# Astrocitoma/ganglioglioma desmoplásico da infância

> Página oficial: https://raras.org/doenca/astrocitomaganglioglioma-desmoplasico-da-infancia
> Fonte: Raras.org — Banco de Dados de Doenças Raras em Português (CC BY-NC-SA 4.0)
> Última atualização: 2026-05-07

## Identificadores

- **ORPHA**: 251940 — https://www.orpha.net/en/disease/detail/251940
- **CID-10**: D33.0

## Descrição clínica

Desmoplastic infantile astrocytoma/ganglioglioma are mixed neuronal-glial tumors representing a histological spectrum of the same tumor. They are usually supratentorially located, large, cystic masses with a peripheral solid component, characterized by prominent desmoplastic stroma and pleomorphic populations of neoplastic cells with either astrocytic or ganglionic differentiation and poorly differentiated cells in variable proportions. They usually present in the first 18 months of age with rapid head growth, bulging anterior fontanel and bone structures over the tumor, signs of raised intracranial pressure (headache, vomiting, papilledema), focal neurological signs and sometimes seizures.

## Epidemiologia e herança


## Ensaios clínicos ativos (4)

- **NCT03975829** [ACTIVE_NOT_RECRUITING]: Pediatric Long-Term Follow-up and Rollover Study — https://clinicaltrials.gov/study/NCT03975829
- **NCT04923126** [RECRUITING]: SJ901: Evaluation of Mirdametinib in Children, Adolescents, and Young Adults With Low-Grade Glioma — https://clinicaltrials.gov/study/NCT04923126
- **NCT02684058** [COMPLETED]: Study of Efficacy and Safety of Dabrafenib in Combination With Trametinib in Pediatric Patients With BRAF V600 Mutation Positive LGG or Relapsed or Refractory HGG Tumors — https://clinicaltrials.gov/study/NCT02684058
- **NCT03115164** [UNKNOWN]: Astrocytoma / Desmoplastic Gamliogliomes (DIA / DIG) - Study of the French Cohort of the Last 20 Years : Clinical, Anatomopathological, Molecular and Radiological Charactersics — https://clinicaltrials.gov/study/NCT03115164

## Importante

O Raras **não diagnostica e não prescreve**. Esta página é educativa e informativa.
Pacientes devem consultar profissionais de saúde qualificados para decisões clínicas.

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**Citação sugerida**: Raras.org — Astrocitoma/ganglioglioma desmoplásico da infância. Disponível em: https://raras.org/doenca/astrocitomaganglioglioma-desmoplasico-da-infancia
**Formato HTML**: https://raras.org/doenca/astrocitomaganglioglioma-desmoplasico-da-infancia
**Formato RDF/Turtle**: https://raras.org/api/rdf?orpha=251940