Rare Onset of Erdheim-Chester Disease in Children and Young Adults: A Case Series and Review of the Literature.
Pediatr Neurosurg
; 59(1): 35-43, 2024.
Article
de En
| MEDLINE
| ID: mdl-38262396
ABSTRACT
INTRODUCTION:
Erdheim-Chester disease (ECD) is a rare histiocytic neoplasm that affects patients, predominantly males aged 40-70 years, with very heterogeneous clinical presentation and prognosis. In 2020, Goyal et al. proposed consensus recommendations for the management of patients with ECD, remarking on the exceptional presentation of the disease in the pediatric population. CASE PRESENTATION The first patient, a 20-year-old male, underwent cervical laminectomy and partial removal of a cervical spine lesion, initially apparently consistent with cervical schwannomas. The second patient, a 9-year-old female, received surgery for an extra-axial lesion of the greater sphenoid wing, radiologically consistent with a meningioma.CONCLUSION:
At present, 15 pediatric cases have been reported in the literature with involvement of the central nervous system, with no consensus on the diagnostic and therapeutic management, as Pegoraro et al. evidenced in their pediatric multicenter case series. The present article adds two new cases of ECD with onset in childhood and young adulthood, who received the diagnosis after neurosurgical procedures.Mots clés
Texte intégral:
1
Collection:
01-internacional
Base de données:
MEDLINE
Sujet principal:
Maladie d'Erdheim-Chester
/
Tumeurs des méninges
/
Méningiome
Type d'étude:
Guideline
/
Prognostic_studies
Limites:
Adult
/
Child
/
Female
/
Humans
/
Male
Langue:
En
Journal:
Pediatr Neurosurg
Sujet du journal:
NEUROCIRURGIA
/
PEDIATRIA
Année:
2024
Type de document:
Article