Final results of the Choroid Plexus Tumor study CPT-SIOP-2000

Introduction Standards for chemotherapy against choroid plexus tumors (CPT) have not yet been established. Methods CPT-SIOP-2000 (NCT00500890) was an international registry for all CPT nesting a chemotherapy randomization for high-risk CPT with Carboplatin/Etoposide/Vincristine (CarbEV) versus Cyclophosphamide/Etoposide/Vincristine (CycEV). Patients older than three years were recommended to receive irradiation: focal fields for non-metastatic CPC, incompletely resected atypical choroid plexus papilloma (APP) or metastatic choroid plexus papilloma (CPP); craniospinal fields for metastatic CPC/APP and non-responsive CPC. High risk was defined as choroid plexus carcinoma (CPC), incompletely resected APP, and all metastatic CPT. From 2000 until 2010, 158 CPT patients from 23 countries were enrolled. Results For randomized CPC, the 5/10 year progression free survival (PFS) of patients on CarbEV (n = 20) were 62%/47%, respectively, compared to 27%/18%, on CycEV (n = 15), (intention-to-treat, HR 2.6, p = 0.032). Within the registry, histological grading was the most influential prognostic factor: for CPP (n = 55) the 5/10 year overall survival (OS) and the event free survival (EFS) probabilities were 100%/97% and 92%/92%, respectively; for APP (n = 49) 96%/96% and 76%/76%, respectively; and for CPC (n = 54) 65%/51% and 41%/39%, respectively. Without irradiation, 12 out of 33 patients with CPC younger than three years were alive for a median of 8.52 years. Extent of surgery and metastases were not independent prognosticators. Conclusions Chemotherapy for Choroid Plexus Carcinoma is feasible and effective. CarbEV is superior to CycEV. A subset of CPC can be cured without irradiation.

Management of Choroid Plexus Papillomas

Introduction: Choroid plexus papillomas are rare neuroepithelial tumors found primarily in children. It represents less than 1% of all central nervous system tumors. Materials and methods: A retrospective study including 14 patients with choroid plexus papilloma tumors were performed at the Neurosurgery Department in Ait IDDIR Health Hospital Establishment between January 2010 and December 2017. In each case, diagnosis was made clinically and confirmed radiologically and histo-pathologically. All patients were operated. Results and discussion: The mean age was 26 years (ranged 3 months –48 years) .In our department, we grouped together 14 cases of choroid plexus papilloma tumors. For mortality we had one case who died during surgery, survival rate for 04 years is 100% .We had not recurrence during the study period.All patients had intracranial hypertension (HIC) without neurological deficit and benefited from brain CT, MRI and an Angiography. The location of the tumor was: Lateral ventricle, Fourth ventricle, Third ventricle. All patients underwent surgical excision with or without ventriculo-peritoneal shunt. Conclusion: Choroid plexus papillomas are rare neuroepithelial tumors, typically considered benign lesions, derived from the choroid plexus and appear like cauliflower.

Rare Brain Tumors with Infrequent Clinical Manifestations: Illustrative Cases

This chapter describes the epidemiology, clinical and neuroimaging features, histological characteristics, surgical approach, outcomes, and prognostic factors of different cases of very rare intracranial tumors, associated with complex clinical syndromes. Highlighting the important aspects in the diagnosis and management that were considered relevant through the experience of our center. Here we included an intracranial Rosai-Dorfman disease manifested as an apparent multiple meningiomatosis, a choroid plexus papilloma clinically manifested as a hemifacial spasm originated by a compression of the facial colliculus, and a neuroenteric cyst associated with Klippel-Feil syndrome. This type of tumor presents a challenge to the neurosurgeon, originating various questions about its management. In this chapter, we present the experience we had with these pathologies to establish the most appropriate management decisions.

Choroid plexus papilloma and atypical choroid plexus papilloma in children: treatment results

Choroid plexus tumors (CPTs) are rare intraventricular tumors, accounting 2–5% of brain tumors in children. Choroid plexus papilloma (CPP) classified by the World Health Organization (WHO) on the basis of histological criteria as benign tumor (WHO grade I), CPPs accounts for 10–20% of brain tumors during the first year of life, the median age of patients with CPP at the time of diagnosis – 3.5 years. Atypical choroid plexus papilloma (APP) is an intermediate (WHO grade II) tumor which was officially included in the WHO classification in 2007. Median age 0,63 years. In this work, for the first time in the Russian literature, we present the outcomes of CPP and APP therapy in a large cohort of patients. The study was approved by the Independent Ethics Committee and the Scientific Council of the D. Rogachev NMRCPHOI. In our study from January 1, 2009 to December 31, 2019, 83 CPP and 37 APP were histologically verified. For all patients, surgical removal of the tumor was the first stage of treatment. In 79 (95.2%) patients with CPP and in 32 (86.5%) children with APP, radical resection was performed at the first stage. In 73.5% of children with CPP and 62.2% of children with APP there was no intra- and postoperative complications. In both CPP and APP, the 5-year EFS in patients with complete resection was statistically significantly higher compared to subtotal tumor resection (100% and 33 ± 27% in CPP, 94 ± 4% and 25 ± 22% for APP) (p < 0.001). Chemoradiation therapy was given to 1 patient with CPP, 4 patients with APP with tumor recurrence/progression.

Factor XI deficiency and delayed hemorrhages after resection of choroid plexus papilloma: illustrative case

BACKGROUND Factor XI deficiency, also known as hemophilia C, is a rare inherited bleeding disorder that may leave routine coagulation parameters within normal range. Depending on the mutation subtype, prolonged activated partial thromboplastin time may occasionally be found. The disease has an autosomal transmission, with an estimated prevalence in the general population of approximately 1 in 1 million. Heterozygosis accounts for partial deficits, but the tendency to bleed is unrelated to the measured activity of factor XI. Diagnosis usually follows unexpected hemorrhages occurring spontaneously or after trauma or surgical procedures. OBSERVATIONS Few cases have been reported in the neurosurgical literature, all occurring spontaneously or after head trauma. Owing to its subtle features, the true incidence of the disease is probably underestimated. The authors report a case of a patient with previously undiagnosed factor XI deficiency who underwent uncomplicated resection of a fourth-ventricle papilloma and experienced delayed, severe hemorrhagic complications. LESSONS The known association between choroid plexus tumors and intracranial bleeding raised differential diagnosis issues. This report may serve to help to investigate delayed hemorrhages after cranial surgery.

Ureaplasma parvum meningitis following atypical choroid plexus papilloma resection in an adult patient: a case report and literature review

Background While Ureaplasma parvum has previously been linked to the incidence of chorioamnionitis, abortion, premature birth, and perinatal complications, there have only been rare reports of invasive infections of the central nervous system (CNS) in adults. Owing to its atypical presentation and the fact that it will yield sterile cultures using conventional techniques, diagnosing U. parvum meningitis can be challenging. Case presentation We describe a case of U. parvum meningitis detected in an adult patient following surgical brain tumor ablation. After operation, the patient experienced epilepsy, meningeal irritation, and fever with unconsciousness. Cerebrospinal fluid (CSF) analysis showed leukocytosis (484 * 106 /L), elevated protein levels (1.92 g/L), and decreased glucose concentrations (0.02 mmol/L). Evidence suggested that the patient was suffering from bacterial meningitis. However, no bacterial pathogens in either CSF or blood were detected by routine culture or serology. The symptoms did not improve with empirical antibiotics. Therefore, we performed metagenomic next-generation sequencing (mNGS) to identify the etiology of the meningitis. Ureaplasma parvum was detected by mNGS in CSF samples. To the best of our knowledge, this case is the first reported instance of U. parvum meningitis in an adult patient in Asian. After diagnosis, the patient underwent successful moxifloxacin treatment and recovered without complications. Conclusions As mNGS strategies can enable the simultaneous detection of a diverse array of microbes in a single analysis, they may represent a valuable means of diagnosing the pathogens responsible for CNS infections and other clinical conditions with atypical presentations.

An unexpected sudden death due to a choroid plexus papilloma: an autopsy case report

Background Choroid plexus papillomas (CPPs) are histopathologically benign and rare central nervous system tumors. These tumors remain more frequent in children than adults. It is infrequent for these tumors to cause a sudden unexpected death. We aim in this case to discuss the unusual and fatal presentation of choroid plexus papilloma and the mechanism of death. Case presentation we report the case of a 61-year-old man with no medical history, diagnosed at autopsy with a previously unknown CPP. Initial complaints were chronic headache occurring in the last month and acute chest pain for two days. The forensic autopsy including the histopathologic examination showed a tumoral mass of the choroid plexus in the fourth ventricle diagnosed as a psammomatous CPP. The cause of death in this case was attributed to a massive cerebral edema caused by the tumoral mass. Conclusions Through this case report, we stress the importance of an early and a vigorous investigation of every headache and an early detection of this tumor and we highlight as well the role of the post mortem examination to detect such a fatal complication.

Choroid plexus papilloma in a free ranging eared dove (Zenaida auriculata)

Choroid plexus tumors (CPT) are rare neoplasms and histologically classified as choroid plexus papilloma (CPP), atypical choroid plexus papilloma (APP), and choroid plexus carcinoma (CPC). These neoplasms have been described in humans, domestic animals (canine, feline, equine, caprine) and some wild animals (cetaceans and Psittacidae birds). To our best knowledge, herein we report the first CCP in a free-ranging eared dove (Columbiformes; Zenaida auriculata, de Murs 1847). Histologically, the ventricle was dilated, with a papillary proliferation (arboriform pattern) in topography of CP. The neoplasm was well-differentiated, composed by a single layer of cuboidal cells, anchored in a delicate fibrovascular stroma. The neoplastic cells exhibited moderate stroma, with well-defined borders and round nuclei, with vesicular chromatin and inconspicuous nucleoli. Mitotic activity was low (<1 mitosis per 10 high-power fields). Immunohistochemistry for cytokeratin markers (AE1/AE3 antibody) were implemented, however, both neoplastic cells and normal epithelial tissues do not show immunoreactivity.

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.