinical signs are syringomyelia, spondylitis myelopathy, multiple sclerosis and spinal extradural or spinal tumors.

In addition to a complete history and physical examination, all patients suspected of having a tumor of the spinal cord would have to undergo MRI imaging. Spinal meningiomas have a homogeneous appearance on MRI and are izointense on both T1-T2 images. Although many meningiomas have microscopic calcification, only sometimes visible on radiographs of the spine. Myelography was once the “gold standard” for assessing spinal meningiomas. They can still provide excellent images of the lesion in patients with absolute or relative contraindications to MRI. All patients with meningiomas are treated operatively. Most patients can be programmed because the symptoms are slowly progressive. There are, however, patients with neurological decline that require emergency decompression and tumor removal. All patients should be treated preoperatively with corticosteroids. The aim of surgical management of spinal meningiomas is their complete resection. Relapse rates of intracranial and spinal meningiomas depend on the degree of surgical resection.

 

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