Radiological imaging of choroid plexus tumors: Systematic approach toward diagnosing a choroid plexus tumor

Choroid plexus tumors (CPTs) are rare intraventricular tumors comprising approximately 1% of all brain tumors. The common locations are the lateral ventricle (most common location in children), the third and fourth ventricles (most common location in adults), and cerebellopontine angle. Here, we present the case of a 10-month-old child with complaints of fever (99.6F), abnormal eye movements, and bilateral papilledema. Ultrasound cranium of the child revealed a well-defined hyperechoic lesion adjacent to the trigone and occipital horn of the right lateral ventricle. No vascularity could be appreciated on color Doppler. Non-contrast computed tomography of the brain showed a well-defined lobulated mass lesion epicentered at the choroid plexus of the right lateral ventricle which was hyperdense relative to the brain parenchyma with specks of calcification. Contrast-enhanced magnetic resonance imaging showed a solid intensely enhancing lobulated mass lesion with frond-like morphology originating from the choroid plexus of the occipital horn of the right lateral ventricle. Surgical excision of the CPT was done under aseptic conditions. We try to reinforce the ultrasound (USG), CT, and MRI findings of a CPT which ultimately came out to be a choroid plexus papilloma (CPP) on histopathological examination.

Persistence of communicating hydrocephalus post choroid plexus tumor resection: Case reports and review of literature

Background: Hydrocephalus is the most common presentation of choroid plexus tumors; it is thought to be caused either by mass effect obstructing the cerebrospinal fluid pathways or secretory properties of the tumor. In these case reports, we present two cases of choroid plexus tumors with persistence of communicating hydrocephalus postoperatively and review similar reports in the literature. Case Description: Case 1: a 2-month-old baby girl presented with bulging fontanelle, sunsetting eyes. Magnetic resonance imaging (MRI) showed large third ventricle mass with communicating hydrocephalus. She underwent complete excision of tumor through transcortical approach with perioperative intraventricular hemorrhage. Hydrocephalus persisted postoperatively and the patient required permanent ventriculoperitoneal (VP) shunt. Case 2: a 16-year-old boy presented decreased visual acuity, papilledema, and morning headaches. MRI showed a tumor in the right ventricle and communicating hydrocephalus. He underwent transparietal resection of the tumor. In both cases, hydrocephalus persisted postoperatively and patients required permanent VP shunt. Review of similar cases showed the majority of cases required permanent shunting. Conclusion: Choroid plexus tumor patients can present with communicating hydrocephalus that may persist post tumor resection for different etiologies. Careful follow-up to determine the need for cerebrospinal fluid diversion through a permanent VP shunt is important.

CHOROID PLEXUS TUMORS IN CHILDREN: ANALYSIS OF CLINICAL DATA

Choroid plexus tumors (CPT) are rare neoplasms accounting for 2–5% of all brain tumours in children, and are most commonly under 2 years of age. Most of these tumors present with symptoms of intracranial hypertension. Survival directly depends on the histological variant of the CPT. Objective of the study: to analyze the current and outcome characteristics of patients with CPT depending on the morphological variant. This study is a multicenter, retrospective, open, uncontrolled, non-comparative, non-randomized, longitudinal study. Materials and methods of research: 152 children with CPT according to the WHO classification of CNS tumors (2007, 2016), aged 0 to 18 years, who received treatment and observed from 2009 to 2019 were included in this study. Results: 83 (54,6%) of 152 children with CPT had choroid plexus papillomas (CPPs), 37 (24,3%) – atypical choroid plexus papillomas (APPs), 32 (21,1%) – choroid plexus carcinomas (CPCs). The median age for CPP was 21 months, for APP – 6 months, for CPC – 30 months. CPTs occurred at the age of less than 24 months in 53,3% of cases. In 70,4% of cases CPT were localized in the lateral ventricles. In most children disease manifested by intracranial hypertension, and this symptomatology in children with CPC was statistically significantly more frequent than in children with CPP (p=0,0042). In 28,9% of patients with CPP, 24,3% with APP and 9,4% with CPC the disease was asymptomatic, and all these patients were diagnosed during routine preventive examination. Five-year event-free survival (EFS) and overall survival (OS) for CPP, APP and CPC were 96%±2% and 99%±1%, 81%±7% and 97%±3%, 44%±10% and 66%±10%, respectively (p<0,0001). Conclusion: for the first time in the national literature the results of a retrospective analysis of clinical characteristics and survival of children suffering from a rare group of diseases such as CPT are collected and presented.

Preoperative Devascularization of Choroid Plexus Tumors: Specific Issues about Anatomy and Embolization Technique

(1) Background: Surgical treatment of choroid plexus tumors is challenging, burdened by a notable risk of bleeding. Neoadjuvant chemotherapy and preoperative embolization have been attempted, with encouraging results; however, the consensus on these procedures is lacking. (2) Methods: We present a case of a 10-month-old girl who underwent preoperative embolization of a hemorrhagic choroid plexus carcinoma of the lateral ventricle via the anterior choroidal artery, followed by total resection. (3) Results: The endovascular procedure was successfully completed, despite the rectification of the anterior choroidal artery associated with the absence of flow proximal to the plexal point. Minimal bleeding was observed during resection and the patient remained neurologically intact. (4) Conclusions: The time from entrance to exit in the anterior choroidal artery should be monitored and regarded as a potential ‘occlusion time’ in this specific group of patients. Nevertheless, our case supports the feasibility and effectiveness of preoperative embolization of a choroid plexus carcinoma of the lateral ventricle via the anterior choroidal artery, without complications. Furthermore, we suggest the use of a fast-embolic agent, such as N-butyl cyanoacrylate glue, as the preferred agent for this specific pathology and patient population.

Aggresomes predict poor outcomes and implicate proteostasis in the pathogenesis of pediatric choroid plexus tumors

Purpose Protein misfolding and aggregation result in proteotoxic stress and underlie the pathogenesis of many diseases. To overcome proteotoxicity, cells compartmentalize misfolded and aggregated proteins in different inclusion bodies. The aggresome is a paranuclear inclusion body that functions as a storage compartment for misfolded proteins. Choroid plexus tumors (CPTs) are rare neoplasms comprised of three pathological subgroups. The underlying mechanisms of their pathogenesis remain unclear. This study aims to elucidate the prognostic role and the biological effects of aggresomes in pediatric CPTs. Methods We examined the presence of aggresomes in 42 patient-derived tumor tissues by immunohistochemistry and we identified their impact on patients’ outcomes. We then investigated the proteogenomics signature associated with aggresomes using whole-genome DNA methylation and proteomic analysis to define their role in the pathogenesis of pediatric CPTs. Results Aggresomes were detected in 64.2% of samples and were distributed among different pathological and molecular subgroups. The presence of aggresomes with different percentages was correlated with patients’ outcomes. The ≥ 25% cutoff had the most significant impact on overall and event-free survival (p-value < 0.001) compared to the pathological and the molecular stratifications. Conclusions These results support the role of aggresome as a novel prognostic molecular marker for pediatric CPTs that was comparable to the molecular classification in segregating samples into two distinct subgroups, and to the pathological stratification in the prediction of patients’ outcomes. Moreover, the proteogenomic signature of CPTs displayed altered protein homeostasis, manifested by enrichment in processes related to protein quality control.

Stereotactic Radiosurgery for Choroid Plexus Tumors: A Report of the International Radiosurgery Research Foundation

BACKGROUND Choroid plexus tumors (CPT) are rare epithelial tumors of the choroid plexus. Gross total resection (GTR) may be curative, but it is not always possible. OBJECTIVE To evaluate the role of Gamma Knife stereotactic radiosurgery (GKSRS) as either a primary or adjuvant management option for WHO grade I-III CPT through a multicenter project. METHODS A total of 32 patients (20 females) with a total of 43 treated tumors were included in the analysis. A total of 25 patients (78%) had undergone initial surgical resection. The median total tumor volume was 2.2 cc, and the median margin and maximum doses were 13 and 25.5 Gy, respectively. RESULTS Local tumor control was achieved in 69% of cases. Local tumor progression-free survival (PFS) rate for low-grade tumors at 1, 3, and 5 yr was 90%, 77%, 58%, respectively. The actuarial local tumor PFS rate for high-grade tumors at 1, 3, and 5 yr was 77%, 62%, and 62%, respectively. There was no significant difference in local tumor control rates between low- and high-grade CPT (P = .3). Gender, age, and degree of resection were not associated with treated tumor PFS. Distant intracranial spread developed in 6 patients at a median of 22 mo after initial SRS. Actuarial distant brain tumor PFS rate at 1, 2, 5, and 10 yr was 93%, 88%, 78%, and 65%, respectively. Three patients (9%) developed persistent symptomatic adverse radiation effects at a median of 11 mo after the procedure. CONCLUSION GKSRS represents a minimally invasive alternative management strategy for imaging defined or surgically recurrent low- and high-grade CPT.

RARE-26. RETROSPECTIVE ANALYSIS OF PEDIATRIC CHOROID PLEXUS TUMORS

BACKGROUND Choroid plexus tumors (CPT) include choroid plexus papilloma (CPP), atypical choroid plexus papilloma (aCPP), and choroid plexus carcinoma (CPC). Because of their rarity, limited data are available on the current status of treatment and outcomes for pediatric CPTs. METHOD We retrospectively reviewed clinical information on patients with CPT patients aged between 0 and 30 years at diagnosis and were treated in 8 institutions in Japan. RESULTS Of forty-two cases initially diagnosed as CPT, 18 cases were reviewed by central pathologists. As a result, the diagnosis of CPC or aCPP in five cases were changed to other tumors including AT/RT and astroblastoma. The remaining 37 cases were subjected to analysis. Median age at diagnosis was two years (0 to 25) and the mean follow-up period was seven years. All 26 patients with CPP (n=20) or aCPP (n=6) underwent gross-total resection without adjuvant therapy. Of them 24 patients are alive without recurrence. Four patients of patients with CPC (n=11) died of cancer. Five patients including three patients experienced local relapse, achieved complete remission after resection of tumor plus chemoradiotherapy. All three patients with dissemination of CPC at diagnosis or relapse died of the disease. At least three patients were diagnosed with Li-Fraumeni syndrome: one died of medulloblastoma and one patient developed osteosarcoma. CONCLUSION Compared with the excellent prognosis of CPP, the survival rates for CPC, especially disseminated CPC are unsatisfactory. Our results also underline the importance of considering genetic testing of TP53 for patients with CPC.