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Choroid plexus tumors in the breast cancer-sarcoma syndrome.

Choroid plexus neoplasms are rare epithelial tumors of the central nervous system. A carcinoma of the choroid plexus occurred in a child from a family with the breast cancer-sarcoma syndrome (Li-Fraumeni or SBLA syndrome), an inherited condition characterized by the development of diverse neoplasms (sarcoma, breast cancer, brain tumors, leukemia, adrenal cortical carcinoma, and others). Choroid plexus carcinomas were identified in two kindreds previously reported with the syndrome. The literature contains reports of choroid plexus neoplasms occurring in families and in individuals with multiple primary tumors. Choroid plexus neoplasm may be a manifestation of the inherited proclivity to tumor development in the breast cancer-sarcoma syndrome.

Adolescent↗

[Effect of choroid plexus on sequestering cadmium and its pathomorphological change].

To investigate whether choroid plexus can sequester cadmium(Cd) and its pathomorphological change, rabbits were given the injections of 1.0, 2.0 and 4.0 mg CdCl2/kg through ear vein, respectively. 4 and 24 hours after Cd administration, animals were sacrificed, the samples of blood, cerebrospinal fluid (CSF), choroid plexus of lateral ventricle, cerebral cortex, liver and kidney were collected and the concentration of Cd in these tissues were measured. Samples of choroid plexus were rushed in saline thoroughly, then fixed. Pathomorphological changes of choroidal epithelial cells were observed in light microscopy and electron microscopy. The result showed that concentration of Cd in choroid plexus was higher than that in blood, CSF and cerebral cortex. In high-dose group, concentration of Cd in CSF was higher than that in other groups. Choroidal epithelial cells appeared vacuolation and granuler degeneration in light microscopy. Choroidal epithelial cells lost their normal slight to moderate convex luminal profiles, and appeared the loss of microvilli, shaped nuclei, increased cytoplasmic vacuolation, increased number of lysosomes, dilated rough endoplasmic reticulum, and widening of the gaps between adjacent cells. Choroid plexus could accumulate and sequester Cd to protect cerebral cortex from the fluxes of Cd in blood cadmium. When concentration of cd reached a certain level, sequestering action of choroid plexus was weak, or disappeared. Choroid plexus of lateral ventricle appeared pathomorphological change.

Animals↗

Immunocytochemical detection of insulin-like growth factor II (IGF-II) in choroid plexus papilloma: a possible marker for differential diagnosis.

BACKGROUND: Insulin-like growth factor II (IGF-II) has been detected in the choroid plexus of animals by means of immunohistochemistry and in situ hybridization, and this factor is thought to play an important role in the central nervous system (CNS). Little is known, however, about the presence and localization of this substance in the choroid plexus and in choroid plexus papilloma in humans. MATERIALS AND METHODS: 5 normal choroid plexus and 10 choroid plexus papillomas were examined for IGF-II by immunocytochemistry. IGF-II was not detected in normal human choroid plexus, whereas it was found in choroid plexus papilloma. Furthermore, to assess the possibility that IGF-II could serve as an immunohistological marker of choroid plexus papilloma, we used the same technique to examine paraffin-embedded samples from various kinds of brain tumors. RESULTS AND CONCLUSION: Our results suggest that IGF-II may be a useful marker for choroid plexus papilloma in differential diagnosis.

Adolescent↗

Fcgamma receptors in human choroid plexus.

Crysotat sections of human choroid plexus adsorbed erythrocytes sensitized with IgG antibodies of human and rabbit origin. No adsorption occurred when the erythrocytes were sensitized with F(ab')2 or Facb fragments. The reaction was strongly inhibited by intact IgG and by Fc fragments and not inhibited by Facb and F(ab')2 or albumin. These properties are similar to those of corresponding receptors in human placenta. The presence of an Fcgamma receptor in choroid plexus may be of significance for the transfer of IgG from blood to cerebrospinal fluid.

Choroid Plexus↗

The interventricular extension of choroid plexus papillomas.

Four cases of choroid plexus papilloma extending through the foramen of Monro are presented. All four patients were evaluated by contrast-enhanced CT and two of these were also evaluated by neurosonography. No other intraventricular tumor commonly extends through a ventricular outlet. The radiographic demonstration of this extension of an intraventricular mass through the foramen of Monro, cerebral aqueduct, or foramen of Luschka or Magendie represents an ancillary diagnostic sign of choroid plexus papilloma.

Cerebral Ventricle Neoplasms↗

Neuroendoscopic removal of large choroid plexus cyst: a case report.

Choroid plexus cysts (CPCs) are the most common neuroepithelial cysts, occurring in more than 50% of some autopsy series. They are typically small and asymptomatic and are discovered incidentally in older patients, usually in the trigone of the lateral ventricle. Symptomatic CPCs (usually exceptionally large, 2-8 cm) are rare. The authors report a case of large symptomatic choroid plexus cyst, located in the trigone of the right lateral ventricle in a 26-yr-old man who presented with headache and vomiting. The patient underwent endoscopic removal through a burr hole placed 3 cm from the midline and just behind the hair line. The histological examination of the cyst wall was consistent with choroid epithelium. Despite of postoperative intraventricular hemorrhage and catheter infection, he discharged home without neurologic deficits. The endoscopic fenestration rather than excision should be considered as the first surgical procedure because the goal of treatment is shrinkage of the cyst until normal cerebrospinal fluid flow is restored.

Adult↗

On the anatomic relation of choroid plexus to brain: a comparative study.

The size of choroid plexuses and cerebral ventricles relative to brain varies widely among vertebrates. The functional significance of this variability has attracted little attention since Herrick's original proposal that large choroid plexuses might enhance oxygen delivery to the brain and therefore be of adaptive value in the transition of vertebrates from water to air breathing. We compared choroid plexus and brain weight or ventricular and brain volume in 40 species from nine vertebrate groups. Both choroid plexus weight and ventricular volume were unrelated to brain size. Plexus weight ranged from 0 to 5.2% of brain weight and ventricular volume from 0.9 to 132% of brain volume. Amid this diversity the dipnoans, chondrosteans, holosteans, amphibians, and crossopterygian examined in this study are exceptional in uniformly having large plexuses. The adaptive significance of large choroid plexuses may lie in the presence of specific homeostatic mechanisms and their role in the response to the increases in PCO2 that accompany the transition to air breathing.

Animals↗

Choroid plexus carcinoma in an infant.

Choroid plexus carcinoma is a rare intracranial neoplasm, affecting mainly very young children. The commonest site is within the lateral ventricles and the prognosis is very poor. We report a seven month old baby boy who presented with raised intracranial pressure and seizures. Brain CT scan showed large intraventricular mass with calcification and hydrocephalus. Total macroscopic resection of the tumour was performed and diagnosis of choroid plexus carcinoma was made. However, the patient died 11 days after the tumour excision. The histopathology of this rare childhood neoplasm is discussed.

Carcinoma↗

Barrier mechanisms for neutrotransmitter monoamines in the choroid plexus.

Studies were performed on choroid plexuses from mouse and rat. The plexus tissue, sympathectomized to avoid interference from adrenergic nerves, is able to accumulate L-DOPA as well as NA and 5-HT against a concentration gradient. Within the plexus tissue these compounds are metabolized by aromatic L-amino acid decarboxylase, MAO and COMT, whose activities were determined radiometrically. On the basis of this and the fate of systemically administered L-DOPA and DA studied by fluorescence histochemistry it is suggested that the epithelium of the choroid plexus provides not only a structural but also an enzymatic barrier for transmitter amines. The enzymatic barrier for amine precursors is considerably less sufficient at this blood-CSF interphase. The trapping of monoamines resembles the enzymatic barrier present in the endothelial cells and pericytes at the microvascular level of the blood-brain interphase.

Animals↗

MRI of cerebellopontine angle choroid plexus papilloma.

We present a cerebellopontine angle choroid plexus papilloma that originated from the tuft of choroid plexus of the fourth ventricle protruding from the foramen of Luschka. MRI and CT did not establish the diagnosis, but the tumor was shown histopathologically to be a choroid plexus papilloma. Distinct features of the tumor on MRI are described and the differential diagnosis of other cerebellopontine angle tumors is discussed.

Cerebellar Neoplasms↗

Persistent large choroid plexus cyst. A case report.

BACKGROUND: Prenatally diagnosed choroid plexus cysts regress or resolve spontaneously during pregnancy. A persistent large choroid plexus cyst with a prenatal diagnosis has not been reported previously. CASE: A 28-year-old, healthy primigravida was referred to our department at 32 weeks' gestation for a suspected fetal intracranial anomaly. Ultrasonography revealed a lateral ventricle 13 mm in width. The ventricle was dilated only in the atrium region. The choroid plexus was not distinct from the inner wall of the ventricle and did not fill the atrium of the lateral ventricle. The patient was followed with bi-weekly ultrasonography until delivery, and the left ventricular width increased from 13 to 17 mm. At 38 weeks' gestation the patient delivered a 3,350-g girl. Magnetic resonance imaging showed a 1.7 x 2.5 x 3.0-cm cyst in the left lateral ventricle. Follow-up magnetic resonance imaging scans at 6 and 11 months showed unchanged findings. Clinical and neurologic examinations at 11 months of age showed normal development. CONCLUSION: Although small (< 1 cm), postnatally persistent choroid plexus cysts are clinically insignificant variants of normal; the prognosis of large, persistent cysts (> or = 1 cm) is less clear. Long-term neurologic follow-up is mandatory for these neonates.

Adult↗

The isolated choroid plexus cyst.

OBJECTIVE: To describe the karyotypes of a population of fetuses with choroid plexus cysts and compare affected fetuses with and without additional ultrasonographic findings. METHODS: The study population included all patients undergoing second-trimester ultrasound examination in a prenatal diagnostic program between January 1993 and October 1995. The records of all cases in which a choroid plexus cyst was found were reviewed, and information was abstracted regarding the fetal karyotype and the presence of other sonographic abnormalities. RESULTS: Two hundred ten cases of choroid plexus cysts were identified among 7617 patients (2.8%) who underwent second-trimester ultrasound examination. The majority of the cases (181, or 86%) involved isolated choroid plexus cysts and the remaining 29 (14%) were associated with additional ultrasonographic findings. Autosomal aneuploidy was found in one patient with an isolated choroid plexus cyst (trisomy 21) and in another with additional findings (trisomy 18); the mothers of both of these patients were at least 35 years old. For those fetuses with known outcome, the risk of aneuploidy with isolated choroid plexus cyst (one in 180) was not statistically significantly different from that associated with choroid plexus cyst accompanied by other sonographic findings (one in 26). More than 1000 fetuses with choroid plexus cysts would have to be studied to determine whether such a difference was real. CONCLUSION: Because of the rarity of aneuploidy, the reported risk for a fetus with an isolated choroid plexus cyst must be interpreted cautiously and should include the baseline risk.

Brain Diseases↗

[Antigenic expression in human choroid plexus carcinoma: report of 2 cases].

Primary neoplasms of choroid plexus are rare. Six morphological variants have been described: papillary, cystic, acinar, mucus-secreting, oncocytic, and anaplastic. The anaplastic variant, the so-called choroid plexus carcinoma, is the rarest of all and can metastasize. The differential diagnosis of the anaplastic variant of choroid plexus neoplasms with adenocarcinomas, melanomas and undifferentiated neoplasms can be troublesome chiefly in adults. The now large use of immunocytochemical techniques in tissue section has become a powerful tool in the analysis of cell lineages, tumoral and non-tumoral. Nevertheless, the choroid plexus neoplasms have shown a complex and a somewhat confusing pattern of antigenic expression. In two choroid plexus carcinomas (one localized in the right lateral ventricle from a boy of 1 year and 9 months old, and the other localized in the left lateral ventricle from a girl of 3 years old) the following antigens were searched (using the avidin-biotin-peroxidase complex): glial fibrillary acidic protein (GFAP) with monoclonal and polyclonal antibodies; cytokeratins of 40-50kDa, cytokeratins of 60-70kDA (callus cytokeratin), neuronal specific enolase (NSE) and S-100 protein with monoclonal antibodies. The two neoplasms showed immunoreactivity against NSE, S-100 protein and cytokeratin of 40-50kDA. The neoplasm of the boy exhibited glial differentiation having immunoreactivity against GFAP with monoclonal and polyclonal antibodies.

Antigens, Neoplasm↗

Autonomic nerves in the mammalian choroid plexus and their influence on the formation of cerebrospinal fluid.

The choroid plexuses of all ventricles receive a well-developed adrenergic and cholinergic innervation reaching both the secretory epithelium and the vascular smooth muscle cells. Also peptidergic nerves, containing vasoactive intestinal polypeptide, are present but primarily associated only with the vascular bed. A sympathetic inhibitory effect on the plexus epithelium has been indicated in determinations of carbonic anhydrase activity and by studies of various aspects of active transport in isolated plexus tissue. Pharmacological analysis in vitro has shown the choroidal arteries to possess both vasoconstrictory alpha-adrenergic and vasodilatory beta-adrenergic receptors. Electrical stimulation of the sympathetic nerves, which originate in the superior cervical ganglia, induces as much as 30% reduction in the net rate of cerebrospinal fluid (CSF) production, while sympathectomy results in a pronounced increase, about 30% above control, in the CSF formation. There is strong reason to believe that the choroid plexus is under the influence of a considerable sympathetic inhibitory tone under steady-state conditions. From pharmacological and biochemical experiments it is suggested that the sympathomimetic reduction in the rate of CSF formation is the result of a combined beta-receptor-mediated inhibition of the secretion from the plexus epithelium and a reduced blood flow in the choroid plexus tissue resulting from stimulation of the vascular alpha-receptors. The choroid plexus probably also represents an important inactivation site and gate mechanism for sympathomimetic amines, as evidenced by considerable local activity of catechol-O-methyl transferase and monoamine oxidase, primarily type B. The CSF production rate is also reduced by cholinomimetic agents, suggesting the presence of muscarinic-type cholinergic receptors in the choroid plexus.

Acetylcholinesterase↗

Sonography of fetal choroid plexus cysts: detection depends on cyst size and gestational age.

OBJECTIVE: To establish diagnostic criteria for the diagnosis of fetal choroid plexus cysts across gestation. METHODS: Prenatal sonographic images of 166 fetuses without choroid plexus cysts were prospectively collected from 13 weeks to term. Texture characteristics of the choroid plexus regions were analyzed to quantify changes as a function of gestational age. A set of 20 fetal sonographic images with choroid plexus cysts were used to create cyst prototypes (1.5-2.5 mm), which were randomly embedded into normal choroid plexus images from varying gestational ages. A test set of 544 images was created, which included 408 images with choroid plexus cysts and 136 images without choroid plexus cysts. Four observers following a blinded study design evaluated the presence of choroid plexus cysts in the images. The influence of cyst size and gestational age on the detection of cysts was measured with receiver operating characteristic analysis and analysis of variance. Observer agreement was characterized by agreement kappa statistics. RESULTS: Texture analysis indicated greater echo texture heterogeneity at earlier gestational ages. The receiver operating characteristic analysis showed a corresponding decrease in diagnostic accuracy for cyst detection at earlier gestational ages. The cyst detection threshold (area under the receiver operating characteristic curve, 0.8) was 2.4 mm (SE, 0.2 mm) for 13 to 21 weeks' gestation and decreased to 1.9 mm (SE, 0.17 mm) for 22 to 38 weeks' gestation. Average interobserver and intraobserver kappa statistics were 0.37 and 0.53, respectively. CONCLUSIONS: Because of the changing echo texture of the choroid plexus through gestation, choroid plexus cysts must be at least 2.5 mm in diameter for confident diagnosis before 22 weeks' gestation and at least 2 mm after 22 weeks.

Adult↗

Colloid cyst of the third ventricle. A comparative immunohistochemical study of neuraxis cysts and choroid plexus epithelium.

In an effort to shed light upon the nature of the colloid cyst, the immunohistochemical properties of 21 examples of this lesion were compared with those of other neuraxial cysts and choroid plexus epithelium. The neuraxial cysts included the following: eight Rathke's cleft cysts, 25 pituitaries containing follicular cysts of the pars intermedia, and four enterogenous cysts. Fifteen examples of normal choroid plexus and 12 choroid plexus papillomas were studied as well. These lesions were examined for localization of the following antigens: cytokeratins, epithelial membrane antigen, secretory component, carcinoembryonic antigen, prealbumin, vimentin, glial fibrillary acidic protein (GFAP), S-100 protein, neuron-specific enolase, 68-kD neurofilament protein, chromogranin, serotonin, and lysozyme, and with Leu-7 monoclonal antibodies. Five colloid cysts were immunostained with monoclonal antibodies that were specific for Clara-cell antigens and surfactant, respectively. Sugar moieties were localized using Ulex europaeus I, and Ricinus communis agglutinin I lectins. All Rathke's cleft cysts and follicular cysts of the pars intermedia as well as three selected colloid cysts were examined for pituitary hormones. The epithelial cells of colloid and enterogenous cysts, as well as those lining follicular and Rathke's cleft cyst, showed uniformly strong reactivity for cytokeratins, epithelial membrane antigen, secretory component, and vimentin, and bound Ulex europaeus lectin. Occasional cells in colloid cysts were positive for Clara cell-specific antigens. Reaction for carcinoembryonic antigen was present on the apical surface of scattered cells of colloid, follicular, and Rathke's cleft cysts. Many cells of follicles in the pars intermedia as well as individual cells of five Rathke's cleft cysts were also immunoreactive for chromogranin, S-100 protein, GFAP, and pituitary hormones. Colloid and enterogenous cysts were negative for prealbumin, S-100 protein, GFAP, and neuron-specific enolase; in all but a few instances, they failed to bind Ricinus communis agglutinin. In contrast, normal choroid plexus and choroid plexus papillomas were positive for prealbumin, S-100 protein, neuron-specific enolase, cytokeratin, vimentin, and Ricinus communis agglutinin receptors; they lacked Ulex europaeus lectin, 56/66-kD cytokeratins, and epithelial membrane antigen. Unlike normal choroid plexus, choroid plexus papillomas were often GFAP-positive. All tissues studied were nonreactive for lysosome, serotonin, and neurofilament, and with Leu-7 antibodies. This study indicates that the immunophenotype of epithelium lining colloid cysts is similar to that of other cysts showing endodermal or ectodermal differentiation and to respiratory tract mucosa. Epithelium of colloid cysts is immunohistochemically different from that of normal or neoplastic choroid plexus. These findings indicate an endodermal rather than neuroepithelial nature for colloid cysts.

Brain Diseases↗

Unusual localization of a choroid plexus papilloma in a 4-year-old female.

Choroid plexus papillomas are rare tumors that are confined to areas in which the choroid plexus is normally located. In children, choroid plexus papillomas are predominantly located in the lateral ventricles. Clinically they present with signs of raised intracranial pressure, such as vomiting and increasing head size. Here we report on the clinical, radiologic, and histologic findings of a 4-year-old female who was found to have a tumor in the posterior fossa that had all the histologic hallmarks of a choroid plexus papilloma. This tumor did not originate from the roof of the fourth ventricle as expected but from the ependymal lining covering the median rostral medulla near the pontomedullary junction, a location that so far has not been reported.

Child, Preschool↗

Psammomatous choroid plexus papilloma: three cases with atypical characteristics.

BACKGROUND: Intravertricular papillary neoplasms are derived from choroid plexus epithelium. Although choroid plexus tumors account for 0.4% to 0.6% of all brain tumors, they represent 2% to 4%. Approximately 80% of choroid plexus carcinomas arise in children. CASES DESCRIPTION: We describe 3 cases of choroid plexus papilloma (CPP) with profuse psammomatous bodies and calcifications that have lost their normal papillary architecture. Immunohistochemistry was positive for glial fibrillary acidic protein in 2 cases, and proliferating cellular nuclear antigen index was higher compared with regular CPPs. All 3 patients were female and were 12, 40, and 48 years old, respectively. CONCLUSION: We describe psammomatous CPPs and suggest a difference from CPPs because of the more aggressive clinical course, and higher nuclear proliferation index (proliferating cellular nuclear antigen) than the CPPs that lack psammoma bodies.

Adult↗