Extracerebral choroid plexus papilloma in a newborn: a rare presentation.

Choroid plexus papillomas (CPPs) are extremely rare lesions that originate in the central nervous system. Still rarely, these can occur in heterotopic locations. We report a case of aneonate who presented at fourth week of life with a small swelling in the left side of the oropharynx. There were no other symptoms. MRI scan revealed a cystic lesion with the possibility of lymphatic malformation or teratoma. Swelling which was gradually increasing in size was electively excised. Histopathological examination revealed it to be an extracerebral CPP. Postoperative period was uneventful, and baby had no further complications. CPP is a rare lesion and, almost always, cannot be anticipated before surgery. Imaging only helps in surgical planning. Complete excision is curative.

Rare Case of Hemorrhagic Atypical Choroid Plexus Papilloma of Fourth Ventricle in Adult: A Diagnostic Dilemma.

Choroid plexus papillomas are highly vascular tumors, and such tumors causing subarachnoid hemorrhage have been reported in literature. Similarly, few articles have reported atypical fourth ventricular choroid plexus tumors in adults. However, such an atypical tumor presenting with grossly hemorrhagic transformation without any acute symptoms could not be found in the literature.

Pure endoscopic ultrasonic removal of choroid plexus papillomas of the third ventricle: technical report of two cases.

BACKGROUND: Tumors of the choroid plexus of the third ventricle are uncommon. Surgical excision is technically challenging because of the rich vascularisation, central location, and high incidence in young children. Open microsurgical resection is considered the standard treatment. However, attempts at purely endoscopic removal of choroid plexus tumors of the third ventricle have also been made in the past, with encouraging results. CASE REPORTS: We report our experience with endoscopic ultrasonic removal of two cases of tumors of the third ventricular choroid plexus. The first case was a large atypical choroid plexus papilloma (WHO grade 2) in the anterior third ventricle associated with hydrocephalus; the second case was a smaller choroid plexus papilloma (WHO grade 1) in the middle/posterior third ventricle without overt hydrocephalus requiring a more anterior neuronavigation guided approach. DISCUSSION AND CONCLUSION: Choroid plexus papillomas of the third ventricle can be safely treated by a purely endoscopic approach because they are usually smaller than their counterparts in the lateral ventricle and often have a recognizable vascular pedicle. Early detection and control of the vascular pedicle at the choroidal border is key to success. The use of ultrasonic aspirator facilitates and expedites endoscopic access. By alternating surface coagulation with fragmentation and aspiration with the ultrasonic aspirator, the tumor can be removed without difficult dissection maneuvers.

How I do it? The surgical resection of the fourth ventricle choroid plexus papilloma.

BACKGROUND: The surgical resection of the large fourth ventricle choroid plexus papilloma (CPP) is complicated, where the challenge is to minimize the impairment of the vermis and the brainstem and restore the cerebrospinal fluid circulation. METHOD: We report a case of large CPP that wholly occluded the fourth ventricle, extended to the Luschka foramen, and underwent radical resection via telovelar approach. The intraoperative endoscope was applied to inspect the tumor residue and the aqueduct's opening. CONCLUSION: This case demonstrates the surgical nuance of the fourth ventricle CPP.

Rare Case of Pediatric Disseminated Choroid Plexus Papilloma: Literature Review and Call for Reclassification.

INTRODUCTION: Choroid plexus tumors are rare neuroectodermal tumors that arise from the choroid plexus. Choroid plexus papillomas (CPPs) represent the lowest grade of these types of tumors and have a WHO grade I designation. Despite their typical low grade, some CPPs can exhibit aggressive behaviors including parenchymal invasion and dissemination throughout the neuro-axis. Due to their association with the choroid plexus, patients with CPP commonly present with signs and symptoms of hydrocephalus and increased intracranial pressure. CASE PRESENTATION: A 2-year-old male presented in extremis with acute hydrocephalus and seizure. He was found to have a large left intraventricular mass with innumerable intraparenchymal and extra-axial cysts throughout his neuro-axis. A literature review revealed five similar disseminated CPP cases with innumerable lesions. This is the youngest reported patient with disseminated CPP and the first with multiple compressive lesions. Following cranial resection and thoracic decompression, the patient's lesions have remained stable (2 years of follow-up). A literature search of the PubMed/Medline databases was performed using the search terms ["disseminated choroid plexus papilloma" OR "choroid plexus papilloma" OR "metastatic choroid plexus papilloma"] up to March 2021. Articles were then screened for similar patient radiographic presentation and histological diagnosis. To mitigate publication bias, referenced articles were utilized to identify other case reports and case series. DISCUSSION/CONCLUSION: We describe a rare case of a lateral ventricle CPP with widespread leptomeningeal dissemination causing acute obstructive hydrocephalus and compressive myelopathy requiring cerebrospinal fluid diversion and intracranial resection followed by thoracic spine decompression. This case report serves to broaden knowledge of disseminated CPP and to encourage complete neuro-axis imaging for choroid plexus tumors. Additionally, we propose a naming paradigm refinement that includes radiographic characteristics.

Infantile Choroid Plexus Papilloma with Multiple Peritumoral Cysts.

Infantile choroid plexus papilloma (CPP) associated with multiple peritumoral cysts is a rare variant of CPP, and clinical course and optimal management are largely unknown. A 9-month-old boy presented with a large solid tumor in the left lateral ventricle associated with multiple peritumoral cysts, arachnoid cysts, and hydrocephalus containing xanthochromic fluid with high protein content. Shrinkage of these cysts and resolution of hydrocephalus were achieved after total resection of the hypervascular solid part of the tumor. Histological examination confirmed the solid part of the tumor as CPP and showed that the wall of the peritumoral cysts consisted of reactive gliosis without neoplastic cells. Follow-up magnetic resonance imaging 12 months after surgery revealed that these cysts remained stable. CPP with nonenhancing peritumoral cysts can be managed by resection of only the solid part of the tumor without permanent cerebrospinal fluid diversion.

Isolated Lumbar Atypical Choroid Plexus Papilloma: A Case Report.

AIM: To report the first case of an isolated lumbar grade II atypical choroid plexus papilloma (CPP). CASE REPORT: A 42-year-old man was admitted to the hospital because of back and leg pain. No urinary or rectal dysfunction was detected. Lumbar magnetic resonance imaging (MRI) showed a well-circumscribed, contrast-enhancing, intradural extramedullary mass at L2-3. He underwent L2 and L3 partial laminectomies for tumor resection and complete resection was achieved without causing neurological deficit. Histopathologic examination of the tumor resulted in a diagnosis of grade II atypical CPP. The Ki-67 staining index was 7%. No lesion was detected on postoperative craniospinal MRI. CONCLUSION: Isolated lumbar atypical CPP in the lumbar region has not been previously reported. In the presence of a single spinal lesion, the diagnosis of CPP should be considered. Unlike metastatic and synchronous tumors, the pathogenesis of isolated choroid plexus tumors within the spinal canal has not been explained.

Paediatric atypical choroid plexus papilloma: is adjuvant therapy necessary?

INTRODUCTION: Choroid Plexus Tumours (CPTs) account for 1-4% of all brain tumours in children. Atypical choroid plexus papillomas (aCPPs) are a subset of these tumours, defined over a decade ago, yet no consensus exists on the optimal approach to their management. METHODS: We conducted a retrospective analysis of all patients treated for CPTs at the Hospital for Sick Children between January 1, 2000, and December 31, 2018, and focused on patients with aCPP. Data extracted from the patient records for analysis included: demographic and clinical features, radiological imaging, surgical and adjuvant therapies, key pathological features, immunohistochemical staining for TP53 and tumour karyotype. Six of seven aCPP samples were profiled using Illumina HumanMethylationEPIC arrays and the top 10,000 most variably methylated probes were visualized using tSNE. Copy number inferencing was also performed. RESULTS: Twenty-nine patients were diagnosed with CPT, seven of whom had a diagnosis of aCPP as confirmed by histological review. Methylation profiling demonstrated that aCPPs clustered with both choroid plexus papillomas (CPPs) and choroid plexus carcinomas (CPCs). Complete resection of the tumour was pursued in all cases of aCPP and no patient received adjuvant therapy. All aCPP patients were alive at last follow up. CONCLUSIONS: This limited case series suggests that paediatric aCPP can be successfully managed with surgical resection alone, followed by a 'watch and wait' approach thus avoiding adjuvant therapies. A deeper understanding of the biology of aCPP is required to identify objective markers which can help provide robust risk stratification and inform treatment strategies.

Cerebello-pontine and Cerebello-medullary Fissure Choroid Plexus Papilloma in a Child - Case Report and Review of Literature.

Choroid plexus papillomas (CPP) are commonly seen in the supratentorial compartment in children and only very rarely in the posterior fossa. CPP in the cerebello-pontine angle and cerebello-medullary fissure (CPA) in the pediatric age group are extremely rare with only seven previous cases reported in literature. The authors present the case of a 7-year-old girl who presented with neck tilt, imbalance, and headache. The MRI showed a lesion with a frond-like appearance in the CPA with heterogeneous enhancement. The tumor was excised radically using a cerebello-medullary fissure approach. The authors review and analyze the literature on this rare pediatric tumor, with specific attention to radiology and the surgical aspects.

Molecular insights into malignant progression of atypical choroid plexus papilloma.

Choroid plexus tumors are rare pediatric neoplasms ranging from low-grade papillomas to overtly malignant carcinomas. They are commonly associated with Li-Fraumeni syndrome and germline TP53 mutations. Choroid plexus carcinomas associated with Li-Fraumeni syndrome are less responsive to chemotherapy, and there is a need to avoid radiation therapy leading to poorer outcomes and survival. Malignant progression from choroid plexus papillomas to carcinomas is exceedingly rare with only a handful of cases reported, and the molecular mechanisms of this progression remain elusive. We report a case of malignant transformation of choroid plexus papilloma to carcinoma in a 7-yr-old male with a germline TP53 mutation in which we present an analysis of molecular changes that might have led to the progression based on the next-generation genetic sequencing of both the original choroid plexus papilloma and the subsequent choroid plexus carcinoma. Chromosomal aneuploidy was significant in both lesions with mostly gains present in the papilloma and additional significant losses in the carcinoma. The chromosomal loss that occurred, in particular loss of Chromosome 13, resulted in the losses of two critical tumor suppressor genes, RB1 and BRCA2, which might play a possible role in the observed malignant transformation.

Pediatric choroid plexus papilloma arising from the cerebellopontine angle: systematic review with illustrative case.

INTRODUCTION: Choroid plexus tumors are uncommon intraventricular tumors that develop from the choroid plexus of the central nervous system. Choroid plexus papillomas arising from the cerebellopontine angle have been reported to almost exclusively occur in adults and are rarely found in children. METHODS: We report a systematic review conducted in accordance with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines of SCOPUS and PubMed databases for case reports and case series of choroid plexus papillomas arising in the cerebellopontine angle in the pediatric population and discuss clinical presentation, imaging features, management options, and outcomes. We also report a case managed at our center. RESULTS: Ten cases of pediatric choroid plexus papillomas arising in the cerebellopontine angle were identified from the systematic review in addition to the case reported here, resulting in a total of eleven cases. The patients' median age was 8 years with a slight female sex predilection (1.2:1). Patients most commonly presented with headache, cerebellar signs, and cranial nerve palsies with median duration of symptoms at 4 months. All patients underwent surgical treatment with majority achieving gross total excision. No deaths were reported at median follow-up of 12 months. Complete neurologic recovery was attained in seven cases while partial recovery was seen in two cases. CONCLUSION: Choroid plexus papillomas found in the cerebellopontine angle in the pediatric population are extremely rare but they should be considered in the differential diagnosis. Complete surgical resection is the mainstay of treatment with excellent outcomes achievable in majority of patients.

Extracerebral choroid plexus papilloma in the pharynx with airway obstruction in a newborn: a case report.

BACKGROUND: Choroid plexus papillomas (CPPs) are rare, usually benign, neoplasms originating in the central nervous system. In this study, we present the first case of a giant airway-obstructing CPP in the pharynx of a newborn. CASE PRESENTATION: A cystic mass located in the pharynx was noted in a fetus at the 29th week of gestation. Elective cesarean section was performed at the 38th week of gestation with successful intubation and ex utero intrapartum treatment. On computed tomography, there was a huge airway-obstructing cystic mass in the choana and pharynx. Elective surgery with total excision was performed, and histological examination confirmed the diagnosis of CPP. CONCLUSION: We report the first case of an extracerebral airway-obstructing CPP in the pharynx of a newborn. Radiologic examinations are not enough for the diagnosis of CPPs, and complete excision of the tumor with histological confirmation is indispensable for accurate diagnosis and treatment.

Papiloma del plexo coroideo del tercer ventrículo. Descripción de un caso / Choroid plexus papilloma of the third ventricle. A case report

El papiloma del plexo coroideo es un tumor poco frecuente del sistema nervioso central, que representa menos del 1% de todas las neoplasias intracraneales. Las ubicaciones habituales son el ventrículo lateral en bebés y niños y el cuarto ventrículo en adultos. El tercer ventrículo es una localización inhabitual, con pocos casos recogidos en la bibliografía. Describimos el caso de un niño de 3 meses que ingresó en nuestro centro con signos de aumento de la presión intracraneal. Los estudios de neuroimagen mostraron una lesión en el tercer ventrículo, con hidrocefalia asociada. Al paciente se le extirpó completamente el tumor mediante abordaje transfrontal y cirugía de derivación ventriculoperitoneal. El curso postoperatorio del niño transcurrió sin incidentes y la imagen de resonancia magnética de seguimiento no reveló tumor residual. La histopatología de la lesión resecada confirmó el diagnóstico de papiloma del plexo coroideo. Discutimos las características clínicas, radiológicas e histológicas de este tipo infrecuente de tumores

Choroid plexus papilloma is an uncommon tumour of the central nervous system, accounting for less than 1% of all intracranial neoplasm. The usual locations are the lateral ventricle in infants and children and the fourth ventricle in adults. The third ventricle is a rare location, with few cases reported in the literature. We describe the case of a 3-month-old boy who was admitted to our centre with signs of raised intracranial pressure. Neuroimaging studies showed a third ventricular mass with associated hydrocephalus. The patient underwent complete tumour removal through a transfrontal approach and ventriculo-peritoneal shunt surgery. Postoperative course of the child was uneventful and follow-up magnetic resonance imaging revealed no residual tumour. Histopathology of the resected lesion confirmed the diagnosis of choroid plexus papilloma. We discuss the clinical, radiological and histological features of this infrequent type of tumours

Hemifacial Spasm Associated with Compression of the Facial Colliculus by a Choroid Plexus Papilloma of the Fourth Ventricle.

BACKGROUND: Hemifacial spasm is an involuntary condition that involves muscles innervated by the ipsilateral facial nerve. There are secondary causes of hemifacial spasm that can produce a typical presentation of symptoms. Extrinsic compression of the facial colliculus at the floor of the fourth ventricle is responsible for <0.6% of the causes of secondary hemifacial spasm, as the cases with this origin reported in the literature are rare. CASE REPORTS: We present the case of a 43-year-old female with hemifacial spasm of typical characteristics 6 months after onset. Upon clinical examination, a severe contraction of the orbicularis oculi, orbicularis oris, and superficial muscles of the neck displaying 50 crisis per hour was revealed. Brain magnetic resonance imaging showed absence of the facial nerve vascular loop in the cisternal portion, with evidence of an intraventricular tumor in relation with the medial portion of the fourth ventricle at the facial colliculus level, indicating a secondary origin of hemifacial spasm. Preoperative electromyography demonstrated irritative electric activity in the muscular branches of the facial nerve. A telovelar approach was performed to the fourth ventricle with intraoperative electrophysiology monitoring, with immediate resolution of the irritative activity after complete tumor resection. The result of the histopathologic study was a choroid plexus papilloma. CONCLUSION: Fourth ventricle tumors with extrinsic compression of the facial colliculus represent <0.6% of the causes of hemifacial spasm. Its relationship with choroid plexuses papilloma is being described as the first case reported in the literature. Clinical correlation, imaging, and intraoperative findings in conjunction with intraoperative electrophysiology recordings allow to predict the resolution of symptoms after resecting the lesion.

Infratentorial choroid plexus tumors in children.

OBJECTIVE: Choroid plexus tumors (CPTs) are rare pediatric intracranial neoplasms, and mostly occur in the lateral ventricle. CPTs located in the infratentorial location are considered to be rare in the pediatric population. We present a series of eight patients treated in the last decade at our institution focusing on clinical presentations and their outcome after excision. METHODS: We performed an institutional retrospective review of patients who underwent surgical resection of infratentorial CPTs during the period from 2008 to 2017. Patients' charts were reviewed for demographic data, clinical presentation, surgical treatment, and follow-up. RESULTS: There were eight patients (6 females and 2 males), with mean age for the cohort at presentation was 9.0 years. They represent 75% of 12 CPTs of all locations treated at the same period in our institution. These 8 infratentorial CPTs were in the fourth ventricle in seven, and in the cerebellopontine angle (CPA) in one. Seven patients had choroid plexus papillomas (WHO grade I) and 1 had an atypical choroid plexus papilloma (WHO grade II). Gross total resection was attempted in all patients. However, two of 3 patients with fourth ventricle floor invasion had subtotal resection with a thin layer of tumor left on the floor. The remaining 6 had a gross total resection. Six patients with preoperative hydrocephalus had a perioperative external ventricular drainage but none required permanent shunting after tumor resection. None showed recurrence/tumor progression without adjuvant therapy during the follow-up period of 20 months to 11 years. CONCLUSION: Infratentorial dominance among pediatric CPTs in this series contradicts previous reports. Infratentorial CPTs are amenable to surgical resection. Unresected small residuals due to invasion to the fourth ventricle floor showed no regrowth during 2 to 3 years follow-up without adjuvant therapy. However, these patients with incomplete resection need watchful observations.

Choroid plexus papilloma of the third ventricle. A case report. / Papiloma del plexo coroideo del tercer ventrículo. Descripción de un caso.

Choroid plexus papilloma is an uncommon tumour of the central nervous system, accounting for less than 1% of all intracranial neoplasm. The usual locations are the lateral ventricle in infants and children and the fourth ventricle in adults. The third ventricle is a rare location, with few cases reported in the literature. We describe the case of a 3-month-old boy who was admitted to our centre with signs of raised intracranial pressure. Neuroimaging studies showed a third ventricular mass with associated hydrocephalus. The patient underwent complete tumour removal through a transfrontal approach and ventriculo-peritoneal shunt surgery. Postoperative course of the child was uneventful and follow-up magnetic resonance imaging revealed no residual tumour. Histopathology of the resected lesion confirmed the diagnosis of choroid plexus papilloma. We discuss the clinical, radiological and histological features of this infrequent type of tumours.