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Choroid Plexus Tumor

CPT

Choroid plexus tumor is a rare occurrence in general practice. Both benign (papillomas) and malignant (carcinomas) variants exist, with the benign type being much more common. They are mainly identified in younger children, but individuals of any age can develop choroid plexus tumors. Symptoms and signs are related to the accumulation of cerebrospinal fluid and raised intracranial pressure and include vomiting, visual changes, seizures, bulging fontanelles, strabismus, and various neurological deficits. Imaging studies are crucial for the diagnosis, but a histopathological examination is often necessary to discriminate between benign and malignant tumors.


Presentation

The vast majority of patients who develop choroid plexus tumor are younger children, with studies estimating a median age at diagnosis of 3-4 years [1] [2]. The clinical presentation of choroid plexus tumors stems from the mechanical obstruction of cerebrospinal fluid (CSF) flow through the ventricular system and increased CSF production, eventually producing chronic raised intracranial pressure and hydrocephalus [1] [3]. Nausea, vomiting, headaches, ataxia, nystagmus, dizziness, and diplopia are typical consequences of hydrocephalus and are highly indicative of a tumor originating from the fourth ventricle [4]. On the other hand, papilledema, hemiparesis, seizures, and changes in the mental status are typically encountered when the tumor is situated in the lateral ventricle [4]. An increased circumference of the head, bulging fontanelles, separated sutures, irritability, and a delayed development are findings that may be encountered in the first few years of life [1] [3] [4]. Development of choroid plexus tumors (both papillomas and carcinomas) is very rare in the adult population, but cases have been documented [2] [5]. Benign choroid plexus tumors - papillomas, carry very high 5-year survival rates (reaching up to 100% in some studies) with an early recognition and appropriate therapy [2] [5], whereas carcinomas have a substantially worse prognosis (as low as 26% of patients survive 5 years after the diagnosis), particularly if the tumor is recognized late [1] [2] [5].

Fatigue
  • This increases the amount of fluid in the ventricles (“water on the brain“- hydrocephalus), increasing the pressure in the brain causing the brain/skull to get larger and causing headache, nausea, vomiting, irritability, fatigue and difficulty feeding[nicklauschildrens.org]
  • Some of the chemotherapy agents are associated with fatigue, hair loss, nausea, vomiting and headache. These side effects can be effectively managed under most circumstances.[danafarberbostonchildrens.org]
  • Symptoms include fatigue, weight gain, constipation, dry skin, depression, and the inability to exercise. It is more common in females and can run in families.[cancer.gov]
Loss of Hair
  • It belongs to a family of compounds called anthraquinones, which have shown anti-inflammatory and anticancer effects. alopecia listen (A-loh-PEE-shuh) The lack or loss of hair from areas of the body where hair is usually found.[cancer.gov]
Aspiration
  • Ultrasonic aspirator: The ultrasonic aspirator is helpful when performing a central debulking and when the tumor is slightly more fibrous and difficult to aspirate with suction alone.[ispn.guide]
  • Certain types of Aspergillus may cause disease, especially in people who have suppressed immune systems. aspirate Aspirate (pronounced AS-pih-rit) refers to fluid, tissue, or other substance that is withdrawn from a body cavity, cyst, or tumor.[cancer.gov]
Nausea
  • Nausea, vomiting, headaches, ataxia, nystagmus, dizziness, and diplopia are typical consequences of hydrocephalus and are highly indicative of a tumor originating from the fourth ventricle.[symptoma.com]
  • […] the central nervous system: Choroid plexus carcinoma (WHO grade III) Choroid atypical plexus papilloma (WHO grade II) Choroid plexus papilloma (WHO grade I) Symptoms vary depending on the size and location of the tumor and typically include headaches, nausea[en.wikipedia.org]
  • This increases the amount of fluid in the ventricles (“water on the brain“- hydrocephalus), increasing the pressure in the brain causing the brain/skull to get larger and causing headache, nausea, vomiting, irritability, fatigue and difficulty feeding[nicklauschildrens.org]
  • Some of the chemotherapy agents are associated with fatigue, hair loss, nausea, vomiting and headache. These side effects can be effectively managed under most circumstances.[danafarberbostonchildrens.org]
  • His headache was distent in nature and sometimes associated with nausea and vomiting. Before consulting us, he had visited a local hospital and had a brain computed tomography (CT) which showed a mass at the left occipital area.[file.scirp.org]
Hearing Problem
  • CASE PRESENTATION: A 40-year-old man was referred with signs of increases intracranial pressure and unilateral hearing problem...[readbyqxmd.com]
Hemianopsia
  • The patient’s postoperative course was uneventful but his visual field study showed a right homonymous hemianopsia.[file.scirp.org]
Retinal Pigmentation
Fussiness
  • Other symptoms you may have noticed in your child include: Headache, possibly manifested as increased fussiness in an infant Nausea and vomiting, particularly first thing in the morning Vision and eye movement problems, resulting from too much pressure[neurosurgeonsofnewjersey.com]
Loss of Initiative
  • The weight loss clinic initiates weight loss to its patients... Read more Weight Loss after Pregnancy A woman gains between 25 and 35 pounds during pregnancy, which is recommended by the doctor.[allpathy.com]
Headache
  • RESULTS: A 9-year-old boy presented with a year-long history of headaches and blindness. His neurological examination was remarkable for focal cranial nerve deficits and cerebellar signs.[ncbi.nlm.nih.gov]
  • Nausea, vomiting, headaches, ataxia, nystagmus, dizziness, and diplopia are typical consequences of hydrocephalus and are highly indicative of a tumor originating from the fourth ventricle.[symptoma.com]
  • Case Report In May 2011, a 21-year-old man with past medical history of asthma presented to us with the chief complaint of morning headache for one month. His headache was distent in nature and sometimes associated with nausea and vomiting.[file.scirp.org]
  • […] tumors of the central nervous system: Choroid plexus carcinoma (WHO grade III) Choroid atypical plexus papilloma (WHO grade II) Choroid plexus papilloma (WHO grade I) Symptoms vary depending on the size and location of the tumor and typically include headaches[en.wikipedia.org]
  • The patient was a 35-year-old woman who had headache of 3-month duration, followed by ataxia for 1month before admission. Brain CT revealed a well enhanced mass at the left cerebello-pontine angle area as well as hydrocephalus.[jkns.or.kr]

Workup

In order to make a prompt diagnosis, it is necessary to perform a thorough clinical assessment comprised of a detailed patient history and a complete physical examination. Once clinical findings point to increased intracranial pressure or hydrocephalus, imaging studies should be immediately employed. Computed tomography (CT) and magnetic resonance imaging (MRI) are the two main studies used, each describing important tumor features [1]. In the case of malignant choroid plexus tumor, CT will show a hypodense intraventricular mass that is lobulated, often containing calcifications that substantially enhance after contrast administration [4]. On MRI, papillomas appear as isointense or hypointense intraventricular masses compared to the brain tissue, while carcinomas tend to be more heterogeneous due to the invasion of adjacent brain tissue and necrosis [6]. Additional findings that point to carcinoma are hypointense or isointense T1-weighted studies and hyperintense T2-weighted studies [4]. However, a definite distinction between the two types of choroid plexus tumors is made after a histopathological examination which examines the level of mitosis, growth pattern, and the degree of necrosis [1] [2] [5]. Expression of vimentin, S-100, and cytokeratin are well-documented in choroid plexus papilloma [4].

Treatment

  • Surgery and biopsy is diagnostic, and radiotherapy has been used as the treatment of choice for choroid plexus carcinoma (CPC) and recurrent choroid plexus papilloma (CPP).[ncbi.nlm.nih.gov]
  • Learn more about different treatment options for brain tumors on our Treatment page . New approaches to treatment are currently in development. These new therapies are offered in organized research studies called clinical trials.[abta.org]
  • Your healthcare team will develop a treatment plan that is right for your child, but the general approach will be the same. The first treatment goal, regardless of whether the tumor is the papilloma or carcinoma form, is removal.[neurosurgeonsofnewjersey.com]
  • Your treatment will depend on the type of tumor you have, as well as its size and location. Doctors often use surgery to remove papilloma (Grade I) and atypical papilloma (Grade II) tumors.[aurorahealthcare.org]

Prognosis

  • Complete resection of this malignant tumor is the primary goal of treatment since it allows best chance of survival and improves the overall prognosis [1] [4] [5].[file.scirp.org]
  • Benign choroid plexus tumors - papillomas, carry very high 5-year survival rates (reaching up to 100% in some studies) with an early recognition and appropriate therapy, whereas carcinomas have a substantially worse prognosis (as low as 26% of patients[symptoma.com]
  • Treatment : palliative medical treatment, palliative ventriculo-peritoneal shunting if obstructive hydrocephalus, surgical resection, radiotherapy Prognosis : tend to invade the ventricular system, can cause drop metastasis in the cerebrospinal fluid[vetstream.com]
  • In contrast to AT/RT, prognosis of CRINET seems to be relatively favorable [ 81 ].[basicmedicalkey.com]
  • Indications for Surgery Treatment of hydrocephalus Diagnosis of the lesion Optimization of treatment: The goal of surgery is to remove a choroid plexus papilloma, thereby curing it, or, in the case of choroid plexus carcinoma, to achieve the best prognosis[ispn.guide]

Etiology

  • Pathogenesis Etiology Arise from the choroid plexus of the lateral, third or fourth ventricles. Most commonly arise in the fourth ventricle. Less often in the choroid plexuses of the lateral and third ventricles.[vetstream.com]
  • One theory of the etiology involves the presence of simian vacuolating virus No. 40 (SV40)–related viral DNA. Pathology: They may arise wherever a choroid plexus exists. Tumoral distribution varies between pediatric and adult patients.[thamburaj.com]
  • Imaging On CT, most lesions appear as isodense/hyperdense well-defined lobulated mass with intense homogeneous enhancement.[ 4 26 ] Microhemorrhages and calcifications are believed to be the etiology for the hyperdensity.[surgicalneurologyint.com]
  • There is no significant sex predilection. [1] Etiology 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.[emedicine.medscape.com]

Epidemiology

  • The Surveillance Epidemiology and End Results (SEER) Database from 1978 to 2009 was queried to define population-based outcomes for all patients with CPTs.[scholars.northwestern.edu]
  • Surveillance, Epidemiology and ERP (1984) Code Manual: the SEER Program. Bethesda, MD 26. Surveillance, Epidemiology and ERP (1988) The SEER Program Code Manual. Bethesda, MD 27.[springermedizin.de]
  • The use of the SEER (Surveillance, Epidemiology and End Results) database allows for the analysis of the relationship between prognostic factors and survival.[karger.com]
  • Garcia-Fructuoso Neurocirugía. 2015; 8 Extraventricular Intraparenchymal Choroid Plexus Tumors in Cerebral Hemisphere: A Series of 6 Cases Qichao Qi,Shilei Ni,Xudong Zhou,Bin Huang,Xingang Li World Neurosurgery. 2015; 84(6): 1660 9 Pediatric choroid plexus tumors: epidemiology[neurologyindia.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology Choroid plexus cells arise from a primitive medullary epithelium and are related to ependymal cells. Choroid plexus tumors may be either papillomas, which are more common, or carcinomas.[vetstream.com]
  • […] rarely described, including the cerebellopontine angle, cerebellomedullary cistern, suprasellar cistern, foramen magnum, and spinal subarachnoid space. 58 – 61 There is a correlation of ventricular location with age, however, that is without an obvious pathophysiologic[clinicalgate.com]

Prevention

  • Radiation therapy : This process destroys cancer cells and prevents others from growing.[aurorahealthcare.org]
  • In these instances, chemotherapy or radiation therapy — sometimes both — are used to kill the abnormal cells and prevent new growth.[wisegeek.com]
  • Knowing what these side effects are can help you and your child prepare for, and, in some cases, prevent these symptoms.[danafarberbostonchildrens.org]
  • However, if your child has a choroid plexus papilloma, the tumor itself may be compressing other structures or preventing the flow of CSF, or it may be overproducing CSF. Over 80 percent of choroid plexus tumors are papillomas.[neurosurgeonsofnewjersey.com]
  • Choroid plexus cyst information including symptoms, diagnosis, misdiagnosis, treatment, causes, patient stories, videos, forums, prevention, and prognosis. Old male presented with.[accumulatedmanage.gq]

References

Article

  1. Gopal P, Parker JR, Debski R, Parker JC Jr. Choroid plexus carcinoma. Arch Pathol Lab Med. 2008;132(8):1350-1354.
  2. Wolff JEA, Sajedi M, Brant R, Coppes MJ, Egeler RM. Choroid plexus tumours. Br J Cancer. 2002;87(10):1086-1091.
  3. Jaiswal S, Vij M, Mehrotra A, et al. Choroid plexus tumors: A clinico-pathological and neuro-radiological study of 23 cases. Asian J Neurosurg. 2013;8(1):29-35.
  4. Sethi D, Arora R, Garg K, Tanwar P. Choroid plexus papilloma. Asian J Neurosurg. 2017;12(1):139-141.
  5. Kishore S, Negi G, Meena H, Anuradha K, Pathak PV, Bansal K. Choroid plexus carcinoma in an adult. J Neurosci Rural Pract. 2012;3(1):71-73.
  6. Koeller KK, Sandberg GD. Armed Forces Institute of Pathology. From the archives of the AFIP. Cerebral intraventricular neoplasms: radiologic-pathologic correlation. Radiographics. 2002;22(6):1473-1505.

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Last updated: 2019-06-28 11:12