Choroid plexus tumors: An institutional series of 25 patients

Abstract

Choroid plexus tumors (CPT) are rare neoplasms that pose considerable treatment challenges. This study reviews a single institute's experience with 25 patients of CPT and attempts to contribute to the general body of knowledge on CPT. A retrospective analysis of the case records of 25 patients operated for CPT since January 1998 and having a minimum of 1 year follow-up. The study group included 12 (48%) cases of choroid plexus papilloma (CPP), 09 (36%) cases of choroid plexus carcinoma (CPC) and 4 cases of atypical CPP. The mean age at presentation was 18.6 years (range, 6 months to 54 years; SD, 18.7) and a male preponderance was noted (17:8). Raised intracranial pressure was the commonest presenting symptom (72%). The tumors were distributed as follows: lateral ventricle (16; 64%), fourth ventricle (5; 20%), fourth ventricle with cerebellopontine angle extension (3; 12%), and third ventricle (1; 4%). A complete surgical excision was achieved in 11 cases of CPP and 8 cases of CPC. Operative complications include pneumocephalus (40%), focal deficits (36%), subdural effusion (32%), and persistent hydrocephalus requiring shunt (24%). All patients with CPP had a good outcome at the end of a mean follow-up of 5.4 years, whereas the median survival for patients with CPCs who underwent a subtotal resection with adjuvant therapy was 36 months. CPTs include a spectra ranging from CPP to CPC. Radiologic and histologic characterization of these tumors is difficult and newer immunohistochemical and genetic studies should be done to differentiate them from each other. Total excision offers a good prognosis and should be attempted for all forms of CPTs. CPPs carry a good prognosis, and adjuvant therapy is not indicated even after partial excision. CPCs and atypical CPCs carry a poor prognosis, and adjuvant therapy improves survival marginally after total excision. Spinal drop metastases are common for CPC and screening of the spine for possible metastasis should be part of the routine preoperative and postoperative investigation protocol.