Management of Choroid Plexus Tumors and the Benefit of Preoperative Embolization in Pediatric Patients: Report of 46 Cases from a Single Institution.

OBJECTIVE: Optimal choroid plexus tumor (CPT) treatment involves gross total resection; however, intraoperative hemorrhage risk remains significant given tumor vascularity. This study describes pediatric CPT management and identifies patients most likely to benefit from preoperative embolization. METHODS: CPTs resected from 1997 to 2021 were included. The characteristics of embolized patients were compared to nonembolized patients; nonembolized patients were further stratified based on open vascular control-pedicle feeder ligation versus no pedicle ligation prior to tumor debulking. Statistical analyses identified factors associated with estimated blood loss (EBL), transfusion, length of stay, and complications. RESULTS: Among the 46 CPT cases identified, 98% achieved gross total resection, and 15% received embolization. Embolized patients were younger, smaller, and had larger tumors compared to nonembolized patients (median: 0.8 vs. 2.1 years; 9.3 vs. 14.4 kg; 91.08 vs. 5.5 cm3). Transfused patients were similarly younger and smaller (P < 0.05) than nontransfused patients. Among nonembolized patients, open vascular control was achieved in smaller tumors (<13 cm3) with significantly lower EBL (P = 0.002). Higher EBL was observed in patients with larger tumors, hydrocephalus, transependymal edema, vomiting, lethargy, and developmental regression (all P < 0.05). Patients with lethargy had longer hospital stays and a higher likelihood of postoperative complications (P < 0.05). There were no significant differences in complication rates between the embolization and nonembolization groups. CONCLUSIONS: Despite higher surgical risk profiles, embolized patients had similar complication rates and postoperative hydrocephalus management as nonembolized patients. Embolization was particularly beneficial in patients at high risk for surgical morbidity, such as those <2 years, weighing <10 kg, and with a tumor volume >15 cm3.

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.

Transcallosal and endoscopic hybrid approach to a rare entity of pediatric intraventricular tumors-cribriform neuroepithelial tumor: a case report and literature review.

PURPOSE: Cribriform neuroepithelial tumor (CRINET) is a provisional category of intraventricular tumors, sharing similarities with AT/RTs, and there is a lack of data about its pathology, prognosis, and surgical approaches in the literature. We have been challenged to describe the surgical approach to a rare case of CRINET and describe the intraoperative features since none has been described before. Surgical resection and chemotherapy hold a great importance of favorable prognosis. METHODS: Twenty-month-old male with intraventricular tumor underwent transcallosal intraventricular tumor resection and endoscopic intraventricular second look stages. The tumor was initially considered choroid plexus carcinoma and histopathological results pointed CRINET. The patient also received Ommaya reservoir for intrathecal chemotherapy employment. The patient's preoperative and postoperative MRI scans and tumor's pathological features are described with a brief history of the disease in the literature. RESULTS: Lack of SMARCB1 gene immunoreactivity and presence of cribriform non-rhabdoid trabecular neuroepithelial cells led to the CRINET diagnosis. The surgical technique helped us to approach directly into the third ventricle and perform total resection and intraventricular lavage. The patient recovered without any perioperative complications and is consulted pediatric oncology for further treatment planning. CONCLUSION: With our limited knowledge on the matter, our presentation may provide an inside to the course and progress of the CRINET as a very rare tumor and may help to set a basis for future investigations focused on its clinical and pathological features. Long courses of follow-up periods are required for establishing treatment modules and assessing the responses to surgical resection techniques and chemotherapy protocols.

Metastasis of the choroid plexuses: A systematic review of the literature and case illustration.

BACKGROUND: Choroid plexus (CP) metastases are an extremely rare condition accounting for less than 1% of brain metastases. Due to its scarcity, little is known about this pathology and its management. Herein, we propose a review of the current literature to help its diagnosis and management. METHODS: Through a literature review based on PubMed/MEDLINE database, we reviewed 94 cases of intraventricular metastasis of solid cancer in 28 full-text articles in English from 1980 to 2010. We have reported epidemiological, clinical, radiological, histological data, as well as management strategies and outcomes. A case report of fourth ventricular pulmonary metastasis illustrates this review. RESULTS: Intraventricular metastases are most often reported in patients in their 6th decade. The clinical presentation is marked by acute hydrocephalus, more rarely lesional bleeding. Three-quarters of intraventricular metastases develop in lateral ventricle, then respectively in the fourth and third ventricles. Kidney cancer accounts for 45% of the cases. The treatment modalities are surgical removal in case of a single lesion and adjuvant radiotherapy and chemotherapy depending on the primary cancer. The prognosis remains poor due to dissemination via the cerebrospinal fluid. CONCLUSION: Multiple choroid plexus metastasis is a rare diagnosis, affecting patients with a specific clinical presentation and a misleading radiological appearance. There is no standard of care for the management of these lesions and surgical approach can be challenging.

Use of a telovelar approach for complete resection of a choroid plexus tumor in a dog.

OBJECTIVE: To describe a telovelar approach to the fourth ventricle for excision of a choroid plexus tumor within the ventricle. ANIMAL: A 3-year-old entire male Chihuahua. STUDY DESIGN: Case report METHODS: A 3-year-old dog with two-month history of progressive vestibular signs and subdued mentation was diagnosed with a fourth ventricle tumor. Gross total resection of the tumor was achieved through a telovelar approach to the fourth ventricle. RESULTS: Complete removal of the tumor was confirmed on immediate postoperative MRI. The dog recovered from the surgical procedure without complications, displaying some neurological deficits as preoperatively. His neurological examination was normal 2 weeks after surgery and remained so until the time of writing this case report (28 months) without additional treatment. CONCLUSION: The telovelar approach allowed complete excision of a choroid plexus tumor located in the fourth ventricle of the dog reported here.

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.

Symptomatic bilateral choroid plexus xanthogranuloma in a pediatric patient.

INTRODUCTION: Central nervous system xanthogranulomas are uncommon clinical entities, and symptomatic bilateral choroid plexus xanthogranulomas are rare. CASE PRESENTATION: We present the case of a 15-year-old male patient with bilateral choroid plexus xanthogranulomas with symptoms of increased intracranial pressure. Gross total resection of the tumor in the left lateral ventricle was performed. The patient improved, and asymptomatic right-sided tumor was monitored at follow-up. CONCLUSION: The main treatment objective in these tumors is gross total resection. Asymptomatic tumors can be followed without intervention. However, surgery should be performed for symptomatic tumors that cause hydrocephalus or symptoms of increased intracranial pressure.

Pre-operative embolization for staged treatment of infantile choroid plexus papilloma.

Choroid plexus papillomas (CPPs) are benign but rare neuroepithelial neoplasms of the choroid plexus that represent the non-malignant form of a spectrum of tumors of the choroid plexus. The vast majority of CPPs present in children under 5 years of age. Some CPPs are diagnosed prenatally, but many of them reach a large size before diagnosis. CPPs typically present with signs and symptoms of hydrocephalus. Treatment of these tumors has traditionally been with surgical resection. Large CPPs in young children present a challenge due to risk of high blood loss during resection. Here, the authors describe the case of a 3-month-old presenting with hydrocephalus and a large CPP of the third ventricle that was managed with a staged strategy of embolization followed by a delayed resection, allowing the tumor to involute prior to surgery.

A choroid plexus metastasis of a prostatic adenocarcinoma mimicking a choroid plexus carcinoma: A case report.

A multicystic intraventricular tumour of the right ventricular atrium was incidentally diagnosed on follow-up imaging of a 61-year-old man with a history of prostatic adenocarcinoma. Surgical resection of the lesion was performed after a one-year radio-clinical follow-up due to progressive expansion of the lesion size and a rising prostate specific antigen blood-level. Morphological features with papillary pattern on pathological examination were compatible with malignant adenocarcinoma or choroid plexus carcinoma. The immunoprofile was conclusive for an exceptional choroid plexus metastasis (CPM) of a prostatic adenocarcinoma. To our knowledge, this is the first report of a proven prostatic origin of a CPM.

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.

Choroid plexus carcinoma with leptomeningeal spread in an adult: a case report and review of the literature.

BACKGROUND: Choroid plexus carcinoma is an intraventricular neoplasm originating from the choroid plexus epithelium and is of rare occurrence in adults. However, owing to the low prevalence of choroid plexus carcinoma, there is very limited information about the disease entity and treatment. Here we report a rare case of choroid plexus carcinoma in an adult patient. CASE PRESENTATION: A 46-year-old South Korean (East Asian) male presented with low back pain, headache, and diplopia. Magnetic resonance imaging demonstrated enhancing mass lesion in the left trigone, cerebellar with leptomeningeal spread. Surgery was performed via left parietal craniotomy, and the lesion was histologically confirmed to be choroid plexus carcinoma. The patient received adjuvant craniospinal irradiation for remnant mass and leptomeningeal spread. Magnetic resonance imaging performed immediately after completion of the treatment revealed a partial decrease in the size of the tumor. However, the patient expired died as a result of acute respiratory distress syndrome before follow-up of long-term outcome. CONCLUSION: Choroid plexus carcinoma with leptomeningeal spread in adults is very important for rapid diagnosis and treatment. In the case of the presence of leptomeningeal spread, craniospinal irradiation can be considered as a treatment method, but may have serious complications. Hence, the technique should be applied with care.

Choroid plexus carcinoma in an adolescent male: a case report.

INTRODUCTION/BACKGROUND: Although central nervous system tumors are the most common etiology of malignancies in the pediatric age group, choroid plexus carcinomas are rare, with an annual incidence rate of 0.10 per 100,000 children. CASE PRESENTATION: We report the case of an adolescent male belonging to central India who had presented with a history of persistent headache, projectile vomiting, neck stiffness, and an episode of generalized tonic-clonic seizure. Neurological examination was suggestive of a space-occupying lesion. Further neuroimaging was suggestive of a large left-sided choroid plexus carcinoma, later confirmed on pathological examination. Gross total resection was achieved and followed by radiation therapy. His recovery was satisfactory without any major events despite suffering from such a malignancy with a poor prognosis. CONCLUSION: In the absence of a global consensus on choroid plexus carcinoma management, our patient underwent a successful gross total resection and received postoperative radiotherapy. He made a satisfactory recovery with a further plan to review with gadolinium-enhanced neuroimaging at a later date. We conclude that, when possible, achieving gross total resection is of utmost importance.

Intracranial Choroid Plexus Carcinomas: Report of 11 Cases from a Single Institution.

BACKGROUND: Choroid plexus carcinoma is a central nervous system tumor pathologically corresponding to World Health Organization grade III. Choroid plexus carcinoma mainly affects pediatric patients with a poor prognosis. Due to its rarity, standardized treatment has not yet been outlined. METHODS: We retrospectively analyzed 11 patients with histopathologically diagnosed choroid plexus carcinoma between January 2008 and December 2016. They were treated with surgical resection with or without adjuvant therapies. The clinical profiles and outcomes were analyzed. RESULTS: The mean age at diagnosis was 16.0 years (median, 7.0 years; range, 4 months to ∼59 years). Gross total resection was achieved in 9 cases, and subtotal resection in 2 cases. Seven patients received adjuvant radiotherapy, and 2 patients underwent chemotherapy. The mean overall survival was 34.8 months, and the mean progression-free survival was 24.5 months. During the follow-up period, 4 patients succumbed to central nervous system dissemination of choroid plexus carcinoma including 2 patients with malignant transformation from atypical choroid plexus papilloma to choroid plexus carcinoma and 1 patient treated with the combined chemotherapy protocol. CONCLUSIONS: In this study, we described the clinicoradiologic characteristics of choroid plexus carcinomas. Surgical resection is the mainstream treatment. Due to the paucity of clinical evidence, the standard regimen of adjuvant therapies still needs further research.

Surgical management of choroid plexus papilloma of the cerebellopontine and cerebellomedullary angle: classification and strategy.

Choroid plexus papillomas (CPPs) are primary neuroectodermal neoplasms that usually arise in the fourth ventricle in adults. In this study, we present 12 patients with CPP arising from the cerebellopontine angle (CPP-CPA) and/or of the cerebellomedullary angle (CPP-CMA) that were treated in our department. Patients who underwent surgery for the treatment for CPP-CPA/CMA from January 2004 to March 2020 were identified by a computer search of their files from the Department of Neurosurgery, Tübingen. CPPs were classified according to their location into type 1 (tumor portion only in the CPA,), type 2 (tumor portions only in the CMA), and type 3 (tumor portions both in the CPA and CMA). Patients were evaluated for initial symptoms, previous therapies in other hospitals, extent of tumor resection, recurrence rate, and complications by reviewing patient documents. Of approximately 1500 CPA lesions, which were surgically treated in our department in the last 16 years, 12 patients (mean age 42 ± 19 years) were found to have CPP-CPA/CMA. Five were male, and seven were female patients. Gross total resection was achieved in nine cases, and a subtotal resection was attained in three cases. Tumor recurrence in the same location after the first surgery in our hospital was observed in 2 patients after 15 and 40 months of follow-up, and in another patient, distant metastases (C3/4 and L3 levels) were observed. Surgical removal of CPP is the treatment of choice, but additional therapeutic options may be necessary in case of remnant tumor portions, recurrence, or malignant transformation.

Management of choroid plexus tumours: A comprehensive study from a tertiary hospital.

OBJECTIVE: Choroid plexus tumours (CPT) are rare intraventricular tumours representing less than 0.5 % of brain tumours. The tumour is commonly located in the supratentorial region, but the location varies depending on the age. We present our experience of managing these tumours in a tertiary hospital. METHODS: Retrospectively, we reviewed our operative database and recruited 80 cases of CPT who underwent surgical treatment in our institute from 1995 to 2018. We analysed the factors affecting the outcome and the perioperative complications of the choroid plexus tumour. RESULTS: A total of 80 choroid plexus tumours were recruited in our retrospective review, of which 44 were choroid plexus papilloma (CPP), 13 were atypical choroid plexus tumours (ACPP), 23 were choroid plexus carcinomas (CPC). The mean age was 16.75 (SD 16.71) in the overall cohort. Males were found to be predominant in all tumour groups (M/F: 46/34). Headache was the most common symptom (52.5 %). Hydrocephalus was seen in 53.8 % of cases. The median overall survival was 89.88 months. Gross total resection was achieved in 62.5 % cases (n = 50/80), and near-total resection in 27. 5 % cases (n = 22/80). The median overall survival was 89.88 months. The median overall survival for CPP, ACPP, CPC was 106.83, 37.37, 36.19 months, respectively. Median Event-free survival was 65.83 months. A Cox regression analysis of predictors of overall survival of atypical CPP and CPC was done, in which age, sex, location, size, the extent of the resection, and complications were considered. The extent of the resection (p = 0.01) and the size (p = 0.02) were related to overall survival CONCLUSION: CPT's are the rare intraventricular tumours, which requires aggressive resection strategies. The extent of resection offers survival benefit based on the histological grades.

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.

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.