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.

Hypertensive nonobstructive hydrocephalus as main magnetic resonance imaging feature in a dog with disseminated choroid plexus carcinomatosis.

Obstructive or nonobstructive hypertensive hydrocephalus is reported in choroid plexus tumors. Choroid plexus tumors typically present as T2-weighted hyperintense intraventricular masses with occasional cerebrospinal fluid-drop metastasis. Acquired neoplastic nonobstructive hydrocephalus without visible mass lesion in magnetic resonance imaging is not reported in dogs. A 4.5-year-old Rhodesian Ridgeback presented with reduced mental status, unilaterally absent pupillary light reflex, and neck pain. Magnetic resonance imaging revealed a nonobstructive hydrocephalus and widened lumbar subarachnoid space with no evidence of a primary mass lesion. Postmortem examination confirmed a disseminated choroid plexus tumor affecting the ependyma and choroid plexi of all ventricles and the cerebral and lumbar subarachnoid space. Disseminated choroid plexus carcinomatosis should be considered as a possible cause of hypertensive hydrocephalus even in absence of a primary mass.

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.

Water-Filled Vesicles of Choroid Plexus Tumors.

OBJECTIVE: Choroid plexus cysts are the most common neuro-epithelial cysts. METHODS: The authors describe 2 cases. The first case is a 1-year-old child presenting with hydrocephalus and cyst of the choroid plexus. The child was treated with endoscopic fenestration of the cysts. RESULTS: The histological examination of the cyst wall was consistent with choroid epithelium and water-filled vesicles. The second case is a 63 year old male with a highly vascularized tumor extending to choroid plexus. A quaductus Silvius was obstructed by the tumor. The histopathologic examination of choroid plexus showed a low number, water-vesicles. CONCLUSIONS: According to our knowledge, there are no previous reports of water-filled vesicles with choroid plexus cyst causing hydrocephalus. The first case is a good example of over secretion of cerebrospinal fluid (CSF) with the water-filled vesicle. The second case with low number of water filled vesicle shows that the cause of hydrocephalus is not over production of CSF, it is blockage of the CSF pathway in Aquaductus Silvius by the tumor itself. These 2 cases are illustrative and more importantly highlight the need to study for water-filled vesicles in these kind of cases.

Temporal Horn Choroid Plexus Papilloma Presenting with Seizures in Adulthood: Clinical Case Report and Review of the Literature.

BACKGROUND: Choroid plexus papillomas (CPPs) are benign World Health Organization grade I tumors that comprise 2%-4% of all brain tumors among children and less than 1% of brain tumors in adults. Most adult cases occur in the fourth ventricle, with only 1 previous report describing an adult patient with a temporal horn CPP. CASE DESCRIPTION: We report a rare case of a temporal horn CPP presenting in an adult with seizures. We performed a minimally invasive subtemporal approach for gross total resection of the lesion. CONCLUSIONS: CPP presenting in the temporal horn is rare among adults. We discuss the surgical nuances of the subtemporal approach for resection and review the literature regarding adult presentation of CPP and the treatment strategies for adult CPP.

Management of Choroid Plexus Tumors in Infants and Young Children Up to 4 Years of Age: An Institutional Experience.

BACKGROUND: Choroid plexus tumors (CPTs) are rare tumors characterized by papillary and intraventricular growth. The young age of presentation of such tumors, especially in infants, and the lack of consensus on adjuvant therapy in case of atypical choroid plexus papilloma (aCPP) and choroid plexus carcinoma (CPC) create dilemma for the management of such tumors. We discuss the presentation, management, complications, and outcome in 15 patients (children 4 years of age and younger) and review pertinent literature. METHODS: We retrospectively analyzed the case records of all patients with CPTs who were operated in our institute from January 2010 to March 2018. We found 15 patients in the age group of 0-4 years of age. The variables analyzed include age, sex, presentation, location, surgical approach, extent of resection, intraoperative blood loss, percentage of blood loss, blood transfused, histopathology, postoperative complications, and outcome. Images were obtained from picture archiving and communication system, and patient details and follow-up were obtained from discharge summary, operative notes, and hospital records. RESULTS: Ten patients had choroid plexus papilloma (CPP), 2 patients had aCPP, and 3 patients had CPC. The mean age was 15.2 months, whereas the median age was 8 months (range, 40 days-4 years). The mean blood loss was 329 mL, whereas the median blood loss was 175 mL. There were a total of 5 deaths, including 3 patients with CPC and 1 each with aCPP and CPP. CONCLUSIONS: CPTs are challenging tumors in infants and very young children because of the potential for massive blood loss. CPP is associated with lesser blood loss and favorable outcome compared with aCPP and CPC. Massive blood loss in CPC and aCPP can be life threatening as has been shown in our series. CPC has a rapid proliferation potential as shown in one of our cases. Attempts at decreasing vascularization of such tumors should be made by various methods, including preoperative embolization and neoadjuvant chemotherapy; however, a consensus on this is lacking.

Spinal drop metastasis from a benign fourth ventricular choroid plexus papilloma in a pediatric patient: case report.

Choroid plexus papillomas (CPPs) are typically benign tumors that can occur in any age group but are more commonly found in pediatric patients. Although these tumors are benign, there are several reports in adult patients of distant metastases present either at the time of diagnosis or occurring months to years after initial resection. Here, the authors report the case of a 14-year-old boy who presented with symptoms of elevated intracranial pressure due to obstructive hydrocephalus that was caused by a large fourth ventricular mass. Preoperative imaging included a full MRI of the spine, which revealed an intradural lesion that encased the distal sacral nerve roots at the tip of the thecal sac and was concerning for a drop metastasis. The patient underwent gross-total resection of both the fourth ventricular and sacral tumors with histology of both lesions consistent with benign CPP (WHO Grade I). In addition, the authors review prior reports of both pediatric and adult patients in whom benign CPPs have metastasized with either benign or atypical pathology found at a distant site. Taking into account this unusual case and reports in the literature, patients with even benign CPPs may warrant initial and routine follow-up imaging of the total neural axis in search of the rare, but possible, occurrence of drop metastasis.

Hidrocefalia por hiperplasia de plexos coroideos en un paciente con mosaicismo de trisomía 9. Un verdadero reto diagnóstico y terapéutico / Hydrocephalus due to hyperplasia of the choroid plexuses in a patient with trisomy 9 mosaicism. A real diagnostic and therapeutic challenge

Introducción. La trisomía 9 es una cromosomopatía inusual en pacientes nacidos vivos, que frecuentemente se acompaña de anomalías funcionales y estructurales del sistema nervioso central. Entre otras muchas alteraciones, varios trabajos en la bibliografía anglosajona demuestran una asociación entre cromosomopatía 9 y patología de los plexos coroideos. Caso clínico. Varón de 4 meses de vida con mosaicismo de trisomía 9 asociado a hidrocefalia secundaria a hiperplasia de los plexos coroideos, que fue remitido por clínica de hipertensión intracraneal. El procedimiento derivativo de líquido cefalorraquídeo por el que optamos inicialmente provocó una ascitis masiva debida a la producción desmesurada de líquido cefalorraquídeo, y desembocó en una cascada de múltiples intervenciones quirúrgicas, entre las que se incluyeron procedimientos endoscópicos y derivativos. Conclusiones. Se trata de un ejemplo más de asociación entre patología de los plexos coroideos y cromosomopatía 9. Debido a su escasa incidencia, es difícil establecer el diagnóstico de hidrocefalia secundaria a hiperplasia de los plexos y, por tanto, el tratamiento más adecuado. En este tipo de hidrocefalia existe un doble mecanismo fisiopatológico, que implica un aumento de producción de líquido cefalorraquídeo y una disminución de su reabsorción. A pesar de tener en cuenta dicha consideración, el tratamiento de la hidrocefalia secundaria a hiperplasia de los plexos supone un verdadero reto que habitualmente pasa por múltiples procedimientos quirúrgicos, desde la plexectomía o coagulación de los plexos coroideos hasta la implantación de dispositivos de derivación de líquido cefalorraquídeo (AU)

Introduction. Trisomy 9 is an unusual chromosome abnormality in live-born patients, which is frequently accompanied by functional and structural anomalies of the central nervous system. Among many other alterations, several studies have been published in the English-speaking literature that show an association between chromosome 9 abnormality and pathologies affecting the choroid plexuses. Case report. We report the case of a 4-month-old male with trisomy 9 mosaicism associated to hydrocephalus secondary to choroid plexus hyperplasia, who was referred due to a clinical picture of intracranial hypertension. The cerebrospinal fluid (CSF) drainage procedure that was initially chosen caused massive ascites due to an excessive production of CSF, and led to a cascade of multiple surgical interventions, which included endoscopic and drainage procedures. Conclusions. This is another example of an association between choroid plexus pathologies and chromosome 9 abnormality. Due to its scarce incidence, diagnosis of hydrocephalus secondary to plexus hyperplasia is difficult, as is selecting its most suitable treatment. In this type of hydrocephalus there is a double pathophysiological mechanism, which involves an increase in CSF production and a decrease in its reabsorption. Despite taking these considerations into account, the treatment of hydrocephalus secondary to plexus hyperplasia is a real challenge that usually leads to multiple surgical interventions ranging from plexectomy or coagulation of the choroid plexuses to the implantation of CSF drainage devices (AU)

Radiation therapy for choroid plexus carcinoma patients with Li-Fraumeni syndrome: advantageous or detrimental?

BACKGROUND: Choroid plexus carcinomas (CPCs) are rare pediatric tumors often associated with Li-Fraumeni Syndrome (LFS), a germline mutation in the TP53 tumor-suppressor gene, predisposing to cancer. MATERIALS AND METHODS: We performed a systemic literature review from 1990-2013 to evaluate the hypothesis that radiation therapy should be avoided in patients with CPC and LFS. Overall survival (OS) was compared using Kaplan-Meier curves and log-rank tests. RESULTS: Twenty-eight patients were documented with CPC and LFS. Eleven out of 17 patients received radiation therapy. The survival of patients receiving radiation was inferior to that of those without radiation [median (±95% confidence interval) 2-year OS=0.18 ± 0.12% versus 0.58 ± 0.12%]. The log-rank tests suggested the difference to be marginally significant (p=0.056). CONCLUSION: This finding provides evidence for pursuing treatment approaches that do not include radiation therapy for patients with LFS.

PET/TC de cuerpo completo para la detección de metástasis de melanoma coroideo / Whole body PET/CT imaging for detection of metastatic choroidal melanoma

El melanoma coroideo es el tumor maligno ocular primario más frecuente en los adultos y las metástasis más frecuentes son a nivel hepático con un mal pronóstico a pesar de los tratamientos. Estas metástasis se han descrito hasta en el 50 % de los melanomas coroideos. Estudios recientes muestran que la ecografía hepática y las analíticas hepáticas tienen baja sensibilidad en el diagnóstico de las metástasis hepáticas de los melanomas de úvea. Exponemos el caso de un paciente con historia de melanoma coroideo. La exploración preoperatoria, incluyendo TC de abdomen, analítica sanguínea y enzimas hepáticos resultó normal. En un PET/TC realizado mostró lesiones en el hígado y pulmones. El PET/TC de cuerpo entero mostró metástasis de un melanoma uveal. El PET/TC es una herramienta útil para la detección de las metástasis hepáticas y extrahepáticas (AU)

Choroidal melanoma is the most common primary intraocular cancer in adults. Metastases are most commonly found in the liver and are rapidly fatal despite aggressive therapy. These metastases have been reported in the follow-up to 50 % of uveal melanoma. However, recent reports have shown that liver ultrasonography and liver function tests have low sensitivity in the diagnosis of metastatic uveal melanoma. The study reports on a patient with a history of choroidal melanoma. Preoperative medical evaluation, including CT imaging of the abdomen, complete blood count and liver enzymes proved negative. A PET/CT was requested and showed lesions in the liver and lungs. Whole-body PET/CT revealed melanoma metastases. The PET/CT is a sensitive tool for the detection and localization of hepatic and extrahepatic metastatic choroidal melanoma (AU)

Spitzoid melanoma in a child with Li-Fraumeni syndrome.

Spitzoid melanoma of childhood is a rare malignancy. The histological features are at the upper end of a range encompassing Spitz nevus and atypical Spitz tumor, the unifying features including large oval, fusiform or polygonal melanocytes with abundant homogeneous-appearing cytoplasma and large vesicular nuclei. The presence of a "bottom-heavy" pattern, strikingly enlarged nuclei and nucleoli in both the upper and lower portions of the lesion, and deep mitotic figures are among the findings that distinguish most of the Spitzoid melanomas from Spitz nevi and atypical Spitz tumors. There are no syndromic associations reported for this malignancy. We report the occurrence of choroid plexus carcinoma, Spitzoid melanoma, and myelodysplasia in a child who was found to carry a germline mutation for TP53. While choroid plexus carcinoma and myelodysplasia have relatively frequently been described, melanomas have been very rarely described in Li-Fraumeni syndrome. The association of Spitzoid melanoma with Li-Fraumeni syndrome, especially in a pediatric patient, has not been reported before.

Endoscopic treatment of a third ventricle choroid plexus cyst.

Choroid plexus cysts are frequent benign intraventricular lesions that infrequently cause symptoms, usually in the form of obstructive hydrocephalus. These instances are even less common in the adult population. When warranted, treatment seeks to reestablish cerebrospinal fluid flow and does not necessarily require resection of the cyst itself. Hence, endoscopic exploration of the ventricles with subsequent cyst ablation is the current treatment of choice for these lesions. Herein we present the case of a 25-year-old female patient with a 3-week history of intermittent headaches. Investigation with computerized tomography (CT) of the head detected supratentorial hydrocephalus, with enlargement of the lateral and third ventricles. Magnetic resonance imaging revealed a homogeneous cystic lesion in the third ventricle. A right-sided, pre-coronal burr hole was carried out, followed by endoscopic exploration of the ventricular system. A third-ventriclostomy was performed. With the aid of the 30-degrees endoscope, a cyst arising from the choroid plexus was visualized along the posterior portion of the third ventricle, obstructing the aqueduct opening. The cyst was cauterized until significant reduction of its dimensions was achieved and the aqueduct opening was liberated. Postoperative recovery was without incident and resolution of the hydrocephalus was confirmed by CT imaging. The patient reports complete improvement of her headaches and has been uneventfully followed since surgery. The video can be found here: http://youtu.be/XBtj_SqY07Q. (http://thejns.org/doi/abs/10.3171/2013.V1.FOCUS12332)

Choroid plexus tumors; management, outcome, and association with the Li-Fraumeni syndrome: the Children's Hospital Los Angeles (CHLA) experience, 1991-2010.

BACKGROUND: Choroid plexus tumors (CPT) are rare, and predominate in early childhood. An association with the Li-Fraumeni syndrome (LFS) has been reported, but the biological and clinical implications of this association remain poorly defined. We have investigated the clinical features and overall survival of all CPT patients treated at Children's Hospital Los Angeles (CHLA) over a 20-year period, with particular attention to the association of CPT with LFS. METHODS: A retrospective evaluation of the course of therapy and clinical outcome was undertaken on the 42 patients diagnosed with and treated for CPT at CHLA from January 1991 to December 2010. Any association with multiple primary tumors and family histories consistent with LFS was investigated in all patients. RESULTS: Six of the 42 patients (16.7%), demonstrated either phenotypic and/or genotypic characteristics consistent with LFS, with either a distinct family history of cancer, a synchronous diagnosis of a different type of cancer, or the subsequent development of metachronous cancers. Of 11 patients with choroid plexus carcinoma tested for TP53 germline mutations, four (36.4%) were positive. A single patient with a choroid plexus papilloma had phenotypic characteristics of LFS but tested negative for TP53. CONCLUSIONS: Children with CPC appear to have a high frequency of TP53 germline mutations in association with LFS. This raises the question whether all children with CPC should be tested for TP53 germline mutations in order to institute screening to enhance early detection and treatment of subsequent cancers.

[Tumors of the lateral ventricle in child: characteristics and specificities]. / Tumeurs du ventricule latéral de l'enfant: particularités et spécificités.

Tumors of the lateral ventricle are not as rare in children as in adults and present complex problems which are to be dealt with often in a context of emergency, on account of their large size and the risk of life-threatening bleeding in a small child. Their diagnostic features are different in children, especially in infants and even more so in the fetus. The main surgical problems are the risk of bleeding and of hydraulic complications. From an oncological perspective, the main tumor types are choroid plexus tumors, subependymal giant-cell astrocytoma, and low-grade gliomas. The developmental outcome of these patients is often poor, on account of severe intracranial hypertension and young age at diagnosis.

Fetal ventriculomegaly secondary to isolated large choroid plexus cysts: prenatal findings and postnatal outcome.

OBJECTIVE: To report the prenatal findings and postnatal outcome of fetal ventriculomegaly associated with isolated large choroid plexus cysts (CPCs). METHOD: Cases of isolated fetal ventriculomegaly and large CPCs (>10 mm) were identified through a search of patient records from 2003 to 2006. Ultrasound (US) findings were reviewed: unilateral or bilateral ventriculomegaly, ventricular size, size of CPCs, and changes on serial scans. Correlation was made with fetal magnetic resonance imaging (MRI), pregnancy outcome, and long-term follow-up. RESULTS: Six cases of isolated large CPCs (12-30 mm) with ventriculomegaly (11-17 mm) were detected on US at 18 to 26 weeks of gestation. Serial prenatal US showed the CPCs resolved (one case) or decreased in size (five cases). Ventricular size became normal during pregnancy in five cases and decreased in size in one case. Fetal MRI performed in three cases showed no additional findings. Five patients had amniocentesis which showed normal karyotype. There was one termination of pregnancy (the fetus showed no abnormality on external examination). There were five healthy newborns, with follow-up to 4.5 years of age (one), 5.5 years (one), and 6 years (three). All had normal physical and developmental outcome. CONCLUSION: Large isolated CPCs may transiently dilate the fetal cerebral ventricles. Follow-up to 6 years has shown normal growth and development.

Large choroid plexus teratoma: a rare cause of congenital hydrocephalus.

Teratomas form the most common type of congenital brain tumors, frequently presenting as stillbirth. The largest neonatal series of intracranial teratomas reported a 12% survival rate. Although the first teratoma of the lateral ventricle was reported in 1961 by Maier, neonatal intracranial teratoma of the lateral ventricle is an extremely rare entity. We report here a large intracranial poorly differentiated teratoma arising from choroid plexus of lateral ventricle. This typically presented at birth with a large congenital hydrocephalus.