Choroid Plexus Tumors: Experience of 10 Cases with Special References to Adult Cases

Abstract

Choroid plexus tumors (CPTs) are rare intraventricular neoplasms accounting for about 0.3–0.6% of all intracranial tumors. This retrospective study on CPTs presents clinico-pathological features and management strategies based on a 20-year single-institutional experience. This series included 10 consecutive patients with pathologically proven CPTs; 5 choroid plexus papillomas (CPPs), 3 atypical CPPs (ACPPs), and 2 choroid plexus carcinomas (CPCs). Their clinical, radiological, and histopathological features as well as management including follow-up studies were reviewed. The patients included five males and five females, aging from 0 years to 61 years with median of 28 years. The affected site was lateral ventricle in two adults and fourth ventricle in eight patients; four children and four adults. The most common symptoms were gait disturbance and memory disturbance. All the patients underwent craniotomy with total, subtotal, and partial removals achieved in 50%, 40%, and 10% of the patients, respectively. The occurrence of the high grade subtypes was 50% in both the adult and pediatric groups. The Ki-67/MIB-1 index increased across the three histological subtypes, from CPP to ACPP and then to CPC. Adjuvant therapy was administered in three patients. The two patients (one adult and one child) with CPC died of whole central nervous system dissemination. At a median of 62-month follow-up, the other eight patients were alive, with only one patient having recurrence and reoperation. The results demonstrate that gross total resection is usually curative for CPP and ACPP, and adjuvant chemoradiotherapy would be required for CPC and incompletely resected ACPP.

Role of available adjuvant therapies following surgical resection of atypical choroid plexus papilloma—a systematic review and pooled analysis

“…Eight of the studies included pediatric patients, 13–15 , 27 , 29 , 30 , 33 , 36 and only 2 studies totally consisted of the adult patient population. 28 , 32 Also, 4 studies had a mixed pediatric and adult population 26 , 31 , 34 , 35 with only one of them providing individual patient data for separate data extraction for the pediatric and adult populations 31 ( Table 1 ). To prevent readers from getting confused by these heterogeneous types of studies with different included populations, we developed a strategy to demonstrate the results of these populations separately within tables.…”

“…Among the 14 included studies, the overall chemotherapy regimen or radiotherapy protocol had been indicated in only 4 studies. 15 , 29 , 31 , 34 Even in these 4 studies, the significant details of the adjuvant treatment protocol were missing. The most used chemotherapeutic agents were Etoposide, Vincristine, Cisplatine, Cyclophosphamide, or Ifosfomide but other agents such as Carboplatine or Thalidomide were also mentioned.…”

“…Unfortunately, this detailed data were only available in 3 of the included studies, 1 of which included the pediatric patients and the 2 other studies consisted of mixed adult and pediatric patients. 15 , 31 , 34 …”

Choroid Plexus Carcinoma in Adults: Two Case Reports

“…For lateral and 3rd ventricle CPCs, the median age of presentation is 1.5 years, and supratentorial CPCs are extremely rare in adults. In fact, we could only retrieve 4 cases from the literature [891011], which are summarized in Table 1. The second case presented here was diagnosed at age 49, and the location of the tumor was more consistent with ‘extraventricular’ than lateral ventricle.…”

Cerebellopontine Angle Primary Choroid Plexus Carcinoma Present in an Adult: Case Report and Literature Review

“…In children, they frequently arise from the lateral ventricles and the fourth ventricle in adults. Rarely, they occur in the cerebellopontine angle (CPA), suprasellar region, or even in the sacral canal [3]. To the author's knowledge, we present the first adult case of CPC presenting in the CPA.…”