Current Management of Choroid Plexus Carcinoma in Children

Abstract

Eleven children with choroid plexus carcinoma are reviewed with respect to presentation, radiographic diagnosis, treatment and pathologic histology. These cases accumulated over a 33-year period and represent 39% of all of our choroid plexus tumors (n = 28). Characteristically, choroid plexus carcinomas are associated with a poor prognosis for long-term survival. Unfortunately, efforts at total resection are hindered in part by the extreme vascularity of the tumor which itself may be of considerable volume. We have recently treated four children who had biopsy or minimal resection of their tumor after which adjuvant chemotherapy was given. Chemotherapy is notcurative but it does cause a reduction in tumor volume and, more importantly, has tended to reduce the tumor vascularity. This allows for a second stage operation which is more safely performed and typically allows for more complete tumor removal. At this point, our length of follow-up is insufficient to conclude if total removal in this manner significantly increases survival but it would seem that consistent gross total removal may be an initial step toward better overall management and prognosis in this tumor type.

Prolonged Survival for Choroid Plexus Carcinoma with Oncocytic Changes: A Case Report

“…Gross total resection is the goal, and pharmacologic intervention including chemotherapy and radiation therapy remains controversial, as this treatment is not curative [3]. We would like to share this unusual case with the medical community to increase clinical understanding in the management and treatment of this malignant tumor.…”

“…Despite multiple reports about the benefits of chemotherapy and radiation therapy, medical treatment for this tumor type is not curative [3]. In a study of an eight month old infant who achieved remission with chemotherapy, local tumor recurrence was reported 13 months after initiation of treatment [14].…”

“…CPCs are aggressive tumors and have a high incidence of recurring and spreading to multiple regions of the body [1]. Several articles recommend chemotherapy before tumor resection in order to increase the probability of complete removal by shrinking the tumor in size and vascularity [1,3,12,15,16] Souweidane et al reports a patient whose tumor size shrank by 29.5% after receiving three cycles of chemotherapy consisting of VP-16, cyclophosphamide, vincristine, and cisplatin [16]. However although total surgical resection was achieved, the patient reportedly was only in remission for 31 months [16].…”

“…However although total surgical resection was achieved, the patient reportedly was only in remission for 31 months [16]. In an article by St. Clair et al pre-operative chemotherapy including VP-16, ifosfamide, and carboplatinum allowed for a safer two-stage tumor removal, due to reduction of tumor vascularity [3].…”

Current Treatment Approaches for Infants with Malignant Central Nervous System Tumors

“…Aggressive resection may be very challenging because of the invasive nature and extreme vascularity of CPCs. In order to achieve a GTR, some authors have suggested a staged approach through the use of neoadjuvant chemotherapy following biopsy of disease [98,101]. For those patients whose tumors are completely resected at diagnosis, the role of adjuvant chemotherapy, radiation therapy, or both remains controversial [100,[103][104][105].…”