Pathology of Choroid Plexus Neoplasms

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Choroid plexus neoplasms are rare, intraventricular, primary central nervous system (CNS) tumors derived from choroid plexus epithelium that are seen predominantly in children. Choroid plexus tumors are graded based on the World Health Organization (WHO) classification scheme and include choroid plexus papilloma (CPP) (WHO grade I) (see the following image), atypical choroid plexus papilloma (WHO grade II), and choroid plexus carcinoma (CPC) (WHO grade III). ^[1]

The overall annual incidence of choroid plexus neoplasms for all ages is 0.3 cases per million. Although these tumors arise in all age groups, their peak incidence is within the mid-teenage years. ^{[2, 3]} In adults, they account for less than 1% of primary intracranial neoplasms, whereas choroid plexus tumors represent up to 5% of pediatric brain tumors, and up to 20% of those arising in children aged 1 year and younger. ^{[4, 5]} Occasional fetal and congenital examples have been presented.

Irrespective of patient age, choroid plexus papillomas outnumber choroid plexus carcinomas by a 5:1 ratio. Up to 90% of choroid plexus tumors in children are papillomas, and up to 70% of all choroid plexus papillomas occur in children younger than 2 years. Choroid plexus carcinomas are also far more common in the pediatric population, with approximately 80% of choroid plexus carcinomas occurring in children. There is no significant sex predilection. ^[1]

Although the vast majority of choroid plexus tumors are sporadic, hereditary factors appear to play a role in the development of some choroid plexus papillomas and carcinomas. Choroid plexus papillomas are a component of Aicardi syndrome, ^[6] and they may also arise in the context of Down syndrome, ^[7]  von Hippel-Lindau disease, ^[8]  and neurofibromatosis type 2. Choroid plexus carcinomas occasionally arise in association with hereditary cancer predisposition syndromes, including the Li-Fraumeni and rhabdoid predisposition syndromes, with germline mutations of TP53 and hSNF5/INI1/SMARCB1, respectively. ^{[9, 10, 11, 12]}

The vast majority of choroid plexus neoplasms arise within the ventricles. In children, the lateral ventricles are the most common location of involvement, followed by the fourth ventricle. In adults, this pattern of localization is reversed, with the fourth ventricle being the most common location, and the lateral ventricles second in frequency. The third ventricle is the least common intraventricular location for choroid plexus neoplasms, irrespective of patient age. ^[1]  Primary extraventricular papillomas are rare and most often occur at the cerebellopontine angle. ^{[13, 14]}  Occasionally, involvement of both lateral or multiple ventricles is encountered. ^[15]

Choroid plexus neoplasms can produce hydrocephalus and increased intracranial pressure by a number of mechanisms, including obstruction of normal cerebrospinal fluid (CSF) flow, overproduction of CSF by the tumor itself, local expansion of the ventricles, or spontaneous hemorrhage. From a clinical standpoint, signs and symptoms related to the above abnormalities would include headache, vomiting, papilledema, ataxia, strabismus, increased head circumference and bulging fontanelles (in infants), developmental delay, and altered mental status. ^[3]

The most frequent route of choroid plexus tumor spread is via seeding of the CSF. Drop metastases may occur throughout the neuroaxis as a result, particularly in the region of the cauda equina. Seeding of the CSF may be seen even in benign choroid plexus papillomas, but leptomeningeal dissemination is much more common in choroid plexus carcinomas. Shunt-related metastases have been reported at abdominal sites, with extracranial metastases to the lung and bone reported in rare cases. ^{[16, 17, 18]}

Typical radioimaging of a choroid plexus papilloma shows a lobulated, solid, well-demarcated intraventricular mass that is isodense to mildly hyperdense on computer tomography (CT) scans; these lesions enhance homogeneously following contrast administration. Choroid plexus papillomas appear homogeneous and isointense to gray matter on T1-weighted magnetic resonance images (MRIs), hyperintense on T2 images, and exhibit intense contrast enhancement (see the image below). More aggressive atypical choroid plexus papillomas may have an irregular margin with adjacent white matter edema. ^[19] (See also the Medscape Reference article Imaging in Choroid Plexus Papilloma.)

In contrast, choroid plexus carcinomas are usually larger than papillomas and display heterogeneous signal patterns on both CT scans and MRIs. Their enhancement characteristics are likewise more variable, and intratumoral calcifications, necrosis, and hemorrhage may be present. Cystic change and flow voids are features shared with choroid plexus papillomas. Unlike choroid plexus papillomas, however, carcinomas frequently invade the adjacent/periventricular brain parenchyma with associated vasogenic edema. Leptomeningeal enhancement correlates with cerebrospinal fluid (CSF) dissemination of the tumor (see the following image). ^{[16, 20]}

Gross inspection of choroid plexus neoplasms is available through autopsy or surgical retrieval of tumor material. Choroid plexus papillomas are generally well-circumscribed, globular, cauliflowerlike masses that frequently adhere to the ventricular wall but which do not invade into the brain parenchyma proper. Often, there is surface stippling that correlates with a papillary microscopic architecture. Their pink to red-brown coloration is due to extensive vascularity. Calcifications, cysts, and hemorrhages may be seen. ^[21] Choroid plexus carcinomas consist of a solid tissue mass with invasion into the periventricular brain parenchyma. Hemorrhage and necrosis are common.

The three World Health Organization (WHO) grades of choroid plexus neoplasms are discussed in this section. Cytopathology and ultrastructural studies of choroid plexus neoplasms are discussed below.

The fibrovascular papillary projections of choroid plexus papilloma are lined by cuboidal to columnar epithelium with significantly more cell crowding, elongation, and stratification in comparison to the orderly cobblestone appearance of normal choroid plexus tissue. They likewise often display a mildly elevated nuclear-to-cytoplasmic ratio, nuclear hyperchromasia and/or irregular nuclear profiles, and occasional mitoses. Nonetheless, the epithelial cells of choroid plexus papillomas are typically cytologically bland, and they may have eosinophilic or clear cytoplasm. ^[22]

On occasion, papillomas may show oncocytic change, melanization, cytoplasmic vacuolization reminiscent of signet ring cells, tubular-glandular architecture, focal ependymal differentiation, or neuropil-like islands. ^{[23, 24, 25]} Degenerative features may be quite prominent, including an angiomalike increase in blood vessels; hyalinization or calcification; xanthomatous or mucinous change; or formation of metaplastic bone, cartilage, or adipose tissue. ^{[25, 26, 27]}  Features such as brain invasion, hypercellularity, necrosis, overt nuclear pleomorphism, and loss of papillary architecture are generally absent. See examples in the following images.

Although they closely resemble grade I choroid plexus papillomas, the hallmark of atypical choroid plexus papillomas (WHO grade II) is their increased mitotic activity (defined as ≥2 mitoses per 10 high-power fields [HPF]) ^[1] (see the first image below). Cribriforming, anastomosing papillary formations, and focal solid growth patterns (see the second image below) may be encountered. Additional histologic features often present in atypical choroid plexus papillomas include hypercellularity, nuclear pleomorphism, and necrosis. ^{[1, 28]}

Although choroid plexus papillomas typically look similar, one to another, choroid plexus carcinomas (WHO grade III) are notable for their significant histologic heterogeneity and ability to mimic a wide variety of both primary brain tumors and metastatic lesions. Choroid plexus carcinomas show frank features of malignancy. According to the 2016 WHO criteria, they must harbor at least four of the five following characteristics: elevated mitotic activity (generally >5 mitoses per 10 HPF), hypercellularity, nuclear pleomorphism, solid growth with sheets of tumor cells, and necrosis. ^{[1, 29]} Papillary architecture may be retained focally in some choroid plexus carcinomas, although it may be completely absent in others. Extensive invasion into the surrounding brain parenchyma is a frequent finding.

Choroid plexus carcinomas may contain cells with a rhabdoid morphology similar to that seen in atypical teratoid/rhabdoid tumors, or small primitive-appearing cells resembling those in medulloblastomas and other central nervous system (CNS) embryonal tumors. ^{[22, 30, 31]} Unusual and degenerative histologic features akin to those noted above for choroid plexus papillomas (especially melanin pigment) may be encountered in occasional choroid plexus carcinomas. ^[32]

The following images illustrate different examples of choroid plexus carcinomas.

Choroid plexus papillomas may closely resemble normal choroid plexus or villous hypertrophy on cytology, except choroid plexus papillomas usually have more papillary clusters, sheets, and isolated single cells. The tumor cells tend to be cuboidal to columnar with a moderate amount of cytoplasm and bland nuclei with fine chromatin. Choroid plexus papillomas may also be difficult to distinguish from papillary ependymoma; papillary ependymomas have multilayered cell clusters with glial processes approaching a central blood vessel. ^[33]

In contrast, the cytologic features of choroid plexus carcinoma include tight three-dimensional clusters and isolated anaplastic cells, variably sized nuclei, a high nuclear-to-cytoplasmic ratio, nuclear indentations and lobulations, single or multiple micronucleoli, and scant, pale, granular cytoplasm. Papillary architecture tends to be minimal or absent, mitotic activity and hypercellularity are obvious, and necrosis is often present. ^[34] (see the following image).

When examined under electron microscopy, choroid plexus tumors are seen to be composed of epithelial cells with interdigitating cell membranes, apical tight junctions, bulbous microvilli, coated vesicles, and cytoplasmic intermediate filaments. A continuous basement membrane separates these cells from the nearby capillaries with their fenestrated endothelial cells. ^[35] Cilia are consistently present in choroid plexus papillomas, but they may have an abnormal arrangement; these are less frequent and more difficult to identify in choroid plexus carcinomas. ^[36]

Pigmented choroid plexus tumors may demonstrate either melanosomes in various stages of development or accumulation of neuromelanin on electron microscopy. ^[32]

Expression of pancytokeratin and vimentin is a consistent feature of choroid plexus papillomas, whereas staining for S100 protein, glial fibrillary acidic protein (GFAP), transthyretin, and synaptophysin is more variable (see the image below). ^{[22, 37, 38, 39, 40]} Ethidium monoazide (EMA) is usually negative, as is carcinoembryonic antigen (CEA). ^{[37, 38]}

Interestingly, expression patterns may vary by tumor location, patient age, and tumor grade. For example, S-100, transthyretin, and GFAP are all less likely to be positive in choroid plexus carcinomas compared with choroid plexus papillomas. S-100 expression tends to be greater in choroid plexus tumors arising in the fourth ventricle, whereas transthyretin expression by these tumors may be higher in patients older than 20 years. ^[37] Although any combination of positivity for CK7 and CK20 may be encountered, it is the limited focal staining of choroid plexus tumors with these markers that is useful in discriminating them from metastatic carcinomas. ^{[25, 41]}

Similar to choroid plexus papillomas, choroid plexus carcinomas are generally immunopositive for cytokeratin; however, s100 and transthyretin are less consistently positive. ^{[22, 37, 38, 40, 42]} Synaptophysin, GFAP, CA19-9, and EMA may all be focally expressed, whereas CEA staining is quite unusual. ^{[39, 43, 44]} MIB-1 (Ki67) proliferative index is typically brisk in choroid plexus carcinomas, ^[45] and the majority show nuclear positivity for p53. ^[46]

Several markers may be helpful in distinguishing choroid plexus neoplasms from other primary central nervous system and metastatic tumors. For example, membranous positivity for excitatory amino acid transporter-1 (EAAT-1) is present in a significant proportion of choroid plexus tumors, whereas EAAT-1 is typically negative in metastatic adenocarcinoma, urothelial and small cell carcinomas, and endolymphatic sac tumors (which may quite closely mimic choroid plexus tumors). ^{[47, 48]}

HEA-125 and Ber-EP4 are negative in most choroid plexus neoplasms, whereas these markers are positive in most metastatic carcinomas. ^[49]

Synaptophysin may also be useful in distinguishing choroid plexus neoplasms from metastatic papillary carcinomas, as synaptophysin is frequently demonstrable in choroid plexus neoplasms but lacking in the later. ^[39]

CA19-9 and CEA, which are positive in some metastatic carcinomas, are also occasionally positive in choroid plexus carcinomas. Although not useful in distinguishing choroid plexus neoplasms from metastatic carcinoma, both of these markers can be detected in peripheral blood and may be efficacious as follow-up tumor markers after treatment. ^{[42, 43, 50]}

The inwardly rectifying potassium channel Kir7.1 and stanniocalcin-1 have been found to be both sensitive and specific markers for choroid plexus neoplasms when compared to other primary brain tumors and metastases. ^{[40, 48]}

Laminin and collagen IV, however, highlight the basement membrane in almost all choroid plexus papillomas and can therefore effectively separate these lesions from papillary ependymomas, which lack such basement membranes. Basement membrane stains may not be quite as useful in choroid plexus carcinomas in which fragmentation of the basement membrane is quite common. ^[51]

E-cadherin is positive in many choroid plexus tumors and may be useful in distinguishing them from ependymomas, as ependymomas are typically negative. ^[52] Podoplanin is positive in almost all choroid plexus tumors, but it is also frequently positive in ependymomas and some other primary brain tumors. ^[53] Lastly, nuclear INI-1 (BAF-47) reactivity is retained in choroid plexus carcinomas, but this finding is absent in atypical teratoid/rhabdoid tumors (AT/RTs), aiding in differentiation between these two neoplasms. ^[54]

See the image below.

Choroid plexus papillomas and atypical papillomas are frequently hyperdiploid, whereas choroid plexus carcinomas tend to mainly harbor chromosomal losses. ^[55]  Comparative genomic hybridization and more recent high-resolution genomic and methylation profiling studies not only confirm genetic differences between choroid plexus papillomas and choroid plexus carcinomas, but they likewise suggest that there are genetic distinctions between adult and pediatric choroid plexus tumors. ^{[55, 56, 57]}  In addition to recurrent gains common to all choroid plexus neoplasms (including gains in regions harboring OTX2, LAMB1, and TRPM3), focal alterations shared by papillomas and atypical papillomas have been detected, as well as separate and distinct carcinoma-specific alterations. ^{[55, 57]}  

With regard to specific genetic alterations, VHL allele loss may be seen in those choroid plexus papillomas arising in the context of von Hippel-Lindau disease. ^[8]  Activation of the Notch signaling pathway may also play a role in the formation of choroid plexus papillomas, with nuclear translocation or overexpression of one or more Notch receptors (Notch 1, 2, and 3) identified in some choroid plexus papillomas. ^[57] Chromosome 22q loss and INI1 alterations have been described for some choroid plexus carcinomas; nonetheless, immunohistochemical studies have indicated that INI1 protein expression remains intact in choroid plexus carcinomas, including those with a “rhabdoid morphology.” ^[54] Lastly, platelet-derived growth factor (PDGF) receptors may be overexpressed or amplified in some choroid plexus carcinomas. PDGF receptor signaling has been suggested as a potential therapeutic target. ^[58]

Choroid plexus tumors need to be distinguished from a wide variety of lesions, both neoplastic and non-neoplastic. In the realm of non-neoplastic lesions, choroid plexus papillomas must be distinguished from both normal choroid plexus and villous hypertrophy of the choroid plexus; as discussed earlier, mild epithelial cell cytomorphologic abnormalities in conjunction with cellular crowding and stratification are reliable features in favor of choroid plexus papillomas. ^[59]

In addition to choroid plexus papillomas, a number of other primary central nervous system (CNS) tumors may exhibit a papillary architecture; papillary ependymoma and astroblastoma would be the most likely to cause diagnostic confusion. Choroid plexus papillomas have a cytokeratin-positive epithelium with an underlying demonstrable basement membrane, and they lack the perivascular pseudorosettes and diffuse positivity for glial fibrillary acidic protein (GFAP) of these other tumors.

On a similar note, anaplastic ependymoma may mimic choroid plexus carcinoma; choroid plexus carcinoma is distinguished by virtue of strong cytokeratin positivity, lack of significant GFAP staining, lack of true or perivascular pseudorosettes, and a present (although often fragmented) basement membrane. Choroid plexus tumors are also typically E-cadherin positive and neural cell adhesion molecule (NCAM) negative, whereas ependymomas exhibit an inverse staining pattern for these markers. ^[52]

Choroid plexus tumors arising in the adult population need to be differentiated from a host of metastatic carcinomas (papillary or otherwise). This workup often necessitates a battery of immunohistochemical studies. Transthyretin and/or S100 expression is supportive evidence for a choroid plexus neoplasm—unfortunately these stains are not 100% reliable. Whereas CK7 and CK20 may not be especially helpful, demonstration of GFAP and/or synaptophysin positivity would not be expected in metastatic carcinomas, and would therefore favor choroid plexus tumor. Additional immunostains may be needed in some cases, choroid plexus carcinomas being positive for EAAT1, Ki17.1, and stanniocalcin-1, whereas positivity for HEA-125 and BerEP4 are indicative of a metastatic carcinoma. ^[49]

As noted previously, choroid plexus carcinomas may contain cells with rhabdoid morphology and immunohistochemical polyphenotypia, thus raising consideration of atypical teratoid/rhabdoid tumor (AT/RT) as a possible diagnosis. To complicate matters somewhat, choroid plexus carcinomas (similar to AT/RT) frequently show chromosome 22q deletions; therefore, fluorescence in situ hybridization (FISH) assays for INI1 (22q11.2) copy number alterations are of minimal utility in this differential diagnosis. In contrast, positive nuclear staining with anti-INI1 (BAF-47) by immunohistochemistry reliably differentiates choroid plexus carcinoma from AT/RT, the latter consistently lacking nuclear positivity with this marker indicative of INI1 protein loss. ^[54]

Other conditions that should be considered when evaluating suspected choroid plexus neoplasms include metastatic cancer with unknown primary site, endolymphatic sac tumor, ^[48]  CNS embryonal tumor or medulloblastoma, and papillary thyroid carcinoma.

The extent of resection and histologic grade are the most important prognostic factors determining recurrence-free and overall survival in patients with choroid plexus tumors, and surgical resection remains the first-line therapy for these patients. ^[60] Choroid plexus papillomas may be cured by gross total resection alone, and even in cases with recurrent disease, the outcome is often still favorable (5-year survival rates of 80-100% following gross total resection and approximately 68% following subtotal resection). ^{[3, 61, 62]} A small percentage of choroid plexus papillomas undergo malignant progression, but this is an extremely rare occurrence. ^[63]

Choroid plexus carcinomas are significantly more aggressive, with a greater tendency for leptomeningeal dissemination and/or recurrence as well as a survival rate around half of that seen with choroid plexus papillomas. ^{[2, 3, 4, 61]}  Choroid plexus carcinomas may have a favorable outcome when gross total resection is combined with adjuvant chemotherapy and/or local radiotherapy. ^{[2, 5, 64, 65, 66]}  One study found that carcinomas with two copies of mutated TP53 were significantly more aggressive and resulted in poorer survival outcomes then carcinomas with only one mutated TP53. ^[57]  Relatively recent evidence suggests that methylation profling may potentially provide useful prognostic information for choroid plexus tumor, in addition to histopathology. ^[56]  Craniospinal irradiation may be helpful in those cases of choroid plexus carcinoma that undergo subtotal resection and/or have disseminated leptomeningeal disease at presentation. ^{[16, 67, 68]}

Not surprisingly, atypical choroid plexus papillomas fall somewhere between these two extremes in terms of their biologic behavior; in general, atypical choroid plexus papillomas have a good prognosis but with a greater risk of local recurrence than conventional choroid plexus papillomas. ^{[1, 69]} In fact, increased mitotic activity is the only histologic feature identified to be independently associated with recurrence: choroid plexus papillomas with increased mitoses carry nearly five times the risk of recurrence compared with less proliferative papillomas. Additional histologic features suggesting a poor prognosis include decreased S-100 protein expression (<50% of cells strongly positive for S-100), lack of immunoreactivity for transthyretin, brain invasion, lack of marked stromal edema, and the presence of necrosis. ^[28]

For more detailed information regarding choroid plexus neoplasms, the authors suggest the following resources:

Louis DN, Ohgaki H, Wiestler OD, et al, eds. WHO Classification of Tumours of the Central Nervous System. Lyon, France: IARC Press; 2016. ^[1]

Mallick S, Benson R, Melgandi W, Rath GK. Effect of surgery, adjuvant therapy, and other prognostic factors on choroid plexus carcinoma: a systematic review and individual patient data analysis. Int J Radiat Oncol Biol Phys. 2017 Dec 1. 99 (5):1199-206. [Medline]. ^[5]

Wrede B, Hasselblatt M, Peters O, et al. Atypical choroid plexus papilloma: clinical experience in the CPT-SIOP-2000 study. J Neurooncol. Dec 2009;95(3):383-92. [Medline]. ^[69]

Ogiwara H, Dipatri AJ Jr, Alden TD, Bowman RM, Tomita T. Choroid plexus tumors in pediatric patients. Br J Neurosurg. Feb 2012;26(1):32-7. [Medline]. ^[61]

Bettegowda C, Adogwa O, Mehta V, et al. Treatment of choroid plexus tumors: a 20-year single institutional experience. J Neurosurg Pediatr. Nov 2012;10(5):398-405. [Medline]. ^[3]

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. Dec 2007;85(3):345-51. [Medline]. ^[68]

Passariello A, Tufano M, Spennato P, et al. The role of chemotherapy and surgical removal in the treatment of choroid plexus carcinomas and atypical papillomas. Childs Nerv Syst. 2015 Jul. 31 (7):1079-88. [Medline]. ^[67]

Merino DM, Shlien A, Villani A, et al. Molecular characterization of choroid plexus tumors reveals novel clinically relevant subgroups. Clin Cancer Res. 2015 Jan 1. 21 (1):184-92. [Medline]. ^[57]

Louis DN, Ohgaki H, Wiestler OD, et al, eds. Chapter 5: choroid plexus tumours. WHO Classification of Tumours of the Central Nervous System. Rev 4th ed. Lyon, France: IARC Press; 2016. 123-9.

Bahar M, Hashem H, Tekautz T, et al. Choroid plexus tumors in adult and pediatric populations: the Cleveland Clinic and University Hospitals experience. J Neurooncol. 2017 May. 132 (3):427-32. [Medline].

Bettegowda C, Adogwa O, Mehta V, et al. Treatment of choroid plexus tumors: a 20-year single institutional experience. J Neurosurg Pediatr. 2012 Nov. 10 (5):398-405. [Medline].

Cannon DM, Mohindra P, Gondi V, Kruser TJ, Kozak KR. Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management. J Neurooncol. 2015 Jan. 121 (1):151-7. [Medline].

Mallick S, Benson R, Melgandi W, Rath GK. Effect of surgery, adjuvant therapy, and other prognostic factors on choroid plexus carcinoma: a systematic review and individual patient data analysis. Int J Radiat Oncol Biol Phys. 2017 Dec 1. 99 (5):1199-206. [Medline].

Frye RE, Polling JS, Ma LC. Choroid plexus papilloma expansion over 7 years in Aicardi syndrome. J Child Neurol. 2007 Apr. 22 (4):484-7. [Medline]. [Full Text].

Hori A, Walter GF, Haas J, Becker H. Down syndrome complicated by brain tumors: case report and review of the literature. Brain Dev. 1992 Nov. 14 (6):396-400. [Medline].

Blamires TL, Maher ER. Choroid plexus papilloma. A new presentation of von Hippel-Lindau (VHL) disease. Eye (Lond). 1992. 6 ( Pt 1):90-2. [Medline].

Zakrzewska M, Wojcik I, Zakrzewski K, et al. Mutational analysis of hSNF5/INI1 and TP53 genes in choroid plexus carcinomas. Cancer Genet Cytogenet. 2005 Jan 15. 156 (2):179-82. [Medline].

Gozali AE, Britt B, Shane L, et al. 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 Jun. 58 (6):905-9. [Medline].

O’Neill AF, Voss SD, Jagannathan JP, et al. Screening with whole-body magnetic resonance imaging in pediatric subjects with Li-Fraumeni syndrome: A single institution pilot study. Pediatr Blood Cancer. 2018 Feb. 65 (2):[Medline].

Amadou A, Waddington Achatz MI, Hainaut P. Revisiting tumor patterns and penetrance in germline TP53 mutation carriers: temporal phases of Li-Fraumeni syndrome. Curr Opin Oncol. 2018 Jan. 30 (1):23-9. [Medline].

Nomura H, Momma F, Furuichi S, Okamoto J. Primary choroid plexus papilloma of the foramen magnum–case report. Neurol Med Chir (Tokyo). 1997 Sep. 37 (9):685-7. [Medline].

Pillai A, Rajeev K, Chandi S, Unnikrishnan M. Intrinsic brainstem choroid plexus papilloma. Case report. J Neurosurg. 2004 Jun. 100 (6):1076-8. [Medline].

Scholsem M, Scholtes F, Robe PA, Bianchi E, Kroonen J, Deprez M. Multifocal choroid plexus papilloma: a case report. Clin Neuropathol. 2012 Nov-Dec. 31 (6):430-4. [Medline].

Meyers SP, Khademian ZP, Chuang SH, Pollack IF, Korones DN, Zimmerman RA. Choroid plexus carcinomas in children: MRI features and patient outcomes. Neuroradiology. 2004 Sep. 46 (9):770-80. [Medline].

Abdulkader MM, Mansour NH, Van Gompel JJ, et al. Disseminated choroid plexus papillomas in adults: a case series and review of the literature. J Clin Neurosci. 2016 Oct. 32:148-54. [Medline].

Donovan DJ, Prauner RD. Shunt-related abdominal metastases in a child with choroid plexus carcinoma: case report. Neurosurgery. 2005 Feb. 56 (2):E412; discussion E412. [Medline].

Shi YZ, Chen MZ, Huang W, et al. Atypical choroid plexus papilloma: clinicopathological and neuroradiological features. Acta Radiol. 2017 Aug. 58 (8):983-90. [Medline].

Taylor MB, Jackson RW, Hughes DG, Wright NB. Magnetic resonance imaging in the diagnosis and management of choroid plexus carcinoma in children. Pediatr Radiol. 2001 Sep. 31 (9):624-30. [Medline].

Patanakar T, Prasad S, Mukherji SK, Armao D. Cystic variant of choroid plexus papilloma. Clin Imaging. 2000 May-Jun. 24 (3):130-1. [Medline].

Barreto AS, Vassallo J, Queiroz Lde S. Papillomas and carcinomas of the choroid plexus: histological and immunohistochemical studies and comparison with normal fetal choroid plexus. Arq Neuropsiquiatr. 2004 Sep. 62 (3A):600-7. [Medline].

Hasselblatt M, Jeibmann A, Guerry M, Senner V, Paulus W, McLendon RE. Choroid plexus papilloma with neuropil-like islands. Am J Surg Pathol. 2008 Jan. 32 (1):162-6. [Medline].

Sav A, Scheithauer BW, Mazzola CA, Ketterling SR, Thompson SJ, Reilly MH. Oncocytic choroid plexus carcinoma: case report. Clin Neuropathol. 2010 Jan-Feb. 29 (1):14-20. [Medline].

Ikota H, Tanaka Y, Yokoo H, Nakazato Y. Clinicopathological and immunohistochemical study of 20 choroid plexus tumors: their histological diversity and the expression of markers useful for differentiation from metastatic cancer. Brain Tumor Pathol. 2011 Jul. 28 (3):215-21. [Medline].

Corcoran GM, Frazier SR, Prayson RA. Choroid plexus papilloma with osseous and adipose metaplasia. Ann Diagn Pathol. 2001 Feb. 5 (1):43-7. [Medline].

Yap WM, Chuah KL, Tan PH. Choroid plexus papilloma with chondroid metaplasia. Histopathology. 1997 Oct. 31 (4):386-7. [Medline].

Jeibmann A, Hasselblatt M, Gerss J, et al. Prognostic implications of atypical histologic features in choroid plexus papilloma. J Neuropathol Exp Neurol. 2006 Nov. 65 (11):1069-73. [Medline].

Naeini RM, Yoo JH, Hunter JV. Spectrum of choroid plexus lesions in children. AJR Am J Roentgenol. 2009 Jan. 192 (1):32-40. [Medline].

Wyatt-Ashmead J, Kleinschmidt-DeMasters B, Mierau GW, et al. Choroid plexus carcinomas and rhabdoid tumors: phenotypic and genotypic overlap. Pediatr Dev Pathol. 2001 Nov-Dec. 4 (6):545-9. [Medline].

Tena-Suck ML, Gomez-Amador JL, Ortiz-Plata A, Salina-Lara C, Rembao-Bojorquez D, Vega-Orozco R. Rhabdoid choroid plexus carcinoma: a rare histological type. Arq Neuropsiquiatr. 2007 Sep. 65 (3A):705-9. [Medline].

Dobin SM, Donner LR. Pigmented choroid plexus carcinoma: a cytogenetic and ultrastructural study. Cancer Genet Cytogenet. 1997 Jul 1. 96 (1):37-41. [Medline].

Buchino JJ, Mason KG. Choroid plexus papilloma. Report of a case with cytologic differential diagnosis. Acta Cytol. 1992 Jan-Feb. 36 (1):95-7. [Medline].

Savage NM, Crosby JH, Reid-Nicholson MD. The cytologic findings in choroid plexus carcinoma: report of a case with differential diagnosis. Diagn Cytopathol. 2012 Jan. 40 (1):1-6. [Medline].

Ghatak NR, McWhorter JM. Ultrastructural evidence for CSF production by a choroid plexus papilloma. J Neurosurg. 1976 Oct. 45 (4):409-15. [Medline].

Felix I, Phudhichareonrat S, Halliday WC, Becker LE. Choroid plexus tumors in children: immunohistochemical and scanning-electron-microscopic features. Pediatr Neurosci. 1987. 13 (5):263-9. [Medline].

Paulus W, Jänisch W. Clinicopathologic correlations in epithelial choroid plexus neoplasms: a study of 52 cases. Acta Neuropathol. 1990. 80 (6):635-41. [Medline].

Kato T, Fujita M, Sawamura Y, et al. Clinicopathological study of choroid plexus tumors: immunohistochemical features and evaluation of proliferative potential by PCNA and Ki-67 immunostaining. Noshuyo Byori. 1996 Nov. 13 (2):99-105. [Medline].

Kepes JJ, Collins J. Choroid plexus epithelium (normal and neoplastic) expresses synaptophysin. A potentially useful aid in differentiating carcinoma of the choroid plexus from metastatic papillary carcinomas. J Neuropathol Exp Neurol. 1999 Apr. 58 (4):398-401. [Medline].

Hasselblatt M, Böhm C, Tatenhorst L, et al. Identification of novel diagnostic markers for choroid plexus tumors: a microarray-based approach. Am J Surg Pathol. 2006 Jan. 30 (1):66-74. [Medline].

Gyure KA, Morrison AL. Cytokeratin 7 and 20 expression in choroid plexus tumors: utility in differentiating these neoplasms from metastatic carcinomas. Mod Pathol. 2000 Jun. 13 (6):638-43. [Medline].

Newbould MJ, Kelsey AM, Arango JC, Ironside JW, Birch J. The choroid plexus carcinomas of childhood: histopathology, immunocytochemistry and clinicopathological correlations. Histopathology. 1995 Feb. 26 (2):137-43. [Medline].

Inamura T, Nishio S, Miyagi Y, et al. Primary choroid plexus carcinoma producing carbohydrate antigen 19-9. Clin Neuropathol. 2000 Nov-Dec. 19 (6):268-72. [Medline].

Kohmura E, Maruno M, Sawada K, Arita N, Yoshimine T. Usefulness of synaptophysin immunohistochemistry in an adult case of choroid plexus carcinoma. Neurol Res. 2000 Jul. 22 (5):478-80. [Medline].

Vajtai I, Varga Z, Aguzzi A. MIB-1 immunoreactivity reveals different labelling in low-grade and in malignant epithelial neoplasms of the choroid plexus. Histopathology. 1996 Aug. 29 (2):147-51. [Medline].

Jay V, Ho M, Chan F, Malkin D. P53 expression in choroid plexus neoplasms: an immunohistochemical study. Arch Pathol Lab Med. 1996 Nov. 120 (11):1061-5. [Medline].

Beschorner R, Schittenhelm J, Schimmel H, et al. Choroid plexus tumors differ from metastatic carcinomas by expression of the excitatory amino acid transporter-1. Hum Pathol. 2006 Jul. 37 (7):854-60. [Medline].

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 Jul. 138 (1):85-9. [Medline].

Gottschalk J, Jautzke G, Paulus W, Goebel S, Cervos-Navarro J. The use of immunomorphology to differentiate choroid plexus tumors from metastatic carcinomas. Cancer. 1993 Aug 15. 72 (4):1343-9. [Medline].

Osada H, Mori K, Yamamoto T, Nakao Y, Wada R, Maeda M. Choroid plexus carcinoma secreting carbohydrate antigen 19-9 in an adult. Case report. Neurol Med Chir (Tokyo). 2006 May. 46 (5):251-3. [Medline].

Furness PN, Lowe J, Tarrant GS. Subepithelial basement membrane deposition and intermediate filament expression in choroid plexus neoplasms and ependymomas. Histopathology. 1990 Mar. 16 (3):251-5. [Medline].

Figarella-Branger D, Lepidi H, Poncet C, et al. Differential expression of cell adhesion molecules (CAM), neural CAM and epithelial cadherin in ependymomas and choroid plexus tumors. Acta Neuropathol. 1995. 89 (3):248-57. [Medline].

Shibahara J, Kashima T, Kikuchi Y, Kunita A, Fukayama M. Podoplanin is expressed in subsets of tumors of the central nervous system. Virchows Arch. 2006 Apr. 448 (4):493-9. [Medline].

Judkins AR, Burger PC, Hamilton RL, et al. INI1 protein expression distinguishes atypical teratoid/rhabdoid tumor from choroid plexus carcinoma. J Neuropathol Exp Neurol. 2005 May. 64 (5):391-7. [Medline].

Japp AS, Gessi M, Messing-Junger M, et al. High-resolution genomic analysis does not qualify atypical plexus papilloma as a separate entity among choroid plexus tumors. J Neuropathol Exp Neurol. 2015 Feb. 74 (2):110-20. [Medline].

Thomas C, Sill M, Ruland V, Witten A, et al. Methylation profiling of choroid plexus tumors reveals 3 clinically distinct subgroups. Neuro Oncol. 2016 Jun. 18 (6):790-6. [Medline].

Merino DM, Shlien A, Villani A, et al. Molecular characterization of choroid plexus tumors reveals novel clinically relevant subgroups. Clin Cancer Res. 2015 Jan 1. 21 (1):184-92. [Medline].

Nupponen NN, Paulsson J, Jeibmann A, et al. Platelet-derived growth factor receptor expression and amplification in choroid plexus carcinomas. Mod Pathol. 2008 Mar. 21 (3):265-70. [Medline].

D’Ambrosio AL, O’Toole JE, Connolly ES Jr, Feldstein NA. Villous hypertrophy versus choroid plexus papilloma: a case report demonstrating a diagnostic role for the proliferation index. Pediatr Neurosurg. 2003 Jul. 39 (2):91-6. [Medline].

Sun MZ, Oh MC, Ivan ME, et al. Current management of choroid plexus carcinomas. Neurosurg Rev. 2014 Apr. 37 (2):179-92; discussion 192. [Medline].

Ogiwara H, Dipatri AJ Jr, Alden TD, Bowman RM, Tomita T. Choroid plexus tumors in pediatric patients. Br J Neurosurg. 2012 Feb. 26 (1):32-7. [Medline].

Dudley RW, Torok MR, Gallegos D, Liu AK, Handler MH, Hankinson TC. Pediatric choroid plexus tumors: epidemiology, treatments, and outcome analysis on 202 children from the SEER database. J Neurooncol. 2015 Jan. 121 (1):201-7. [Medline].

Jeibmann A, Wrede B, Peters O, Wolff JE, Paulus W, Hasselblatt M. Malignant progression in choroid plexus papillomas. J Neurosurg. 2007 Sep. 107 (3 suppl):199-202. [Medline].

Zaky W, Dhall G, Khatua S, et al. Choroid plexus carcinoma in children: the Head Start experience. Pediatr Blood Cancer. 2015 May. 62 (5):784-9. [Medline].

Lafay-Cousin L, Keene D, Carret AS, et al. Choroid plexus tumors in children less than 36 months: the Canadian Pediatric Brain Tumor Consortium (CPBTC) experience. Childs Nerv Syst. 2011 Feb. 27 (2):259-64. [Medline].

Cannon DM, Mohindra P, Gondi V, Kruser TJ, Kozak KR. Choroid plexus tumor epidemiology and outcomes: implications for surgical and radiotherapeutic management. J Neurooncol. 2015 Jan. 121 (1):151-7. [Medline].

Passariello A, Tufano M, Spennato P, et al. The role of chemotherapy and surgical removal in the treatment of Choroid Plexus carcinomas and atypical papillomas. Childs Nerv Syst. 2015 Jul. 31 (7):1079-88. [Medline].

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 Dec. 85 (3):345-51. [Medline].

Wrede B, Hasselblatt M, Peters O, et al. Atypical choroid plexus papilloma: clinical experience in the CPT-SIOP-2000 study. J Neurooncol. 2009 Dec. 95 (3):383-92. [Medline].

Christine Fuller, MD Professor of Pathology, Cincinnati Children’s Hospital Medical Center; Professor of Pathology, University of Cincinnati College of Medicine

Christine Fuller, MD is a member of the following medical societies: American Association of Neuropathologists, American Society for Clinical Pathology, College of American Pathologists, Society for Neuro-Oncology, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Kimberly A Stogner-Underwood, MD Neuropathology Fellow, Department of Pathology, Virginia Commonwealth University School of Medicine

Kimberly A Stogner-Underwood, MD is a member of the following medical societies: American Medical Association, American Medical Student Association/Foundation, College of American Pathologists, Medical Society of Virginia, South Carolina Medical Association, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Adekunle M Adesina, MD, PhD Professor, Medical Director, Section of Neuropathology, Director, Molecular Neuropathology Laboratory, Texas Children’s Hospital, Department of Pathology and Immunology, Baylor College of Medicine

Adekunle M Adesina, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Neuropathologists, College of American Pathologists, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Pathology of Choroid Plexus Neoplasms

Research & References of Pathology of Choroid Plexus Neoplasms|A&C Accounting And Tax Services
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