Since most patients present with symptoms of intracranial hypertension, treatment of hydrocephalus goes along with planning for the actual tumor resection. Unless the patient is rapidly deteriorating, urgent CSF drainage is not necessary. At the time of surgery, a ventricular drain is usually placed in order to reduce brain tension and allow sufficient retraction. An external ventricular drain may be left in place after surgery in order to monitor ICP and to determine if shunting is required in the early postoperative period. Matson and others have reported that the successful removal of a tumor obviates the need for shunting. However, it is likely that other factors such as ventricular bleeding, postoperative changes, or meningitis can also render the patient shunt dependent. Ellenbogen’s series noted that 37 % of surviving patients required shunting (Ellenbogen et al. 1989). Two other series reported much higher rates of shunt dependency, ranging from 57 % to 78 % of cases reported (Humphreys et al. 1987; Lena et al. 1990). There is some evidence that patients with larger tumors, or hydrocephalus present prior to surgery, are at higher risk for requiring permanent CSF diversion (Safaee et al. 2013 #154).

Conventional catheter angiography is not required for diagnosis. Rather, its primary role is as a preoperative adjunct to define the blood supply and can be combined with embolization to reduce tumor vascularity. Angiography clearly indicates that the vascular supply of papillomas is from normal choroidal vessels, which often enlarge as the tumor grows. Tumors of the lateral ventricle or third ventricle are generally supplied by branches of the anterior or posterior choroidal arteries. Mass effect tends to displace the internal occipital artery and the basal vein of Rosenthal in an inferior direction. A fourth ventricle tumor receives its blood supply from medullary or vermian branches of the posteroinferior cerebellar artery.

The goal of surgery is gross total resection (GTR), as measured by postoperative MR imaging. As with most intracranial tumors, the exact approach is determined by avoiding eloquent tissue (primary motor or sensory cortex, speech centers, and visual cortex). The two features of choroid plexus tumors that can make resection exceedingly difficult are (1) profuse vascularity and (2) large size. The tumor’s arterial vessels arborize rapidly, and so control of hemorrhage within the tumor requires slow and tedious dissection. The most effective strategy focuses on initial exposure of the feeding artery and its ligation (Fig. 9.4). In general, en bloc excision is recommended (Raimondi and Gutierrez 1975). For lateral ventricle papillomas, a cerebral incision posterior to the angular gyrus allows access to the entire trigone and permits the pedicle of the tumor to be identified and coagulated. For more anteriorly located tumors, an incision can be made in the frontal convolutions and the lateral ventricle approached from an anterolateral direction. Lateral ventricle tumors can also be approached through a cerebrotomy through the superior or middle temporal gyrus.

Third ventricle tumors are rare and are approached usually through a midline transcallosal route. The anterior aspect of the ventricle is entered through a generous opening in the corpus callosum extending from the rostrum to the supraoptic recess. In this way, the tumor can be separated from the choroid of the tela choroidea where it is usually attached and the accompanying bridging vessels can be identified and divided.

Fourth ventricle tumors almost always produce triventricular obstructive hydrocephalus and may require preoperative shunting and stabilization as noted earlier. Tumors in this location arise from the caudal part of the roof of the fourth ventricle and may extend into the lateral recesses, or through the foramen of Magendie. The approach is through a standard midline posterior fossa craniotomy exposing the vermis and tonsils. The blood supply from branches of the PICA is visualized from a medial vantage.

In a recent systematic review, GTR clearly had a favorable impact upon survival for CPCs (Sun et al 2014). Nevertheless, GTR with carcinoma is achieved in less than 50 % of cases. Combined with adjunctive therapy, either radiation or chemotherapy, survival following GTR ranges from 67 % to 91 % (Fitzpatrick et al. 2002). Technical considerations with CPC include the expected increased tumor vascularity as well as additional difficulties relating to the lack of a well-developed plane between the brain and tumor and excessive friability of the tumor tissue. The rate of recurrence associated with GTR alone suggests that adjunctive therapy is useful, although definitive guidelines are not available (Fitzpatrick et al. 2002; Sun et al. 2014).

Most chemotherapy regimens rely upon cyclophosphamide, etoposide, vincristine, and a platinum agent (St Clair et al. 1991; Packer et al. 1992; Berger et al. 1998). Wolff et al. noted that only 8 of 22 carcinomas responded to chemotherapy, a disappointing observation (Wolff et al. 2002). Use of combination chemotherapy (ifosfamide, carboplatin, and etoposide) after an initial surgical procedure was found to reduce tumor volume and allow a more complete resection during a second-stage operation (St Clair et al. 1991; Razzaq and Cohen 1997; Lafay-Cousin et al. 2011). Importantly, the vascularity of the tumor appeared to be greatly reduced, as measured blood loss during the second procedure was on an average 15 % of blood volume compared to an average of 64 % of blood volume during the first procedure. Recent meta-analyses have noted that administration of chemotherapy resulted in a survival advantage for patients with completely or incompletely resected carcinomas and that second-look surgery is of benefit for those patients with incompletely resected CPCs (Wrede et al. 2005, 2007). Chemotherapy was also beneficial in the subgroup of patients who did not receive radiation. These observations, although retrospective in nature, suggest that aggressive therapy including chemotherapy and further attempts to remove any remaining tumor should be pursued when possible.

Postoperative radiation is usually recommended if the child is over 3 years of age, although this therapy has not been subjected to a clinical trial. Radiation is also used in the presence of leptomeningeal dissemination, subtotal resection (STR), and drop metastases. In one series, ten patients with CPC were treated with either chemotherapy or craniospinal radiation (Chow et al. 1999). Some of these patients demonstrated no evidence of disease following chemotherapy alone, but others required radiation to achieve disease control. The authors do suggest that radiation can be used as salvage therapy, but whether radiation for all patients with carcinoma would reduce the relapse rate remains unclear. Certainly, this should be judiciously used in children under 3 years of age. Fitzpatrick et al. noted that following STR, radiation therapy, either alone or in combination with chemotherapy, offered a survival advantage (Fitzpatrick et al. 2002). The question of which adjunctive therapy to use following GTR remains unclear, although the presence of relapse despite chemotherapy and radiation suggests that surgery alone is not sufficient for CPC. Wolff et al. support this view and state that GTR alone is insufficient for carcinoma and should be supplemented with radiation (Wolff et al. 1999). The role of conformal radiation and radiosurgery is unknown, nor is the role of intrathecal chemotherapy. The experience reported by Packer suggests that disease relapse confers a poor prognosis (Packer et al. 1992).