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<p>Desmoplastic Infantile Astrocytoma (DIA) and Desmoplastic infantile Ganglioglioma (DIG) are rare <i>World Health Organization (WHO)</i> Grade 1 brain neoplasms that primarily affect children under two years of age. They consist of both neuronal and glial elements, and although the prognosis is favorable, some of these neoplasms may exhibit malignant biological behavior. We performed an updated narrative review to study this rare neoplasm's epidemiology, clinical features, treatment, and patients' prognosis. Relevant articles were retrieved by searching PubMed/<i>Medical Literature Analysis and Retrieval System Online (MEDLINE)</i> and <i>Scopus Database (SCOPUS)</i> databases. In total, 103 articles with 222 patients were included. The mean age of patients was about 33&#xa0;months, and a slight male predominance was observed. Hydrocephalus was the most common initial clinical manifestation in infantile cases, while seizures were most common in non-infantile cases. A High ki-67 index (over 20%) was observed in about 10% of the patients and was associated with a significantly raised risk of recurrence (p = 0.03). Various <i>B-Raf Proto-Oncogene, Serine/Threonine Kinase (BRAF)</i> mutations were observed in one-third of the cases, and a not-statistically significant correlation was observed between BRAF mutations and the risk for recurrence (p &lt; 0.01). Tumor total resection was achieved in 54.6% of the patients, and adjuvant therapy, primarily chemotherapy, was utilized in 14.8% of the cases. The mean progression-free survival (PFS) was 331.7 (95% Confidence Interval (CI): 270.6–392.8) months, and the mean overall survival was 379.8 (344.7–414.9) months. Recurrence was observed in 6.2% of the patients, while tumor dissemination was observed in 2.2%. Tumor's total resection, age group, and sex were not statistically correlated with worse PFS and OS. A fatal outcome and multifocal lesions were significantly associated with tumor recurrence or dissemination (p &lt; 0.05). DIA/DIG has a generally promising prognosis, high ki-67, and multifocal localization of the tumors, which may be factors related to a more aggressive course.</p>

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Desmoplastic infantile ganglioglioma and astrocytoma: A narrative review of the literature

  • 期刊论文

  • 发表日期：

    2025年10月22日

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  • Marios Lampros,
  • Petros Baxevanidis,
  • Konstantinos Tsegkelidis,
  • George A. Alexiou,
  • Spyridon Voulgaris

摘要

Desmoplastic Infantile Astrocytoma (DIA) and Desmoplastic infantile Ganglioglioma (DIG) are rare World Health Organization (WHO) Grade 1 brain neoplasms that primarily affect children under two years of age. They consist of both neuronal and glial elements, and although the prognosis is favorable, some of these neoplasms may exhibit malignant biological behavior. We performed an updated narrative review to study this rare neoplasm's epidemiology, clinical features, treatment, and patients' prognosis. Relevant articles were retrieved by searching PubMed/Medical Literature Analysis and Retrieval System Online (MEDLINE) and Scopus Database (SCOPUS) databases. In total, 103 articles with 222 patients were included. The mean age of patients was about 33 months, and a slight male predominance was observed. Hydrocephalus was the most common initial clinical manifestation in infantile cases, while seizures were most common in non-infantile cases. A High ki-67 index (over 20%) was observed in about 10% of the patients and was associated with a significantly raised risk of recurrence (p = 0.03). Various B-Raf Proto-Oncogene, Serine/Threonine Kinase (BRAF) mutations were observed in one-third of the cases, and a not-statistically significant correlation was observed between BRAF mutations and the risk for recurrence (p < 0.01). Tumor total resection was achieved in 54.6% of the patients, and adjuvant therapy, primarily chemotherapy, was utilized in 14.8% of the cases. The mean progression-free survival (PFS) was 331.7 (95% Confidence Interval (CI): 270.6–392.8) months, and the mean overall survival was 379.8 (344.7–414.9) months. Recurrence was observed in 6.2% of the patients, while tumor dissemination was observed in 2.2%. Tumor's total resection, age group, and sex were not statistically correlated with worse PFS and OS. A fatal outcome and multifocal lesions were significantly associated with tumor recurrence or dissemination (p < 0.05). DIA/DIG has a generally promising prognosis, high ki-67, and multifocal localization of the tumors, which may be factors related to a more aggressive course.