Tips for Managing Pineal Region Tumors in Children – Tad Tomita, M.D. and Amanda Muhs Saratsis, M.D.

Utilizing Clinical Information to Dictate Treatment

One of the great challenges to managing tumors of the pineal region is the deep-seated location and widely varied pathology of these lesions, with equally varied responses to available treatments. It is therefore imperative to use all available tools at the clinician’s disposal to determine tumor pathology in order to tailor the appropriate treatment, including medical vs. surgical management, and, when warranted, the best surgical approach. For example, as mentioned above, pineoblastomas and teratomas (mature or immature) respond poorly to adjuvant therapy and hence require surgical removal, while germinomas are exquisitely sensitive to radiation and chemotherapy and hence do not require complete surgical removal once the diagnosis is confirmed. In addition, marker-positive NGGCTs may be treated with chemotherapy and radiation alone, followed by second look surgery if response is not adequate. In all cases the clinician must use his or her judgment and the available data, including clinical presentation, radiographic appearance, presence of tumor markers, and tissue histopathology to determine the best course of treatment.

Choosing an Approach

As discussed, the anticipated pathology and tumor anatomy must be carefully considered when choosing a surgical strategy. The possible need for CSF diversion and extent of resection must factor into the clinician’s surgical goals and approach. The various approaches listed here, including open vs. endoscopic techniques, can all provide surgical success depending on tumor anatomy, pathology, and surgeon comfort. The senior author has experienced excellent success with an occipital transtentorial approach with even large midline lesions.

Successful Management

Success in treating pineal region tumors is largely dependent on the pathology of the tumor. When complete resection is possible, total cure can be achieved for benign lesions such as pineocytomas or pineal region astrocytomas. Close follow-up and study of postoperative radiologic imaging can ensure an excellent result. In cases of malignant pathology or residual tumor, careful consideration of the use of adjuvant treatments and reoperation can make the difference in patient outcomes.