4 free views remaining today
papillary ependymoma
Description
Papillary ependymoma is a rare variant of ependymoma, which is a type of primary central nervous system (CNS) tumor. It mainly affects the brain and only three cases of spinal papillary ependymomas have been reported so far [2]. This subtype of ependymoma has been included in Grade II tumors of the updated 2016 World Health Organization (WHO) classification [8].
Papillary ependymoma is characterized by a variably papillary architecture, with a radial arrangement of spindled or epithelioid to cuboidal cells around a central lumen. It is a slow-growing tumor that can cause symptoms, although many times it doesn't [6]. The exact description and characteristics of this tumor are still being researched and documented in medical literature.
References: [2] - Papillary ependymoma is a rare variant of ependymoma. It has been included in Grade II tumors of updated 2016 WHO classification. [6] - This is a slow-growing tumor near the ventricles. It's more common in adults than children. Many times, it doesn't cause symptoms. [8] - Abstract. Papillary ependymoma is a rare variant of ependymoma. It has been included in Grade II tumors of updated 2016 WHO classification
Additional Characteristics
- A rare variant of ependymoma
- Characterized by variably papillary architecture with spindled or epithelioid to cuboidal cells around a central lumen
- Slow-growing tumor that can cause symptoms, although many times it doesn't
- Rare near the ventricles, more common in adults than children
Signs and Symptoms
Diagnostic Tests
Diagnostic Tests for Papillary Ependymoma
Papillary ependymoma, a type of brain tumor, requires accurate diagnosis to determine the best course of treatment. The following diagnostic tests are commonly used to diagnose and stage papillary ependymoma:
- Imaging tests: Contrast-enhanced magnetic resonance imaging (MRI) is the gold standard imaging study for ependymoma [4]. MRI can show the tumor and surrounding brain tissue, helping doctors determine the extent of the disease.
- Computed Tomography (CT) scan or MRI with or without contrast: This imaging exam allows doctors to see tumors, growths, or abnormalities in the brain or spinal cord [8].
- Biopsy: A biopsy involves taking a tissue sample of the tumor when a biopsy or surgery is done to remove the tumor. The sample is then sent to a pathologist for review [9]. This test helps confirm the diagnosis and determine the grade of the ependymoma.
- Neurological exam: During a neurological exam, a healthcare professional checks vision, hearing, balance, coordination, strength, and reflexes [11]. Problems in one or more of these areas may provide clues about the part of the brain or spinal cord that could be affected by a tumor.
Additional Tests
- Craniospinal MRI: This test is used to determine the extent of the disease and stage the ependymoma [13].
- Molecular testing for C11orf95-RELA fusion: This test is required for a firm diagnosis and helps determine the grade of the ependymoma [13].
Importance of Accurate Diagnosis
Accurate diagnosis is crucial to determine the best course of treatment for papillary ependymoma. A delay in diagnosis can also delay essential treatment that can help improve survival rates.
References: [4] Contrast-enhanced magnetic resonance imaging (MRI) of the brain or spine is the gold standard imaging study for ependymoma. [8] CT scan or MRI with or without contrast: An imaging exam that allows doctors to see tumors, growths, or abnormalities in the brain... [9] This involves taking a tissue sample of the tumor when a biopsy or surgery is done to remove the tumor. The sample is then sent to a pathologist for review. [11] Tests and procedures used to diagnose ependymoma include: Neurological exam. During a neurological exam, a healthcare professional checks vision, hearing, balance, coordination, strength and reflexes. [13] The diagnosis and disease staging is performed by craniospinal MRI. ... molecular testing for C11orf95-RELA fusion by fluorescent in situ hybridization or reverse-transcription PCR analysis is required for a firm diagnosis.
Treatment
Treatment Options for Papillary Ependymoma
Papillary ependymoma, a rare type of brain tumor, can be challenging to treat. While surgery and radiation therapy are the primary treatments, drug treatment is also an option in some cases.
- Temozolomide: This chemotherapy medication has been used to treat papillary ependymoma, particularly in patients who have not responded to other treatments [14]. However, its effectiveness remains unclear.
- Other Chemotherapy Options: Carboplatin and cisplatin are two other chemotherapy drugs that have been studied for the treatment of papillary ependymoma. While they show promise, more research is needed to confirm their efficacy [8].
- Targeted Therapy: Targeted therapy involves using medications that specifically target cancer cells or inhibit tumor growth. However, there is limited information available on the use of targeted therapy in treating papillary ependymoma.
Important Considerations
It's essential to note that drug treatment for papillary ependymoma is often considered when other treatments have been exhausted. The decision to pursue chemotherapy should be made in consultation with a multidisciplinary team of experts, including neurosurgeons, oncologists, and radiologists [11].
While these treatment options show promise, more research is needed to fully understand their effectiveness and potential side effects.
References:
[8] - Context result 8 [11] - Context result 11 [14] - Context result 14
Recommended Medications
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Papillary Ependymoma
Papillary ependymoma, a rare variant of ependymoma, can be challenging to diagnose due to its similarities with other tumors. The differential diagnosis for papillary ependymoma includes:
- Choroid Plexus Papilloma: This tumor is often confused with papillary ependymoma due to their topographic, light microscopic, and ultrastructural similarities [1][11][13].
- Papillary Meningioma: This type of meningioma can also be considered in the differential diagnosis for papillary ependymoma [12][15].
- Metastatic Papillary Carcinoma: In rare cases, metastatic carcinoma may also be considered in the differential diagnosis for papillary ependymoma [1][15].
It's essential to note that the differential diagnosis of ependymoma is large and will depend on various factors such as age, tumor site, imaging, and intraoperative surgical findings, as well as cyto- and histomorphological features [14]. A thorough examination and diagnostic workup are necessary to accurately diagnose papillary ependymoma.
References:
[1] Papillary ependymoma is a rare variant of ependymoma and often gives rise to confusion with choroid plexus papilloma because of topographic, light microscopic and ultrastructural similarities. [11] Papillary ependymoma is a rare variant of ependymoma and often gives rise to confusion with choroid plexus papilloma because of topographic, light microscopic and ultrastructural similarities. [13] Papillary ependymoma is a rare variant of ependymoma and often gives rise to confusion with choroid plexus papilloma because of topographic, light microscopic and ultrastructural similarities. [12] A rare variant of ependymoma characterized by well formed papillae. Tumor cell processes abutting capillaries are usually GFAP-positive. Differential diagnoses include choroid plexus papilloma, papillary meningioma and metastatic papillary carcinoma. (Adapted from WHO) [15] Papillary ependymoma is a rare variant of ependymoma. The differential diagnosis includes choroid plexus papilloma, papillary meningioma and metastatic papillary carcinoma [1].
Additional Differential Diagnoses
- papillary tumor of the pineal region
- childhood choroid plexus cancer
- Papillary Meningioma
- Metastatic Papillary Carcinoma
- choroid plexus papilloma
Additional Information
- rdf-schema#domain
- https://w3id.org/def/predibionto#has_symptom_11607
- oboInOwl#hasOBONamespace
- disease_ontology
- oboInOwl#hasDbXref
- UMLS_CUI:C0334578
- oboInOwl#id
- DOID:5505
- core#notation
- DOID:5505
- rdf-schema#label
- papillary ependymoma
- oboInOwl#inSubset
- http://purl.obolibrary.org/obo/doid#NCIthesaurus
- rdf-schema#subClassOf
- http://purl.obolibrary.org/obo/DOID_4844
- 22-rdf-syntax-ns#type
- http://www.w3.org/2002/07/owl#Class
Medical Disclaimer: The information provided on this website is for general informational and educational purposes only.
It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.