Management of Pineal Region Tumors in Children

Tumors of the pineal region may be managed with a combination of surgical and medical intervention on the basis of clinical presentation, laboratory markers, and radiographic findings.

Initial Management at Presentation

• Determining need for surgery: The initial assessment of a child with a pineal region tumor requires consideration of clinical and radiographic findings. Assessment must include the acuity of the child and the need for histological verification of tumor diagnosis, cytoreduction of tumor mass, and restoration of CSF pathways. These objectives may be achieved with CSF diversion and tumor resection procedures utilizing an open or endoscopic approach. Tissue histology is used to guide the decision for gross total surgical resection and the need for adjunctive therapy. For example, pineoblastomas and teratomas (mature or immature) require surgical extirpation due to their lack of response to adjuvant therapy.  
• Determining need for adjunctive therapies:Children with lesions that do not favor surgical intervention, depending on radiographic appearance and tissue histology, may be considered for chemotherapy or radiation treatment. Consideration of the need for temporary or permanent CSF diversion due to the presence of or risk for development of obstructive hydrocephalus must still be considered even in cases where surgical resection is not undergone.

Adjunctive Therapies

Tumors of the pineal region differ in terms of their responsiveness to chemotherapy depending on tumor histology.

• Germ cell tumors: Germinomas and NGGCTs demonstrate sensitivity to radiation and chemotherapy, while germ cell tumors and teratomas show minimal response to adjuvant therapy.
• Pineal parenchymal tumors: Pineocytomas are considered benign lesions, and surgical excision can lead to a cure.  In contrast, pineoblastomas typically affect infants and young children and tend to recur and disseminate without appropriate adjuvant therapy. 
• Astrocytomas of pineal region: Due to their intrinsic nature, astrocytomas are not often amenable to surgical resection. Although slow growing, they may exhibit dissemination and are responsive to chemotherapy and radiation.

Follow-up

• Imaging and serum tumor markers: Follow-up for children with pineal region tumors includes office evaluation, serial MRI, and laboratory testing for tumor markers, depending on tumor histology in order to monitor clinical symptoms and tumor progression or response to therapy.