Spinal chordoid meningioma in a child: A case report and review of the literature

Abstract

Abstract.As an uncommon subtype of meningioma, chordoid meningioma (CM) of the spinal canal is extremely rare. There have been only two reported cases of intraspinal CM in the literature, and this lesion has not been previously reported in the pediatric age group. To the best of our knowledge, the present study reports the first case of spinal chordoid meningioma in a pediatric patient. A 12-year-old female presented with a 3-month history of progressive numbness and weakness in the right-side limbs, and intermittent pain in the neck and right shoulder. Spinal magnetic resonance imaging (MRI) revealed an intraspinal lesion at the C2-3 level with irregularly heterogeneous enhancement. The patient underwent a C2-3 laminotomy. Due to adhesion to the dura and proximity to the vertebral artery, the tumor was partially removed intraoperatively. The post-operative course was uneventful and the symptoms were apparently relieved. The patient experienced recurrence 5 years subsequent to surgery. MRI revealed an extradural regrown tumor at the C2-5 level. Partial removal combined with radiotherapy was performed. However, the patient experienced progression of tetraplegia and succumbed to severe pneumonia and respiratory failure 5 months subsequent to the second surgery. In the present study, the clinicoradiological findings and treatment outcome of this rare entity are discussed, in addition to a review of the relevant literature. Spinal CMs should be included in the differential diagnosis of intraspinal tumors of the pediatric spine. Multidisciplinary treatment, consisting of total surgical removal and adjuvant radiotherapy, should be considered due to the aggressive nature of this abnormality and the risk of long-term recurrence.

A Case of Thoracic Extradural Chordoid Meningioma: Focusing on Radiologic Features

“…Surgical removal of the tumor is the mainstay of treatment and complete excision is important to minimize local recurrence (2-4). The role of adjuvant radiation therapy is controversial (3,4).…”

Meningioma cordoide: revisión de la literatura y reporte de un caso

“…El meningioma cordoide fue documentado por primera vez en 1988 por Kepes, et al, (8), quienes describieron a 7 pacientes entre los 8 y 19 años con irregularidades hematológicas, en particular relacionados con la enfermedad de Castleman (9). El meningioma cordoide representa una rara variante que comprende menos del 0,5 % y el 1,0 % de todos los meningiomas intracraneales (10)(11), son más comunes en pacientes menores de 18 años (12), aunque se presentan en todas las edades siendo más agresivos a menor edad (2) sin diferencias por sexo (13). El meningioma cordoide se compone de células fusiformes o epitelioides dispuestas en racimos y los cables de cordoma como en una matriz mixoide ofrecen a menudo un prominente infiltrado lymphoplasmacellular (14).…”

“…Se ha encontrado que el síntoma más común en los pacientes con meningioma cordoide es la cefalea severa que no mejora con tratamiento convencional y los demás síntomas dependen de la localización y tamaño del tumor, sin manifestaciones clínicas específicas (13). Solo en los casos asociados a enfermedad de Castleman se correlaciona con anemia hipocrómicas resistente al tratamiento con hierro, se han documentado algunos casos de disgammaglobuli-nemia con retraso del crecimiento (27) y se han reportado episodios de convulsiones tónico-clónicos generalizadas, pero este hallazgo es excepcional (28,29).…”