Intracranial undifferentiated pleomorphic sarcoma of right frontal lobe: a case report

BACKGROUND: Intracranial undifferentiated pleomorphic sarcoma (UPS) is extremely rare and mostly seen among the middle-aged people. The patients are asymptomatic or have atypical manifestations such as headaches and seizures. The imaging findings can be easily misdiagnosed as glioma. Surgical resection is the primary treatment, followed by adjuvant radiotherapy and chemotherapy; however, the average overall survival remains relatively short. CASE PRESENTATION: In this article, we report the case of an 11-year-old girl who presented with headache, dizziness, and focal seizures. Imaging found an irregular lesion in the right frontal lobe, with heterogenous contents and mass effect. The patient underwent resection and postoperative pathological examination confirmed UPS. Adjuvant radiotherapy and chemotherapy were then administered. At the 35-month follow-up, the patient was in good condition with no evidence of recurrence. CONCLUSIONS: This case highlights the diagnostic and therapeutic challenges of intracranial UPS, particularly in pediatric patients, given its rarity and nonspecific clinical and radiological features. Although UPS is typically aggressive with a poor prognosis, early and complete surgical resection combined with adjuvant radiotherapy and chemotherapy may improve outcome.