Abstract
Choroid plexus tumors (CPTs) are rare neoplasms arising from the choroid plexus epithelium, and occur predominantly in infants and children. CPTs may be associated with TP53 germline mutations (Li–Fraumeni syndrome), but the majority of CPTs are sporadic. Most commonly, CPTs present with signs of increased intracranial pressure; on imaging they are characterized as intraventricular contrast-enhancing masses. Histopathologically, CPTs comprise benign choroid plexus papilloma (CPP, WHO grade I), atypical choroid plexus papilloma (APP, WHO grade II), and malignant choroid plexus carcinoma (CPC, WHO grade III). Complete surgical resection may be curative for CPPs, while CPCs are additionally treated with chemotherapy and radiation. In this chapter we discuss these features in detail, as well as differential diagnosis and immunohistochemical studies. Particular emphasis is placed on the molecular alterations, how they can be detected in the laboratory using immunohistochemistry and molecular testing, and how they may lead to novel-targeted therapeutic approaches.
Access provided by Autonomous University of Puebla. Download chapter PDF
Similar content being viewed by others
Keywords
- Choroid plexus tumors
- Pediatric age group
- Choroid plexus papilloma
- Atypical choroid plexus papilloma
- Choroid plexus carcinoma
- p53
- Histopathology
- Molecular diagnostics and therapeutics
Definition
Choroid plexus tumors (CPTs) are neoplasms derived from the choroid plexus epithelium of the brain. Three types have been described. (1) Choroid plexus papilloma (CPP), with delicate fibrovascular connective tissue covered by a single layer of epithelial cells with round or oval monomorphic nuclei defined as WHO grade I tumors [1] (Fig. 12.1b). (2) Atypical choroid plexus papillomas (APPs) are characterized by increased mitotic activity and are WHO grade II tumors. [1] (Fig. 12.1c). A mitotic index of two or more mitoses per 10 randomly selected high power fields (HPFs) (one HPF corresponding to 0.23 mm2) can be used to establish the diagnosis of APP [2]. (3) Choroid plexus carcinomas (CPCs) are characterized by frank histological signs of malignancy, including at least four of the following five features: (a) frequent mitoses (usually greater than 5 per 10 HPFs), (b) increased cellular density, (c) nuclear pleomorphism, (d) blurring of the papillary pattern, and (e) necrosis (Figs. 12.1d and 12.2). These tumors correspond to WHO grade III [1].
Clinical Features
Epidemiology
CPTs are rare, representing between 4 and 9 % of brain tumors in population-based studies [3, 4]. The typical age of presentation is the first year of life. However, CPTs can occur as rare congenital brain tumors [5, 6] and in adults, including the elderly [7–9]. These tumors also occur in other species including canines [10–14]. While most tumors are sporadic, CPTs are reported in: Li–Fraumeni syndrome (discussed below), pediatric patients with large melanocytic skin lesions [15], Pierpont syndrome [16], hypermelanosis Ito [17], and Aicardi syndrome [18]. CPPs can also be seen in von Hippel–Lindau disease [19], but it is not known if loss of the VHL allele contributes to the pathogenesis of these tumors. It is possible that these abnormalities indicate rare pathways of tumorigenesis that are yet to be elucidated.
Clinical Presenting Features
Raised intracranial pressure by hydrocephalus is the main presenting feature of CPTs [20, 21]. Other presenting symptoms may include blindness, non-focal symptoms such as convulsions [20] or focal signs such as hemiparesis [22, 23]. Large amounts of cerebral spinal fluid (CSF) production may also be encountered even when a drain or shunt is placed [24]. Sometimes highly vascularized tumors present as acute intracranial hemorrhage without previous symptoms [25]. Tumor locations in the cerebello-pontine angle may result in more specific signs such as unilateral rhinorrhea and otorrhea [26], or trigeminal neuralgia [27].
Imaging and Tumor Localization
The typical imaging characteristics of CPTs include intraventricular location and contrast enhancement indicating a high degree of vascularization. Invasion of brain tissue is a characteristic finding for CPCs as opposed to CPPs. CPTs are more common in the lateral ventricles [28] than in the third or fourth ventricles [29]. The cerebello-pontine angle is a typical location when the tumor originates in the choroid plexus of the fourth ventricle [30]. Ectopic locations unrelated to the ventricles/choroid plexus have also been described [23, 31–34]. Lateral ventricle tumors are more frequent in infants, while cerebello-pontine angle tumors occur more frequently in adults [30]. An unusual feature of CPTs is that even WHO grade I and II tumors have the potential to metastasize [35–39]. The typical metastatic route is through the CSF [39, 40], often to the spine but also with surprising frequency to the internal ear canals, pituitary stalk, and interpeduncular fossa [41]. Metastases can occur sometimes many years after primary tumor resection. Imaging of the entire craniospinal axis is recommended [40, 42, 43]. Rarely, hematogeneous metastases can occur to other parts of the brain [44], abdomen [45], bone [46, 47], and lung [48]. Abdominal seeding has been described in patients with ventricular-peritoneal shunts [49].
Imaging techniques for CPTs differ in several aspects from that for other brain tumors. As CPTs are the most frequent brain tumors detected on prenatal ultrasound [50–53], ultrasound remains an important diagnostic tool until the fontanels close [6, 51, 54]. On magnetic resonance imaging (MRI), CPTs show inhomogeneity on T2-weighted images, and moderate to marked contrast enhancement. Diagnostic specificity increases when age and intraventricular location are considered. Contrast enhancement, a common finding in these tumors, might be related to the rich vascular stroma. However, extraventricular tumors might not demonstrate contrast enhancement [34]. Differentiating the degree of malignancy on MRI can be difficult. Nevertheless, some general patterns may be observed. CPPs are usually irregular, lobulated, and solid-cystic masses, whereas CPC may present as a poorly defined, mixed-intensity mass [55]. Extensive peritumoral edema and necrosis is more frequent in CPC than in CPP [56, 57]. A thin capsule may be seen in CPP [55].
More recently, nuclear medicine methods and molecular imaging may improve diagnostic ability and also address challenging clinical issues including how to distinguish between tumor and postsurgical scars/postradiation pseudo-progression. For example, sestamibi, an agent that accumulates in mitochondria, has been used to distinguish CPTs from other brain tumors or postsurgical scars [58–62].
Pathology and Diagnostics
Macroscopy
CPTs appear macroscopically as space-occupying lesions located in the ventricles and, less commonly, in extraventricular locations. Grossly, CPPs are well demarcated with a cauliflower-like appearance. They may be attached to the ventricular wall. CPCs usually show varying degrees of invasion into the surrounding brain. High vascularity and hemorrhages are frequent.
Histopathology
CPTs are typically comprised of epithelial cells with a round or oval nucleus and small amount of surrounding cytoplasm. CPTs are fragile, and drop metastases due to CSF spread are not uncommon [63]. Key cytologic features of CPTs in the CSF include variably sized clusters to frank papillary fragments, and cells that retain epithelial features such as sharply defined cell borders [43, 64].
Specific features of the different CPTs are described below, but from a practical point of view, these tumors can be histologically classified by where they fall along the spectrum of three key histologic features: (1) growth pattern—papillary to solid, (2) mitotic activity/cellular atypia—few or none/absent to moderate/moderate to severe, (3) necrosis—little to none or prominent.
CPPs, the least malignant of CPTs, are composed of delicate fibrovascular connective tissue fronds covered by a single layer of epithelial cells with round or oval monomorphic nuclei (Fig. 12.1b). CPPs can be distinguished from normal choroid plexus (Fig. 12.1a) by an overall increased amount of choroid plexus epithelium with flatter papillae (compared to the typical cobbled stone appearance of normal choroid plexus) comprising cells with increased nuclear to cytoplasmic ratios (Fig. 12.1a, b). These cells rest upon a basement membrane that can be elucidated with special stains for collagen. Mitotic activity is extremely low. Brain invasion, high cellularity, necrosis, nuclear pleomorphism, and focal blurring of the papillary pattern are unusual, but may occur and should prompt the consideration of APP. Rarely, CPPs acquire unusual histological features, including oncocytic change, mucinous degeneration, melanization as well as formation of bone, cartilage, adipose tissue, or neuropil islands. CSF-mediated metastases can occur despite the histologic classification of WHO grade I [38].
An intermediate grade CPT, the atypical choroid plexus papilloma (APP) is recognized by the WHO (Grade II) (Figs. 12.1c and 12.3b). These tumors are defined by the presence of two or more mitoses per ten HPFs in what otherwise appears to be a CPP [1, 2]. Additional histologic features that have been reported in APP by some authors include increased cellularity, nuclear pleomorphism, solid growth, and necrosis. However, none of these features are required for the diagnosis of APP and the isolated occurrence of atypical histological features does not automatically imply malignancy.
CPC is a high-grade (WHO III) tumor of the choroid plexus that demonstrates frank signs of malignancy. In contrast to lower grade CPTs, CPCs demonstrate at least four of the following five features (1) frequent mitoses (Fig. 12.2C, typically more than 5 per 10 HPF), (2) increased cellular density (Figs. 12.1d and 12.2 b, c), (3) nuclear pleomorphism (Figs. 12.1d and 12.2b, c), (4) blurring of the papillary pattern (Figs. 12.1d and 12.2b, c), and (5) necrosis (Fig. 12.2b). Invasion of adjacent brain tissue by CPC is common (Fig. 12.2a). In CPCs that are truly anaplastic, identification of epithelial features may become quite challenging.
Immunohistochemistry
CPTs demonstrate expression of a wide range of immunohistochemical markers, a reminder that despite their epithelial appearance, these cells have a neuroepithelial developmental origin. The typical, though variably expressed, pattern includes S-100 protein, synaptophysin, vimentin, cytokeratins (Fig. 12.4d), glial fibrillary acidic protein (GFAP) (Fig. 12.4c), and transthyretin (TTR) (Fig. 12.4a) [65–67]. While TTR is expressed in normal choroid plexus and in many CPTs, it is unfortunately nonspecific and therefore unreliable as a precise marker of choroid plexus origins in any given tumor. However, immunohistochemical detection for membranous expression of the inward rectifier potassium channel Kir7.1 is considered specific for CPT [68] (Fig. 12.5). The Ki67/MIB index can be helpful in refining tumor grade [69] (Fig. 12.3).
Histological Differential Diagnosis
Depending on the age group, the differential diagnosis of CPTs includes atypical teratoid/rhabdoid tumor (AT/RT), central nervous system (CNS), primitive neuroectodermal tumors (PNET), papillary ependymoma, oligodendroglioma, neurocytoma, papillary tumor of the pineal region (PTPR), and metastases [70–75]. However, the first differential diagnosis to consider is normal choroid plexus. Typically, normal choroid plexus can be distinguished readily enough when the lesion is entirely papillary, with papillae that are not overly cellular and which exhibit a cobblestone surface, and the absence of mitoses (Figs. 12.1a, b). Further, normal choroid plexus epithelial cells express SERCA3, but SERCA3 expression is decreased in CPTs [76].
AT/RT frequently arises in the differential diagnosis, especially in young children [77]. While AT/RT may occasionally demonstrate poorly differentiated epithelial structures, this histologic pattern is generally quite rare. When such cases do arise, the diagnosis may be resolved by immunohistochemical staining for expression of SMARCB1, which is retained in all CPTs (Fig. 12.4b) [73], as well as choroid plexus marker Kir7.1, which stains the majority of CPC but not AT/RT (Fig. 12.5) [78].
Occasionally in pediatric cases, a supratentorial PNET, particularly the medulloepithelioma, may enter into the differential diagnosis. These tumors can usually be distinguished on histologic grounds; they have tubular structures rather than papillary architecture and comprise cells with embryonal rather than epithelial features. Furthermore, medulloepithelioma is characterized by 19q13.42 amplification and LIN28 expression, linking these rare tumors to embryonal tumor with abundant neuropil and true rosettes (ETANTR) [79, 80]. Cribriform neuroepithelial tumor (CRINET) is a rare tumor characterized by cribriform strands and well-defined surfaces, which may be misinterpreted as CPC. Unlike the majority of CPC, however, CRINET is characterized by SMARCB1 loss as well as EMA staining of surfaces [79]. In contrast to AT/RT, prognosis of CRINET seems to be relatively favorable [81].
Papillary ependymomas share the intraventricular location and confusion may arise in CPPs with elongated tumor cells that may give the appearance of overlapping histopathological features (Fig. 12.6a). One important clue to the differential diagnosis is the presence of a delicate basement membrane in CPTs, a feature consistently lacking in ependymomas. While GFAP may be present in both, it is generally stronger and more diffuse in ependymomas. Rarely, synchronous appearance of CPT and ependymoma has been described [82, 83]. PTPR has to be considered in children and young adults with tumors of third ventricular location. The majority of PTPRs can be distinguished from CPTs by absent staining for epithelial membrane antigen and Kir7.1, as well as the presence of distinct MAP-2 immunoreactivity [68].
The endolymphatic sac tumor (ELST) is a low-grade carcinoma originating in the ear. These extremely rare tumors are capable of invading the cerebello-pontine angle and might be mistaken for CPTs in this region. Kir7.1 and EAAT-1 (glutamate transporter) are typically positive in CPTs but absent in ELSTs [84]. The choroid plexus is a common site for metastases and this should be considered in any adult with CPTs. Renal cell carcinoma [85], thyroid carcinoma [86, 87], and cholangiocellular carcinoma [88] primaries have all been reported.
Pathogenesis and Molecular Genetics
Pathogenesis
CPTs were the first models for virally induced brain tumors. Simian Virus 40 (SV40), which naturally infects Asian macaques, has been shown to induce CPTs. The virus is capable of transforming human choroid cells in vitro [89–91] and creates CPTs in hamsters and mice in vivo [92–97]. Transgenic mice harboring the SV40 large T-antigen gene develop CPPs by 80–90 days [98, 99]. SV40 is frequently found in human CPTs [100–103]. The T-antigen of the SV40 virus binds to tumor suppressor genes such as p53 [104] and pRB [99]. This suggests virus-induced tumorigenesis. However, an unintended natural experiment that occurred when the vaccine for poliomyelitis was contaminated with the SV40 virus in India did not produce clear evidence of increased incidence of CPTs. It still remains to be conclusively established if SV40 induces CPTs in humans.
Only limited data are available regarding molecular genetic alterations in CPT. Using comparative genomic hybridization, gains of chromosomes 5, 7, and 9 as well as losses of chromosomes 10 and 22q could be demonstrated in CPP. In contrast, CPC mainly showed gains of chromosomes 1, 4, 12, and 20 as well as losses of 5, 18, and 22q [105]. These findings could be extended using high-resolution methods, showing recurrent copy number gains of chromosomes 1, 2, 4, 12, and 20 as well as losses of chromosomes 5, 6, 16, 18, 19, and 22 in CPC. Clustering analysis separated choroid plexus carcinomas into two groups: one characterized by marked losses and the other characterized by gains across the chromosomes. Chromosomal losses of 9, 19p, and 22q were significantly more frequent in younger children (<36 months), whereas gains on chromosomes 7 and 19, and chromosome arms 8q, 14q, and 21q prevailed in older patients [106].
The involvement of the TP53 tumor suppressor gene in CPT patients was first suggested by the occurrence of CPC in families with Li–Fraumeni syndrome [107–109], and by the observation of p53 inactivation in tumor tissues [69]. A Canadian group reported a large CPT population with p53 alterations [110]. A Brazilian study confirmed these finding on a larger scale [111–113] for a specific mutation TP53 mutation: R337H. This TP53 mutation is also linked to adreno-cortical carcinoma. Interestingly, high-resolution single nucleotide polymorphism (SNP) array analysis did reveal extremely high total structural variation in TP53-mutated CPC tumor genomes compared with TP53 wild-type tumors and CPPs [110]. However, in the absence of TP53 germline mutations CPTs may still arise through the same pathway driven by somatic mutations [114]. Even though a close association between TP53 mutation status and nuclear accumulation of p53 protein is often claimed [110], the majority of CPTs show only weak and focal nuclear staining, suggesting that p53 immunohistochemistry might not be a reliable indicator of TP53 mutations in these tumors.
Other molecular events in the pathogenesis of CPT are not yet as well characterized. TP53 mutations are unlikely to be the only event in the pathogenesis of CPTs. Patients with multiple resections show progression of CPTs with a tendency to increasing degrees of malignancy [40], and CPTs may arise from teratomas [115], indicating an accumulation of events leading to the final phenotype. Several other pathways have been suggested to be operative in the biology of CPTs. In mice, over-expression of notch3 initiated the formation of CPTs [116]. Some evidence suggests alterations of notch signaling also occur in human CPT [116, 117].
By comparing gene expression profiles obtained from human CPP cells with that of nonneoplastic choroid plexus epithelial cells, the transcription factor TWIST1 was identified to be highly expressed in CPP and also promoted proliferation and invasion in vitro [118]. Amplification and activating mutations of tyrosine receptor signaling pathways play an important role in the biology of human cancer. In CPC, amplification and over-expression of PDGF receptors has been described [119]. Furthermore, in immortalized choroid plexus epithelial cells, PDGF-BB exhibited a time- and dose-dependent proliferative response, which was significantly attenuated by the tyrosine-kinase inhibitor imatinib [120], providing a rationale for the development of treatments targeting PDGF receptor signaling in CPT.
The role of epigenetic alterations in CPT is also poorly understood. In pediatric brain tumors, human telomerase reverse transcriptase (hTERT) promoter methylation has been shown to be associated with tumor progression and poor prognosis. Methylation of the hTERT promoter has also been reported in the majority of CPCs [121]. The clinical utility of these findings for CPCs remain to be elucidated. Similarly, the prognostic and predictive role of MGMT promoter methylation, which occurs frequently in CPTs [122], remains to be determined.
Clinical Aspects and Treatments
Prognosis and Current Treatment
Due to the low incidence of CPTs, few randomized trials have been conducted [123, 124]. Most data come from individual case reports [50, 125], case series [23, 58, 126–129], or systematic literature reviews [130–133]. These data suggest that histological classification appears to be the most reliable prognostic parameter [134, 135]. Patients with CPPs have long-term survival rates exceeding 95 % when completely resected. In contrast, CPCs in patients treated with surgical resection and radiation therapy have 5-year survival rates of approximately 60 %. Primary and metastatic CPC in infants from Li–Fraumeni families fare even worse, with 5-year survival rates of less than 5 % [130].
Tumor resection is of very high therapeutic value in CPTs [28, 135–141]. In particular, gross total resection was found to be of significant prognostic value in meta-analyses, [130, 134, 141] thereby confirming the institutional experiences of many groups [23, 124, 142]. Meta analyses also confirmed the value of a second resection [143]. However, attempts at radical resection should be made with caution, since the high vascularity of these tumors also translates into a high rate of intratumoral bleeding [136], and other surgical complications such as tension pneumoventricle [144] and hyperacute disseminated intravascular coagulation [145]. Newer surgical techniques might reduce morbidity and mortality. These include endoscopic [137] and combined endoscopic and microsurgical approaches [146]. For tumors of the foramen of Luschka, a telovelar approach has been proposed [147]. Preoperative embolization may reduce the operative risk [6, 50, 148, 149]. In one case the tumor regressed after embolization without the need for resection [150]. Similarly, preoperative intensive chemotherapy may reduce the risk for intraoperative hemorrhage even when the size of the tumor does not shrink significantly [151].
Radiation therapy can increase survival of CPTs in patients old enough to receive therapeutic doses [129, 130, 134, 152, 153]. For example, CPPs may be sensitive to radiation therapy [39] and CPCs may show a survival benefit with craniospinal irradiation [152]. However, long-term sequelae of radiation are particular devastating for the developing brains of young children and limit the use of this modality.
Chemotherapy improved survival rates at least in the subgroup of incompletely resected CPC [42, 132]. The five most frequently used drugs are cisplatin, vincristine, cyclophosphamide, carboplatin, and etoposide. Of those, etoposide (VP16) was most frequently used in protocols and had the most convincing survival benefit in various multivariate analyses [133]. More recently reports suggest temozolomide is less promising [125]. In a prospective clinical trial, CPT-SIOP-2000, cyclophosphamide was found equally effective to carboplatin. As a result of these experiences, the benefit of chemotherapy (including high-dose chemotherapy recently reported for an adult patient [154] is becoming more widely accepted, at least for young children [135, 153, 155, 156]. However, intensive chemotherapy is associated with its own risks, and fatal complications have been described [136]. These studies highlight the need for better targeted and less toxic agents.
The influence of germline TP53 mutations on prognosis and efficacy of treatment remains controversial. A large series of patients treated mainly with intensive chemotherapy including ifosfamide etoposide carboplatin (ICE) show a significantly worse prognosis in patients with Li–Fraumeni syndrome [110]. A second report of patients treated with various chemotherapeutic protocols, among them head start III, described long-term survivors among the Li–Fraumeni population [157]. However, data from the Brazilian family with TP53-R337H mutations failed to show statistically significant differences in survival on treatment [111]. Finally, in the international CPT study, there was no significant difference between Li–Fraumeni and non-Li–Fraumeni families. These differences in results prompt further prospective evaluations.
Unique biological features of CPTs may provide leads to novel therapeutic approaches. The blood–brain barrier is located typically in the vascular wall and is characterized by tight junctions between endothelial cells. In contrast, choroid plexus capillaries are leaky. As this feature of leaky blood vessels is maintained in CPTs, systemic medication may reach tumor cells even among the most differentiated CPTs without hindrance from the blood–brain barrier. The normal choroid plexus also functions as an immunological gate to the CNS, including interferon-γ signal mediated entry of circulating leucocytes for immune surveillance [158], and IL-6 production [159]. It is possible that these immune pathways could be leveraged to develop novel therapeutic approaches against CPTs in the future.
Summary
CPTs are tumors arising from the choroid plexus and based on histologic criteria are classified as CPP, APP, and CPC, which correspond to WHO grades I, II, and III, respectively. CPTs occur in all ages but are more common in childhood, peaking in incidence during the first decade of life. Histological grading remains a key prognostic factor and several ancillary immunohistochemical tests can aid in establishing their diagnoses. CPPs are usually treated with surgical resection, whereas a combination of surgical resection and/or chemo/radiation therapy may be used for higher-grade tumors. The biology of CPTs is poorly understood. Factors implicated in the pathogenesis of CPTs include alterations in p53- and SV40-induced viral transformation. However, the molecular genetics of CPT initiation and progression have not been otherwise elucidated and should provide fruitful avenues for future research.
References
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol. 2007;114:97–109.
Jeibmann A, Hasselblatt M, Gerss J, Wrede B, Egensperger R, Beschorner R, Hans VH, Rickert CH, Wolff JE, Paulus W. Prognostic implications of atypical histologic features in choroid plexus papilloma. J Neuropathol Exp Neurol. 2006;65:1069–73.
Zulch, ed. Brain tumors: their biology and pathology. New York: Springer; 1957.
Stagno V, Mugamba J, Ssenyonga P, Kaaya BN, Warf BC. Presentation, pathology, and treatment outcome of brain tumors in 172 consecutive children at CURE Children’s hospital of Uganda. The predominance of the visible diagnosis and the uncertainties of epidemiology in sub-Saharan Africa. Childs Nerv Syst. 2014;30:137–46.
Wilhelm M, Hirsch W, Merkenschlager A, Stepan H, Geyer C, Kiess W. A rare case of congenital choroid plexus carcinoma. Pediatr Hematol Oncol. 2012;29:643–6.
Ditz C, Nowak G, Koch C, Merz H, Tronnier V. Atypical choroid plexus papilloma in a newborn: prenatal diagnosis, preoperative tumor embolization, and resection. J Neurol Surg A Cent Eur Neurosurg. 2013;74:59–63.
Jusue-Torres I, Ortega-Zufiria JM, Tamarit-Degenhardt M, Poveda-Nunez PD. Atypical choroid plexus papilloma in adults: case report and literature review. Neurocirugia. 2012;23:116–21.
Kishore S, Negi G, Meena H, Anuradha K, Pathak PV, Bansal K. Choroid plexus carcinoma in an adult. J Neurosci Rural Pract. 2012;3:71–3.
Umredkar AA, Chhabra R, Bal A, Das A. Choroid plexus papilloma of the fourth ventricle in a septuagenarian. J Neurosci Rural Pract. 2012;3:402–4.
Kurtz HJ, Hanlon GF. Choroid plexus papilloma in a dog. Vet Pathol. 1971;8:91–5.
Ribas JL, Mena H, Braund KG, Sesterhenn IA, Toivio-Kinnucan M. A histologic and immunocytochemical study of choroid plexus tumors of the dog. Vet Pathol. 1989;26:55–64.
Steiss JE, Cox NR, Knecht CD. Electroencephalographic and histopathologic correlations in eight dogs with intracranial mass lesions. Am J Vet Res. 1990;51:1286–91.
Ohashi F, Kotani T, Onishi T, Katamoto H, Nakata E, Fritz-Zieroth B. Magnetic resonance imaging in a dog with choroid plexus carcinoma. J Vet Med Sci. 1993;55:875–6.
Pastorello A, Constantino-Casas F, Archer J. Choroid plexus carcinoma cells in the cerebrospinal fluid of a Staffordshire Bull Terrier. Vet Clin Pathol. 2010;39:505–10.
Kinsler VA, Aylett SE, Coley SC, Chong WK, Atherton DJ. Central nervous system imaging and congenital melanocytic naevi. Arch Dis Child. 2001;84:152–5.
Vadivelu S, Edelman M, Schneider SJ, Mittler MA. Choroid plexus papilloma and Pierpont syndrome. J Neurosurg Pediatr. 2013;11:115–8.
Morigaki R, Pooh KH, Shouno K, Taniguchi H, Endo S, Nakagawa Y. Choroid plexus papilloma in a girl with hypomelanosis of Ito. J Neurosurg Pediatr. 2012;10:182–5.
Frye RE, Polling JS, Ma LC. Choroid plexus papilloma expansion over 7 years in Aicardi syndrome. J Child Neurol. 2007;22:484–7.
Blamires TL, Maher ER. Choroid plexus papilloma. A new presentation of von Hippel-Lindau (VHL) disease. Eye (Lond). 1992;6(Pt 1):90–2.
Bleggi-Torres LF, Urban LA, Antoniuk A, Carboni P, Ramina R, Gugelmin ES. Choroid plexus carcinoma: report of 15 cases. Arq Neuropsiquiatr. 2000;58:505–11.
Jaiswal S, Vij M, Mehrotra A, Kumar B, Nair A, Jaiswal AK, Behari S, Jain VK. Choroid plexus tumors: a clinico-pathological and neuro-radiological study of 23 cases. Asian J Neurosurg. 2013;8:29–35.
Mishra A, Srivastava C, Singh SK, Chandra A, Ojha BK. Choroid plexus carcinoma: case report and review of literature. J Pediatr Neurosci. 2012;7:71–3.
Pendleton C, Olivi A, Jallo GI, Quinones-Hinojosa A. Unique challenges faced by pediatric neurosurgeon Harvey Cushing in 1909 at Johns Hopkins: a choroid plexus tumor of the lateral ventricle mimicking a cerebellar lesion. Childs Nerv Syst. 2011;27:1145–8.
Phi JH, Shin CH, Wang KC, Park SH, Kim SK. Catastrophic electrolyte imbalance caused by excessive production and overdrainage of cerebrospinal fluid in an infant with choroid plexus papilloma. Childs Nerv Syst. 2011;27:1153–6.
Maimone G, Ganau M, Nicassio N, Paterniti S. Paratrigonal choroid plexus papilloma presenting with satellite multiple supra- and infratentorial hemorrhages. Neuroanatomical basis and pathological hypothesis. Int J Surg Case Rep. 2013;4:239–42.
Kinoshita Y, Wasita B, Akatsuka K, Kambe A, Kurosaki M, Watanabe T. Choroid plexus papilloma presenting with cerebrospinal fluid rhinorrhea and otorrhea: case report. Neurol Med Chir (Tokyo). 2010;50:930–3.
Jia DZ, Zhou MD, Jiang YQ, Li G. Trigeminal neuralgia caused by a choroid plexus papilloma of the cerebellopontine angle: case report and review of the literature. J Int Med Res. 2010;38:289–92.
Ogiwara H, Dipatri Jr AJ, Alden TD, Bowman RM, Tomita T. Choroid plexus tumors in pediatric patients. Br J Neurosurg. 2012;26:32–7.
Mahta A, Kim RY, Kesari S. Fourth ventricular choroid plexus papilloma. Med Oncol. 2012;29:1285–6.
Khoddami M, Gholampour Shahaboddini R. Choroid plexus papilloma of the cerebellopontine angle. Arch Iran Med. 2010;13:552–5.
Ma YH, Ye K, Zhan RY, Wang LJ. Primary choroid plexus papilloma of the sellar region. J Neurooncol. 2008;88:51–5.
Bian LG, Sun QF, Wu HC, Jiang H, Sun YH, Shen JK. Primary choroid plexus papilloma in the pituitary fossa: case report and literature review. Acta Neurochir (Wien). 2011;153:851–7.
Imai M, Tominaga J, Matsumae M. Choroid plexus papilloma originating from the cerebrum parenchyma. Surg Neurol Int. 2011;2:151.
Xiao A, Xu J, He X, You C. Extraventricular choroid plexus papilloma in the brainstem. J Neurosurg Pediatr. 2013;12:247–50.
Leys D, Pasquier F, Lejeune JP, Lesoin F, Petit H, Delandsheer JM. Benign choroid plexus papilloma. 2 local recurrences and intraventricular seeding. Neurochirurgie. 1986;32:258–61.
Domingues RC, Taveras JM, Reimer P, Rosen BR. Foramen magnum choroid plexus papilloma with drop metastases to the lumbar spine. AJNR Am J Neuroradiol. 1991;12:564–5.
Enomoto H, Mizuno M, Katsumata T, Doi T. Intracranial metastasis of a choroid plexus papilloma originating in the cerebellopontine angle region: a case report. Surg Neurol. 1991;36:54–8.
McEvoy AW, Galloway M, Revesz T, Kitchen ND. Metastatic choroid plexus papilloma: a case report. J Neurooncol. 2002;56:241–6.
Zachary G, George J, Jaishri B, Peter B, Stephanie T. Management of disseminated choroid plexus papilloma: a case study. Pediatr Blood Cancer. 2014;61:562–3.
Stuivenvolt M, Mandl E, Verheul J, Fleischeuer R, Tijssen CC. Atypical transformation in sacral drop metastasis from posterior fossa choroid plexus papilloma. BMJ Case Rep. 2012;2012.
Al-Abdullah AA, Abu-Amero KK, Hellani A, Alkhalidi H, Bosley TM. Choroid plexus papilloma metastases to both cerebellopontine angles mimicking neurofibromatosis type 2. J Neurol. 2011;258:504–6.
Menon G, Nair SN, Baldawa SS, Rao RB, Krishnakumar KP, Gopalakrishnan CV. Choroid plexus tumors: an institutional series of 25 patients. Neurol India. 2010;58:429–35.
Savage NM, Crosby JH, Reid-Nicholson MD. The cytologic findings in choroid plexus carcinoma: report of a case with differential diagnosis. Diagn Cytopathol. 2012;40(1):1–6.
Allen J, Wisoff J, Helson L, Pearce J, Arenson E. Choroid plexus carcinoma—responses to chemotherapy alone in newly diagnosed young children. J Neurooncol. 1992;12:69–74.
Geerts Y, Gabreels F, Lippens R, Merx H, Wesseling P. Choroid plexus carcinoma: a report of two cases and review of the literature. Neuropediatrics. 1996;27:143–8.
Hayakawa I, Fujiwara K, Tsuchida T, Aoki M. Choroid plexus carcinoma with metastasis to bone (author’s transl). No Shinkei Geka. 1979;7:815–8.
Valladares JB, Perry RH, Kalbag RM. Malignant choroid plexus papilloma with extraneural metastasis. Case report. J Neurosurg. 1980;52:251–5.
Sheridan M, Besser M. Fatal pulmonary embolism by tumor during resection of a choroid plexus papilloma: case report. Neurosurgery. 1994;34:910–2. discussion 912.
McCallum S, Cooper K, Franks DN. Choroid plexus carcinoma. Cytologic identification of malignant cells in ascitic fluid. Acta Cytol. 1988;32:263–6.
Hartge DR, Axt-Fliedner R, Weichert J. Prenatal diagnosis and successful postnatal therapy of an atypical choroid plexus papilloma-Case report and review of literature. J Clin Ultrasound. 2010;38:377–83.
Anselem O, Mezzetta L, Grange G, Zerah M, Benard C, Marcou V, Fallet-Bianco C, Adamsbaum C, Tsatsaris V. Fetal tumors of the choroid plexus: is differential diagnosis between papilloma and carcinoma possible? Ultrasound Obstet Gynecol. 2011;38:229–32.
Vassallo M, Maruotti GM, Quarantelli M, Pastore G, Paladini D. Choroid plexus carcinoma: prenatal characterization by 3-dimensional sonography and magnetic resonance imaging, perinatal management, and natural history. J Ultrasound Med. 2012;31:337–9.
Renna MD, Pisani P, Conversano F, Perrone E, Casciaro E, Renzo GC, Paola MD, Perrone A, Casciaro S. Sonographic markers for early diagnosis of fetal malformations. World J Radiol. 2013;5:356–71.
Lysyy O, Puzhevsky A, Strauss S. Choroid plexus papilloma in an infant: ultrasound diagnosis. Eur J Pediatr. 2012;171:1717–8.
Zhang TJ, Yue Q, Lui S, Wu QZ, Gong QY. MRI findings of choroid plexus tumors in the cerebellum. Clin Imaging. 2011;35:64–7.
Yan C, Xu Y, Feng J, Sun C, Zhang G, Shi J, Hao P, Wu Y, Lin B. Choroid plexus tumours: classification, MR imaging findings and pathological correlation. J Med Imaging Radiat Oncol. 2013;57:176–83.
Vandesteen L, Drier A, Galanaud D, Clarencon F, Leclercq D, Karachi C, Dormont D. Imaging findings of intraventricular and ependymal lesions. J Neuroradiol. 2013;40:229–44.
Wolff JE, Myles T, Pinto A, Rigel JE, Angyalfi S, Kloiber R. Detection of choroid plexus carcinoma with Tc-99m sestamibi: case report and review of the literature. Med Pediatr Oncol. 2001;36:323–5.
Kirton A, Kloiber R, Rigel J, Wolff J. Evaluation of pediatric CNS malignancies with (99m)Tc-methoxyisobutylisonitrile SPECT. J Nucl Med. 2002;43:1438–43.
Subbiah V, Ketonen L, Bruner JM, Nunez R, Weinberg J, Wolff JE. 99mTc-sestamibi scan differentiates tumor from other contrast enhancing tissue in choroid plexus tumors. J Pediatr Hematol Oncol. 2010;32:160–2.
Finnema SJ, Stepanov V, Ettrup A, Nakao R, Amini N, Svedberg M, Lehmann C, Hansen M, Knudsen GM, Halldin C. Characterization of [C]Cimbi-36 as an agonist PET radioligand for the 5-HT and 5-HT receptors in the nonhuman primate brain. Neuroimage. 2013;84C:342–53.
Korchi AM, Garibotto V, Ansari M, Merlini L. Pseudoprogression after proton beam irradiation for a choroid plexus carcinoma in pediatric patient: MRI and PET imaging patterns. Childs Nerv Syst. 2013;29:509–12.
Wieczorek V, Kluge H, Linke E, Zimmermann K, Kuehn H-J, Witte O, Isenmann S Pathological CSF cell findings in primary and metastatic CNS tumor, malignant lymphoma and leukemia. In: Atlas of CSF Cytology; 2007, p 79.
Berger P. Smears and frozen sections in surgical neuropathology: a manual. Baltimore: PB Medical Publishing; 2009.
Muthuphei MN. Divergent differentiation in choroid plexus papilloma. An immunohistochemical study of five cases. Cent Afr J Med. 1995;41:103–4.
Megerian CA, Pilch BZ, Bhan AK, McKenna MJ. Differential expression of transthyretin in papillary tumors of the endolymphatic sac and choroid plexus. Laryngoscope. 1997;107:216–21.
Hayashi H, Aoki M, Tsugu H, Hirakawa K, Yoshino S, Fukushima T, Inoue T, Nabeshima K. A case of choroid plexus papilloma with stromal sclerosis and indistinct papillary structures. Brain Tumor Pathol. 2012;29:37–42.
Hasselblatt M, Bohm C, Tatenhorst L, Dinh V, Newrzella D, Keyvani K, Jeibmann A, Buerger H, Rickert CH, Paulus W. Identification of novel diagnostic markers for choroid plexus tumors: a microarray-based approach. Am J Surg Pathol. 2006;30:66–74.
Vajtai I, Varga Z, Bodosi M, Voros E. Melanotic papilloma of the choroid plexus: report of a case with implications for pathogenesis. Noshuyo Byori. 1995;12:151–4.
Rorke LB, Packer R, Biegel J. Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood. J Neurooncol. 1995;24:21–8.
Burger PC, Yu IT, Tihan T, Friedman HS, Strother DR, Kepner JL, Duffner PK, Kun LE, Perlman EJ. Atypical teratoid/rhabdoid tumor of the central nervous system: a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma: a Pediatric Oncology Group study. Am J Surg Pathol. 1998;22:1083–92.
Judkins AR, Mauger J, Ht A, Rorke LB, Biegel JA. Immunohistochemical analysis of hSNF5/INI1 in pediatric CNS neoplasms. Am J Surg Pathol. 2004;28:644–50.
Judkins AR, Burger PC, Hamilton RL, Kleinschmidt-DeMasters B, Perry A, Pomeroy SL, Rosenblum MK, Yachnis AT, Zhou H, Rorke LB, Biegel JA. INI1 protein expression distinguishes atypical teratoid/rhabdoid tumor from choroid plexus carcinoma. J Neuropathol Exp Neurol. 2005;64:391–7.
Judkins AR, Eberhart CG, Wesseling P Atypical teratoid/rhabdoid tumor. WHO classification of tumors of the central nervous system. 2007; 4:147–149.
Tripathy K, Misra A, Misra D, Pujari S, Nayak M, Rath J. Melanotic choroid plexus carcinoma of the posterior fossa. J Clin Neurol. 2011;7:105–6.
Ghezali LA, Arbabian A, Jeibmann A, Hasselblatt M, Hallaert GG, Van den Broecke C, Gray F, Brouland JP, Varin-Blank N, Papp B (2013) Loss of endoplasmic reticulum calcium pump expression in choroid plexus tumours. Neuropathol Appl Neurobiol. 2013; [Epub ahead of print].
Schittenhelm J, Nagel C, Meyermann R, Beschorner R. Atypical teratoid/rhabdoid tumors may show morphological and immunohistochemical features seen in choroid plexus tumors. Neuropathology. 2011;31:461–7.
Hasselblatt M, Blumcke I, Jeibmann A, Rickert CH, Jouvet A, van de Nes JA, Kuchelmeister K, Brunn A, Fevre-Montange M, Paulus W. Immunohistochemical profile and chromosomal imbalances in papillary tumours of the pineal region. Neuropathol Appl Neurobiol. 2006;32:278–83.
Ibrahim GM, Huang A, Halliday W, Dirks PB, Malkin D, Baskin B, Shago M, Hawkins C. Cribriform neuroepithelial tumour: novel clinicopathological, ultrastructural and cytogenetic findings. Acta Neuropathol. 2011;122:511–4.
Korshunov A Sturm D, Ryzhova M, et al. Embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma, and medulloepithelioma share molecular similarity and comprise a single clinicopathological entity. Acta Neuropathol. 2013; [Epub ahead of print].
Hasselblatt M, Oyen F, Gesk S, Kordes U, Wrede B, Bergmann M, Schmid H, Fruhwald MC, Schneppenheim R, Siebert R, Paulus W. Cribriform neuroepithelial tumor (CRINET): a nonrhabdoid ventricular tumor with INI1 loss and relatively favorable prognosis. J Neuropathol Exp Neurol. 2009;68:1249–55.
Bollo RJ, Zagzag D, Samadani U. Synchronous choroid plexus papilloma of the fourth ventricle and ependymoma of the filum terminale: case report. Neurosurgery. 2010;67:E1454–9; discussion E1459.
Hayashi Y, Mohri M, Nakada M, Hamada J. Ependymoma and choroid plexus papilloma as synchronous multiple neuroepithelial tumors in the same patient: a case report and review of literature. Neurosurgery. 2011;68:E1144–7; discussion E1147.
Schittenhelm J, Roser F, Tatagiba M, Beschorner R. Diagnostic value of EAAT-1 and Kir7.1 for distinguishing endolymphatic sac tumors from choroid plexus tumors. Am J Clin Pathol. 2012;138:85–9.
Siomin V, Lin JL, Marko NF, Barnett GH, Toms SA, Chao ST, Angelov L, Vogelbaum MA, Navaratne K, Suh JH, Weil RJ. Stereotactic radiosurgical treatment of brain metastases to the choroid plexus. Int J Radiat Oncol Biol Phys. 2011;80:1134–42.
Kitagawa Y, Higuchi F, Abe Y, Matsuda H, Kim P, Ueki K. Metastasis to the choroid plexus from thyroid cancer: case report. Neurol Med Chir (Tokyo). 2013;53(11):832–6.
Manzil FF, Bender LW, Scott JW. Evaluation of rare choroid plexus metastasis from papillary thyroid carcinoma with multimodality imaging. Clin Nucl Med. 2014;39(6):551–3.
Kurisu K, Kamoshima Y, Terasaka S, Kobayashi H, Kubota K, Houkin K. A case of metastatic choroid plexus tumor from cholangiocellular carcinoma. No Shinkei Geka. 2011;39:991–7.
Shein HM, Enders JF, Levinthal JD. Transformation induced by simian virus 40 in human renal cell cultures. II. Cell-virus relationships. Proc Natl Acad Sci U S A. 1962;48:1350–7.
Carruba G, Dallapiccola B, Brinchi V, de Giuli MC. Ultrastructural and biological characterization of human choroid cell cultures transformed by Simian Virus 40. In Vitro. 1983;19:443–52.
Carruba G, Dallapiccola B, Mantegazza P, Garaci E, Micara G, Radaelli A, De Giuli MC. Transformation of human choroid cells in vitro by SV40. Ultrastructural and cytogenetic analysis of cloned cell lines. J Submicrosc Cytol. 1984;16:459–70.
Kirschstein RL, Gerber P. Ependymomas produced after intracerebral inoculation of SV40 into new-born hamsters. Nature. 1962;195:299–300.
Davis LE, Nager GT, Johnson RT. Experimental viral infections of the inner ear. II. Simian virus 40 induced tumors of the temporal bone. Ann Otol Rhinol Laryngol. 1979;88:198–204.
Brinster RL, Chen HY, Messing A, van Dyke T, Levine AJ, Palmiter RD. Transgenic mice harboring SV40 T-antigen genes develop characteristic brain tumors. Cell. 1984;37:367–79.
Small JA, Blair DG, Showalter SD, Scangos GA. Analysis of a transgenic mouse containing simian virus 40 and v-myc sequences. Mol Cell Biol. 1985;5:642–8.
Reynolds RK, Hoekzema GS, Vogel J, Hinrichs SH, Jay G. Multiple endocrine neoplasia induced by the promiscuous expression of a viral oncogene. Proc Natl Acad Sci U S A. 1988;85:3135–9.
Enjoji M, Iwaki T, Hara H, Sakai H, Nawata H, Watanabe T. Establishment and characterization of choroid plexus carcinoma cell lines: connection between choroid plexus and immune systems. Jpn J Cancer Res. 1996;87:893–9.
Cho HJ, Seiberg M, Georgoff I, Teresky AK, Marks JR, Levine AJ. Impact of the genetic background of transgenic mice upon the formation and timing of choroid plexus papillomas. J Neurosci Res. 1989;24:115–22.
Chen J, Tobin GJ, Pipas JM, Van Dyke T. T-antigen mutant activities in vivo: roles of p53 and pRB binding in tumorigenesis of the choroid plexus. Oncogene. 1992;7:1167–75.
Tabuchi K, Kirsch WM, Low M, Gaskin D, Van Buskirk J, Maa S. Screening of human brain tumors for SV40-related T antigen. Int J Cancer. 1978;21:12–7.
Bergsagel DJ, Finegold MJ, Butel JS, Kupsky WJ, Garcea RL. DNA sequences similar to those of simian virus 40 in ependymomas and choroid plexus tumors of childhood. N Engl J Med. 1992;326:988–93.
Lednicky JA, Garcea RL, Bergsagel DJ, Butel JS. Natural simian virus 40 strains are present in human choroid plexus and ependymoma tumors. Virology. 1995;212:710–7.
Martini F, Iaccheri L, Lazzarin L, Carinci P, Corallini A, Gerosa M, Iuzzolino P, Barbanti-Brodano G, Tognon M. SV40 early region and large T antigen in human brain tumors, peripheral blood cells, and sperm fluids from healthy individuals. Cancer Res. 1996;56:4820–5.
Palmiter RD, Chen HY, Messing A, Brinster RL. SV40 enhancer and large-T antigen are instrumental in development of choroid plexus tumours in transgenic mice. Nature. 1985;316:457–60.
Rickert CH, Wiestler OD, Paulus W. Chromosomal imbalances in choroid plexus tumors. Am J Pathol. 2002;160:1105–13.
Ruland V, Hartung S, Kordes U, Wolff JE, Paulus W, Hasselblatt M. Choroid plexus carcinomas are characterized by complex chromosomal alterations related to patient age and prognosis. Genes Chromosomes Cancer. 2014;53(5):373–80.
Garber JE, Burke EM, Lavally BL, Billett AL, Sallan SE, Scott RM, Kupsky W, Li FP. Choroid plexus tumors in the breast cancer-sarcoma syndrome. Cancer. 1990;66:2658–60.
Yuasa H, Tokito S, Tokunaga M. Primary carcinoma of the choroid plexus in Li-Fraumeni syndrome: case report. Neurosurgery. 1993;32:131–3; discussion 133–134.
Kleihues P, Schauble B, zur Hausen A, Esteve J, Ohgaki H. Tumors associated with p53 germline mutations: a synopsis of 91 families. Am J Pathol. 1997;150:1–13.
Tabori U, Shlien A, Baskin B, Levitt S, Ray P, Alon N, Hawkins C, Bouffet E, Pienkowska M, Lafay-Cousin L, Gozali A, Zhukova N, Shane L, Gonzalez I, Finlay J, Malkin D. TP53 alterations determine clinical subgroups and survival of patients with choroid plexus tumors. J Clin Oncol. 2010;28:1995–2001.
Custodio G, Taques GR, Figueiredo BC, Gugelmin ES, Oliveira Figueiredo MM, Watanabe F, Pontarolo R, Lalli E, Torres LF. Increased incidence of choroid plexus carcinoma due to the germline TP53 R337H mutation in southern Brazil. PLoS One. 2011;6:e18015.
Seidinger AL, Mastellaro MJ, Paschoal Fortes F, Godoy Assumpcao J, Aparecida Cardinalli I, Aparecida Ganazza M, Correa Ribeiro R, Brandalise SR, Dos Santos AS, Yunes JA. Association of the highly prevalent TP53 R337H mutation with pediatric choroid plexus carcinoma and osteosarcoma in southeast Brazil. Cancer. 2011;117:2228–35.
Giacomazzi J, Selistre SG, Rossi C, Alemar B, Santos-Silva P, Pereira FS, Netto CB, Cossio SL, Roth DE, Brunetto AL, Zagonel-Oliveira M, Martel-Planche G, Goldim JR, Hainaut P, Camey SA, Ashton-Prolla P. Li-Fraumeni and Li-Fraumeni-like syndrome among children diagnosed with pediatric cancer in Southern Brazil. Cancer. 2013;119(24):4341–9.
Lv SQ, Song YC, Xu JP, Shu HF, Zhou Z, An N, Huang QL, Yang H. A novel TP53 somatic mutation involved in the pathogenesis of pediatric choroid plexus carcinoma. Med Sci Monit. 2012;18:CS37–41.
Dessauvagie BF, Ruba S, Robbins PD. Choroid plexus papilloma arising in a mature cystic teratoma of a 32-year-old female. Pathology. 2013;45:88–9.
Dang L, Fan X, Chaudhry A, Wang M, Gaiano N, Eberhart CG. Notch3 signaling initiates choroid plexus tumor formation. Oncogene. 2006;25:487–91.
Beschorner R, Waidelich J, Trautmann K, Psaras T, Schittenhelm J. Notch receptors in human choroid plexus tumors. Histol Histopathol. 2013;28:1055–63.
Hasselblatt M, Mertsch S, Koos B, Riesmeier B, Stegemann H, Jeibmann A, Tomm M, Schmitz N, Wrede B, Wolff JE, Zheng W, Paulus W. TWIST-1 is overexpressed in neoplastic choroid plexus epithelial cells and promotes proliferation and invasion. Cancer Res. 2009;69:2219–23.
Nupponen NN, Paulsson J, Jeibmann A, Wrede B, Tanner M, Wolff JE, Paulus W, Ostman A, Hasselblatt M. Platelet-derived growth factor receptor expression and amplification in choroid plexus carcinomas. Mod Pathol. 2008;21:265–70.
Koos B, Paulsson J, Jarvius M, Sanchez BC, Wrede B, Mertsch S, Jeibmann A, Kruse A, Peters O, Wolff JE, Galla HJ, Soderberg O, Paulus W, Ostman A, Hasselblatt M. Platelet-derived growth factor receptor expression and activation in choroid plexus tumors. Am J Pathol. 2009;175:1631–7.
Castelo-Branco P, et al. Methylation of the TERT promoter and risk stratification of childhood brain tumours: an integrative genomic and molecular study. Lancet Oncol. 2013;14:534–42.
Hasselblatt M, Muhlisch J, Wrede B, Kallinger B, Jeibmann A, Peters O, Kutluk T, Wolff JE, Paulus W, Fruhwald MC. Aberrant MGMT (O6-methylguanine-DNA methyltransferase) promoter methylation in choroid plexus tumors. J Neurooncol. 2009;91:151–5.
Wolff J (2008) Chroid plexus tumoren. In: Korinthenberg + Ritter: Pädiatrische Hämatologie und Onkologie ISBN: 3-540-03702-0 2:2.
Wolff JE, Finlay JL. Choroid plexus tumors. In: Carroll WL, Finlay JL, editors. Cancer in children. Sudbury: Jones & Bartlett; 2009.
Misaki K, Nakada M, Mohri M, Hayashi Y, Hamada J. MGMT promoter methylation and temozolomide response in choroid plexus carcinoma. Brain Tumor Pathol. 2011;28:259–63.
Asai A, Hoffman HJ, Hendrick EB, Humphreys RP, Becker LE. Primary intracranial neoplasms in the first year of life. Childs Nerv Syst. 1989;5:230–3.
Packer RJ, Perilongo G, Johnson D, Sutton LN, Vezina G, Zimmerman RA, Ryan J, Reaman G, Schut L. Choroid plexus carcinoma of childhood. Cancer. 1992;69:580–5.
Berger C, Thiesse P, Lellouch-Tubiana A, Kalifa C, Pierre-Kahn A, Bouffet E. Choroid plexus carcinomas in childhood: clinical features and prognostic factors. Neurosurgery. 1998;42:470–5.
Sun MZ, Oh MC, Ivan ME, Kaur G, Safaee M, Kim JM, Phillips JJ, Auguste KI, Parsa AT. Current management of choroid plexus carcinomas. Neurosurg Rev. 2014;37(2):179–92. discussion 192.
Wolff JE, Sajedi M, Coppes MJ, Anderson RA, Egeler RM. Radiation therapy and survival in choroid plexus carcinoma. Lancet. 1999;353:2126.
Wolff JE, Gnekow AK, Kortmann RD, Pietsch T, Urban C, Graf N, Kuhl J. Preradiation chemotherapy for pediatric patients with high-grade glioma. Cancer. 2002;94:264–71.
Wrede B, Liu P, Wolff JE. Chemotherapy improves the survival of patients with choroid plexus carcinoma: a meta-analysis of individual cases with choroid plexus tumors. J Neurooncol. 2007;85:345–51.
Berrak SG, Liu DD, Wrede B, Wolff JE. Which therapy works better in choroid plexus carcinomas? J Neurooncol. 2011;103:155–62.
Wolff J. International choroid plexus tumor initiative. Med Ped Oncol. 2002;39:76.
Koh EJ, Wang KC, Phi JH, Lee JY, Choi JW, Park SH, Park KD, Kim IH, Cho BK, Kim SK. Clinical outcome of pediatric choroid plexus tumors: retrospective analysis from a single institute. Childs Nerv Syst. 2014;30(2):217–25.
Lafay-Cousin L, Keene D, Carret AS, Fryer C, Brossard J, Crooks B, Eisenstat D, Johnston D, Larouche V, Silva M, Wilson B, Zelcer S, Bartels U, Bouffet E. Choroid plexus tumors in children less than 36 months: the Canadian Pediatric Brain Tumor Consortium (CPBTC) experience. Childs Nerv Syst. 2011;27:259–64.
Meng H, Feng H, Zhang L, Wang J. Endoscopic removal of a cystic choroid plexus papilloma of the third ventricle: a case report and review of the literature. Clin Neurol Neurosurg. 2011;113:582–5.
Mizowaki T, Nagashima T, Yamamoto K, Kawamura A, Yoshida M, Kohmura E. Optimized surgical approach to third ventricular choroid plexus papillomas of young children based on anatomical variations. World Neurosurg. 2013; pii: S1878–8750(13)00458-0.
Safaee M, Oh MC, Sughrue ME, Delance AR, Bloch O, Sun M, Kaur G, Molinaro AM, Parsa AT. The relative patient benefit of gross total resection in adult choroid plexus papillomas. J Clin Neurosci. 2013;20:808–12.
Safaee M, Clark AJ, Bloch O, Oh MC, Singh A, Auguste KI, Gupta N, McDermott MW, Aghi MK, Berger MS, Parsa AT. Surgical outcomes in choroid plexus papillomas: an institutional experience. J Neurooncol. 2013;113:117–25.
Sun MZ, Ivan ME, Clark AJ, Oh MC, Delance AR, Oh T, Safaee M, Kaur G, Bloch O, Molinaro A, Gupta N, Parsa AT. Gross total resection improves overall survival in children with choroid plexus carcinoma. J Neurooncol. 2014;116(1):179–85.
Wrede B, Peters Ove Peter Thall, Hasselblatt Martin, Pietsch Torsten, Kortmann Rolf-D., Warmuth-Metz Monika, Mahajan Anita, Lucia Leskova, Xuemei Wang, Wolff Johannes EA (2009) CPT-SIOP-2000 study: Interim results January 2009. Turkish Journal of Cancer 2009: http://wwwturkjcancerorg/archivephp3
Wrede B, Liu P, Ater J, Wolff JE. Second surgery and the prognosis of choroid plexus carcinoma—results of a meta-analysis of individual cases. Anticancer Res. 2005;25:4429–33.
Goncalves MB, Nunes CF, Melo Jr JO, Guimaraes RD, Klescoski Jr J, Landeiro JA. Tension pneumoventricle after resection of a fourth ventricle choroid plexus papilloma: an unusual postoperative complication. Surg Neurol Int. 2012;3:116.
Moiyadi AV, Jalali R, Menon S. Hyperacute disseminated intravascular coagulation following surgery for a choroid plexus carcinoma in a child. Neurol India. 2010;58:485–6.
Reddy D, Gunnarsson T, Scheinemann K, Provias JP, Singh SK. Combined staged endoscopic and microsurgical approach of a third ventricular choroid plexus papilloma in an infant. Minim Invasive Neurosurg. 2011;54:264–7.
Lee CC, Lin CF, Yang TF, Hsu SP, Chen HH, Chen SC, Shih YH. Telovelar approach for choroid plexus papilloma in the foramen of Luschka: a safe way using a neuromonitor. Clin Neurol Neurosurg. 2012;114:249–53.
Trivelato FP, Manzato LB, Rezende MT, Barroso PM, Faleiro RM, Ulhoa AC. Preoperative embolization of choroid plexus papilloma with Onyx via the anterior choroidal artery: technical note. Childs Nerv Syst. 2012;28:1955–8.
Haliasos N, Brew S, Robertson F, Hayward R, Thompson D, Chakraborty A. Preoperative embolisation of choroid plexus tumours in children: part I-does the reduction of perioperative blood loss affect the safety of subsequent surgery? Childs Nerv Syst. 2013;29:65–70.
Wind JJ, Bell RS, Bank WO, Myseros JS. Treatment of third ventricular choroid plexus papilloma in an infant with embolization alone. J Neurosurg Pediatr. 2010;6:579–82.
Lafay-Cousin L, Mabbott DJ, Halliday W, Taylor MD, Tabori U, Kamaly-Asl ID, Kulkarni AV, Bartels U, Greenberg M, Bouffet E. Use of ifosfamide, carboplatin, and etoposide chemotherapy in choroid plexus carcinoma. J Neurosurg Pediatr. 2010;5:615–21.
Mazloom A, Wolff JE, Paulino AC. The impact of radiotherapy fields in the treatment of patients with choroid plexus carcinoma. Int J Radiat Oncol Biol Phys. 2010;78:79–84.
Bettegowda C, Adogwa O, Mehta V, Chaichana KL, Weingart J, Carson BS, Jallo GI, Ahn ES. Treatment of choroid plexus tumors: a 20-year single institutional experience. J Neurosurg Pediatr. 2012;10:398–405.
Samuel TA, Parikh J, Sharma S, Giller CA, Sterling K, Kapoor S, Pirkle C, Jillella A. Recurrent adult choroid plexus carcinoma treated with high-dose chemotherapy and syngeneic stem cell (bone marrow) transplant. J Neurol Surg A Cent Eur Neurosurg. 2013;74 Suppl 1:e149–54.
Addo NK, Kamaly-Asl ID, Josan VA, Kelsey AM, Estlin EJ. Preoperative vincristine for an inoperable choroid plexus papilloma: a case discussion and review of the literature. J Neurosurg Pediatr. 2011;8:149–53.
Mosleh O, Tabori U, Bartels U, Huang A, Schechter T, Bouffet E. Successful treatment of a recurrent choroid plexus carcinoma with surgery followed by high-dose chemotherapy and stem cell rescue. Pediatr Hematol Oncol. 2013;30:386–91.
Gozali AE, Britt B, Shane L, Gonzalez I, Gilles F, McComb JG, Krieger MD, Lavey RS, Shlien A, Villablanca JG, Erdreich-Epstein A, Dhall G, Jubran R, Tabori U, Malkin D, Finlay JL. Choroid plexus tumors; management, outcome, and association with the Li-Fraumeni syndrome: the Children’s Hospital Los Angeles (CHLA) experience, 1991–2010. Pediatr Blood Cancer. 2012;58:905–9.
Kunis G, Baruch K, Rosenzweig N, Kertser A, Miller O, Berkutzki T, Schwartz M. IFN-gamma-dependent activation of the brain’s choroid plexus for CNS immune surveillance and repair. Brain. 2013;136:3427–40.
Zhang X, Wu C, Song J, Gotte M, Sorokin L. Syndecan-1, a cell surface proteoglycan, negatively regulates initial leukocyte recruitment to the brain across the choroid plexus in murine experimental autoimmune encephalomyelitis. J Immunol. 2013;191:4551–61.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer Science+Business Media New York
About this chapter
Cite this chapter
Venneti, S., Hasselblatt, M., Wolff, J.E., Judkins, A.R. (2015). Choroid Plexus Tumors. In: Karajannis, M., Zagzag, D. (eds) Molecular Pathology of Nervous System Tumors. Molecular Pathology Library, vol 8. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-1830-0_12
Download citation
DOI: https://doi.org/10.1007/978-1-4939-1830-0_12
Published:
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-4939-1829-4
Online ISBN: 978-1-4939-1830-0
eBook Packages: MedicineMedicine (R0)