Increase of primary intracranial sarcoma in children: Clinical manifestations, diagnosis, and management.

BACKGROUND

Primary intracranial sarcomas (PISs) are very rare malignant tumors, and there is paucity of data on it, exclusively in patients <18 years old. We report pediatric PIS at a tertiary hospital in Peru, where the incidence of PIS has increased in recent years.

METHODS

We retrospectively analyzed data in children diagnosed with PIS based on clinical presentation, imaging studies, and histopathology between January 2020 and December 2023.

RESULTS

Twenty-five cases were identified. The median age was 5 years. There is slight female predominance (56%). On presentation, 68% of patients had features of intracranial hypertension (ICH), others had convulsions or motor deficits. There was radiologic evidence of cerebral hemorrhage in 80% of those with features of ICH and convulsion. All but one case had a supratentorial tumor. Emergency craniotomy was done in 84% of cases, and gross total resection (GTR) was achieved in the first surgery in 72% of cases. We used an adjuvant chemotherapyradiotherapy-chemotherapy (CTX-RT-CTX) regimen in 72% of cases, but 12% started this scheme 2 weeks after surgical resection. The cases followed up for more than a year that were managed with CTX-RT-CTX after GTR had a survival greater than a year, compared to the cases that received complementary treatment after 4 weeks.

CONCLUSION

PIS among children represents an infrequent pathology that, in the last years, its incidence has increased in Peru. The presence of intracerebral hemorrhage is a very suggestive finding of this diagnosis; therefore, emergent surgical management is an option before an irreversible ICH presents. Adjuvant treatment with the CTX-RT-CTX regimen started 2 weeks after GTR may improve survival in children with PIS.