18º Congresso Brasileiro de Neurologia Infantil

Dados do Trabalho


Título

HYPOGLOSSAL NERVE SCHWANNOMA: A CASE REPORT IN A PEDIATRIC PATIENT

Apresentação do caso

History: An 8-year-old previously healthy male patient sought medical attention for a four-month history of gait disturbance, associated with dysarthria and headache with progressive worsening. Upon examination, a MRI identified a heterogeneous mass with cystic components and magnetic susceptibility artifacts in between, suggestive of hemoglobin degradation/calcifications, measuring approximately 5.6 x 4.0 x 4.5 cm in the right ponto-cerebellar cistern. The mentioned expansive lesion determines extrinsic compressive effect on the right cerebellar hemisphere and brainstem, with obliteration of the IV ventricle, reduction of the amplitude of the mesencephalic aqueduct, contact with the sigmoid sinus laterally, and cranial displacement of the right trigeminal nerve.

Surgery: A right-sided retrosigmoid craniotomy was performed, with gross total resection, and two anchoring points were visualized next to the right cerebellar hemisphere.

Postoperative: The patient had a good postoperative outcome, except for persistent dysphagia, which led the medical team to contraindicate oral intake.

Diagnosis: Histopathological examination classified the tumor fragments as astrocytomas, however, immunohistochemistry showed that it was actually a Ki 67 1% schwannoma, indicating that the tumor is benign and has a low cellular growth rate, which is a good prognosis for the patient.

Discussão

Schwannoma of the Hypoglossal nerve is a benign tumor developed from the Schwann cells that involve that nerve. This type of tumor accounts for only 5% of the non-vestibular schwannomas. In addition, this disease rarely occurs in pediatrics patients, turning the described case relevant to the scientific community. Common symptoms include: dysphagia, hoarseness, cervical pain and tongue paresis. These symptoms may vary depending on the location of the tumor - usually intracranial. With slow growth, the schwannomas usually cause clinical manifestations when they are approximately 2 cm in diameter. Complete surgical resection is the treatment of choice, but it can be hard because of the important neurovascular structures that surround it. In pediatric patients, prognosis is usually favorable.

Comentários finais

The report presents a rare case of hypoglossal schwannoma, with an unusual patient age and tumor characteristic, which may be useful for the medical community dealing with similar cases.

Referências (se houver)

Santarius, T., Dakoji, S., Afshari, F. T., Raymond, F. L., Firth, H. V., Fernandes, H. M., & Garnett, M. R. (2012). Isolated hypoglossal schwannoma in a 9-year-old child. Journal of neurosurgery. Pediatrics, 10(2), 130–133. Available in: https://doi.org/10.3171/2012.3.PEDS11555

Fornaro, R., Salerno, A., Filip, D. C., Caratto, E., Caratto, M., & Casaccia, M. (2017). Schwannoma of the hypoglossal nerve: Review of the literature based on an illustrative case. Molecular and clinical oncology, 7(2), 288–294. Available in: https://doi.org/10.3892/mco.2017.1297

Palavras Chave

Schwannoma; Hypoglossal nerve; Pediatric neurosurgery

Declaração de conflito de interesses de TODOS os autores

We, Victor Alves de Souza; André Bedin, Jorge Wladimir Junqueira Bizzi; Samir Cezimbra dos Santos; Octávio Ruschel Karam; Ana Clara Medeiros de Souza, Gabriel Bolner, Manuella Giusti Fin and Morghana Machado da Rosa, certify that we do not have any conflicts of interest relating to the article.

Área

Neoplasias

Instituições

Santa Casa de Misericórdia de Porto Alegre - Rio Grande do Sul - Brasil, Universidade Federal de Ciências da Saúde de Porto Alegre - Rio Grande do Sul - Brasil

Autores

Victor Alves Souza, André Bedin, Jorge Wladimir Junqueira Bizzi, Octávio Ruschel Karam, Ana Clara Medeiros de Souza Thibes, Samir Cezimbra dos Santos, Gabriel Bolner , Manuella Giusti Fin, Morghana Machado da Rosa