Abstract
Pineal parenchymal tumors of intermediate differentiation (PPTIDs) account for a significant proportion of pineal tumors and are classified as grade II/III according to the WHO classification. The management of PPTIDs remains controversial because of limited reported cases and the absence of standardized treatment guidelines. We present a case of an eight-year-old male child who presented with vomiting and a sudden squint of the eyes. Imaging revealed a well-defined heterogeneous enhancing lesion in the pineal region with acute hydrocephalus. The patient underwent surgical resection, and the tumor was diagnosed as PPTID. Local recurrence occurred 10 months later, requiring a second surgical intervention and adjuvant radiation therapy. A follow-up showed a regression of the tumor and improvement in symptoms. A literature review of reported PPTID cases revealed variability in clinical presentation, treatment approaches, and outcomes. Headaches were the most common symptom, and surgical resection was the primary treatment modality. Adjuvant therapies such as radiation therapy and chemotherapy were utilized in some cases. Tumor recurrence was observed in several instances, underscoring the need for long-term follow-up. In conclusion, PPTIDs are rare brain tumors with challenging diagnosis and management. Surgical resection remains the mainstay of treatment; however, the optimal approach is uncertain. Standardized reporting and larger studies are necessary to establish guidelines for the management of PPTIDs and improve long-term outcomes.