Management and Outcome

Surgery has assumed an important role in the management of pineal region tumors. Radiographic features are not diagnostic and therapy and outcome are dependent on tumor type. Management of hydrocephalus requires urgent attention, since patients can develop acute obstruction and a herniation syndrome. Nearly all patients with pineal region tumors present with symptomatic hydrocephalus. The standard of care has been placement of a ven-triculoperitoneal shunt (VPS), at which time CSF can be collected. More recently, endoscopic management of obstructive hydrocephalus with a ventriculostomy, CSF sampling and biopsy of the tumor has gained popularity. Disadvantages of a permanent VPS for the initial management of hydrocephalus in these patients include shunt malfunction, infection and, rarely, peritoneal seeding of tumors. In many cases, if the tumor can be removed, the patient may not need the shunt at all. Endoscopic third ventriculostomy allows for successful treatment of the obstructive hydrocephalus, collection of CSF for markers and cytology and allows for multiple biopsies under direct endoscopic vision as a one-step procedure [22]. We have utilized this approach in the initial management of all patients with pineal region tumors presenting with hydrocephalus. If the biopsy is adequate and demonstrates a germinoma or primary malignant glioma without significant mass effect, the patient is managed with radiation and chemotherapy. For NGGCT, pineal cell tumors and large glial tumors, the patient can then undergo a more elective craniotomy appropriate for the location and size of tumor. Stereotactic biopsy of tumors of this region has also gained popularity but still carries a risk of damage to the complex venous anatomy. A concern with either endoscopic or stereotactic biopsy is sampling error. Germinomas can contain nests of malignant germ cell tumor that would significantly alter therapy and outcome. Likewise, a glial tumor may contain mixed cell types or focal areas of a higher-grade neoplasm, although this is less of a problem since radiographic appearance can help in choosing the most appropriate region to biopsy.

There are many surgical approaches to the pineal region (Figure 13.5). Three surgical approaches are most commonly used: infraten-torial supracerebellar, suboccipital transtentorial and paramedian transcallosal. The surgeon's degree of comfort and experience with the procedure and the size and extension of the tumor should determine the most appropriate approach chosen in order to minimize complications.

Surgical results depend more on the tumor's invasiveness and relationship with surrounding structures than on which approach is utilized. The deep location of these tumors makes surgery risky, with possible damage to the tectum, thalamus or deep venous system. The supratentorial approaches are best suited for

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