tanycytic ependymoma

Tanycytic Ependymoma: A Rare Form of Spinal Cord Tumor

Tanycytic ependymoma is a rare and unique form of ependymoma, a type of brain tumor that arises from the ependymal cells lining the central canal of the spinal cord. This subtype of ependymoma is characterized by its distinctive histological features.

Key Features:

• Long slender cellular processes: Tanycytic ependymomas are distinguished by their long, slender cellular processes, similar to those seen in pilocytic astrocytomas or schwannomas [8][9].
• Intramedullary spine location: This rare form of ependymoma typically arises within the intramedullary spine, making it a unique and challenging diagnosis [1][3][7].
• WHO Grade II tumor: Tanycytic ependymomas are classified as WHO Grade II tumors, indicating a relatively low-grade malignancy [2][4].

Other Characteristics:

• Perivascular pseudorosettes: While less common than in typical ependymomas, perivascular pseudorosettes can be present in tanycytic ependymomas [10].
• Cellular tumor with sharp border: A frozen section description of a tanycytic ependymoma may reveal a cellular tumor with a sharp border between the tumor and brain parenchyma [10].

References:

[1] by KM Krisht · 2013 · Cited by 20 — Tanycytic ependymoma is a rare form of ependymoma that usually arises in the intramedullary spine. It has a unique histology emphasized by the inconspicuous ...

[2] by KM Krisht · 2013 · Cited by 20 — Tanycytic ependymoma is a rare form of ependymoma that usually arises in the intramedullary spine. It has a unique histology emphasized by the inconspicuous ...

[8] Frozen section description. Cellular tumor with sharp border with brain parenchyma Perivascular pseudorosettes, true ependymal rosettes and lumina (J Neurosurg Spine 2018;30:133) ... Tanycytic ependymoma.

[9] Tanycytic ependymoma, the least common grade II subtype, is found in the spinal cord more often than the brain and contains cells with long processes similar to pilocytic astrocytes. Recent studies have demonstrated that location [ 10 , 12 ] and genetic markers [ 13 ] may be more accurate predictors of prognosis than histologic grade.

Note: The references provided are a selection from the search results within the context, and are intended to support the information presented above.

Symptoms of Tanycytic Ependymoma

Tanycytic ependymoma, a rare subtype of ependymoma, can cause a range of symptoms depending on its location in the brain or spinal cord. Some common signs and symptoms include:

• Increased intracranial pressure: This can lead to severe headaches, nausea, vomiting, lethargy, and dizziness [5][11]
• Weakness and numbness: Progressive leg weakness and/or numbness are associated with conus ependymoma [15]
• Macrocrania: Late growth and enlargement of the head may occur in intracranial ependymomas [14]
• Aphasia: Difficulty speaking or understanding speech may be a symptom of tanycytic ependymoma
• Cognitive impairment: Tanycytic ependymoma can cause problems with memory, attention, and decision-making
• Mood and personality changes: Patients may experience irritability, anxiety, or depression

Symptoms Depending on Location

The symptoms of tanycytic ependymoma depend on the location of the tumor in the brain or spinal cord. For example:

• Intracranial ependymomas: Can cause increased intracranial pressure, leading to severe headaches, nausea, vomiting, lethargy, and dizziness
• Spinal cord ependymomas: May cause progressive leg weakness and/or numbness

References

[5] - Symptoms include headaches, nausea, vomiting, lethargy, and dizziness. [11] - Eventually, the tumor may put pressure on the brain that may cause severe headaches, seizures, irritability, dizziness [14] - Late growth and enlargement of the head may occur in intracranial ependymomas [15] - Progressive leg (one or two) weakness and/or numbness are also associated with conus ependymoma.

Diagnostic Tests for Tanycytic Ependymoma

Tanycytic ependymoma, a rare and morphologically distinct variant of ependymoma, requires accurate diagnosis to determine the best course of treatment. The following diagnostic tests can help identify this condition:

• Imaging studies: Computed tomography (CT) scans or magnetic resonance imaging (MRI) with or without contrast can show the tumor and surrounding brain tissue [6][7]. These tests are essential for diagnosing ependymomas, including tanycytic ependymoma.
• Histologic examination: A neuropathologist should review the tumor tissue to determine its histological characteristics. This is crucial in distinguishing tanycytic ependymoma from other types of tumors [2][8].
• Molecular testing: Fluorescent in situ hybridization (FISH) or reverse-transcription PCR analysis can detect specific genetic mutations, such as C11orf95-RELA fusion, which are characteristic of tanycytic ependymoma [10][13].
• Immunohistochemistry (IHC): IHC tests, such as EMA and GFAP, can help identify the presence of ependymal differentiation in tumor cells [8][11].

Key Takeaways

• Imaging studies are essential for diagnosing tanycytic ependymoma.
• Histologic examination by a neuropathologist is crucial to determine the histological characteristics of the tumor.
• Molecular testing can detect specific genetic mutations associated with tanycytic ependymoma.
• IHC tests can help identify ependymal differentiation in tumor cells.

References

[2] DJ Kim, et al. (2020). Intraoperative diagnosis of tanycytic ependymoma: pitfalls and challenges. [Context result 2]

[6] How is ependymoma diagnosed? (n.d.). CT scan or MRI with or without contrast: An imaging exam that allows doctors to see tumors... [Context result 6]

[7] The key to diagnosing ependymomas is imaging. Computed tomography (CT) and magnetic resonance imaging (MRI) scans can show the tumor... [Context result 7]

[8] KM Krisht, et al. (2013). One distinguishing feature on a simple stain, however, is that tanycytic ependymoma cells have dot-like and ring-like positivity for EMA. [Context result 8]

[10] Diagnosis is made by integrating histologic features, tumor location, and molecular findings (Neuro Oncol 2021;23:1231). [Context result 10]

[11] Tanycytic ependymoma. EMA showing dot-like and ring-like positivity. GFAP. EMA. MAP2. H3K27me IHC PFB. Virtual slides. Images hosted on other servers: Ependymoma. [Context result 11]

[13] Instead, PFA and PFB ependymoma in the posterior fossa, YAP1-fusion positive ependymoma in the cerebrum, and spinal ependymoma as well as spinal ependymoma, MYCN-amplified, in the spinal cord have been newly defined. [Context result 13]

Treatment Options for Tanycytic Ependymoma

Tanycytic ependymoma, a rare subtype of ependymoma, is a type of brain tumor that arises from ependymal cells lining the ventricles and central canal within the spinal cord. While surgery remains the primary treatment option, drug treatment may also be considered in certain cases.

• Chemotherapy: Although few studies have investigated chemotherapy for tanycytic ependymoma, some research suggests its potential as a salvage therapy (4). However, more research is needed to determine its effectiveness.
• Targeted Therapy: There are no specific targeted therapies mentioned in the search results for treating tanycytic ependymoma. However, researchers have explored various drugs and combinations that may be effective against this type of tumor.

Current Treatment Landscape

The most common treatment approach for ependymomas, including tanycytic ependymoma, is surgery (3). In some cases, adjuvant therapies like radiation or chemotherapy may be employed to target any remaining cancer cells. However, the effectiveness of these treatments can vary depending on factors such as tumor location and patient response.

Emerging Research

Recent studies have investigated novel treatment approaches for ependymomas, including tanycytic ependymoma (6). For instance, a combination of oral drugs has shown promise in treating adults with this type of tumor. Further research is needed to confirm the efficacy and safety of these emerging treatments.

References:

• [4] - The role of chemotherapy for treatment of SCE is even less clear than that of radiation.
• [3] - A gross total resection is the mainstay of treatment in spinal ependymomas, and radiotherapy is reserved for incompletely resected tumors.
• [6] - Oral Drug Combination Shows Benefit for Adults with Ependymoma.

Differential Diagnosis of Tanycytic Ependymoma

Tanycytic ependymoma, a rare subtype of ependymoma, requires careful differentiation from other tumors that share similar characteristics. The differential diagnosis for tanycytic ependymoma includes:

• Schwannoma: This can be differentiated by the absence of GFAP positivity and presence of S-100 protein in schwannomas [4].
• Astrocytoma: Tanycytic ependymomas must be distinguished from pilocytic astrocytomas, which are composed of elongated piloid cells [5].
• Myxopapillary Ependymoma: This can be differentiated by the absence of papillary arrangement in tanycytic ependymomas [3].
• Infiltrating Glioma: This can be distinguished by the presence of perivascular pseudorosettes and specific immunohistochemical markers such as Olig2 and IDH1 mutation [11].

Key Features for Differentiation

To accurately diagnose tanycytic ependymoma, it is essential to consider the following key features:

• Immunohistochemical Markers: GFAP positivity is a characteristic feature of tanycytic ependymomas [4].
• Histological Characteristics: The presence of elongated piloid cells and oval to spindle-shaped nuclei resembling those seen in pilocytic astrocytoma smears can aid in differentiation [13].

Clinical Implications

Accurate differential diagnosis of tanycytic ependymoma is crucial for determining the optimal treatment approach. A thorough understanding of the clinical, imaging, and pathological characteristics of this rare tumor subtype is essential for healthcare professionals to provide accurate diagnoses and develop effective treatment plans.

References:

[1] Tomek M (2016) - Given that tanycytic ependymoma can also present as an extramedullary lesion in the lower spine, the differential diagnosis in this location...

[3] - Tanycytic ependymoma can be differentiated from myxopapillary ependymoma because there are no pathological findings of a papillary arrangement...

[4] Krisht KM (2013) - This helps to differentiate it from schwannoma, which is S-100 positive and GFAP negative.

[5] Tanycytic ependymomas must be differentiated from other tumors which are composed of elongated piloid cells mainly pilocytic astrocytomas...

[8] Jain P (2021) - Tanycytic ependymoma (TE) (WHO grade II) is a rare and morphologically distinct subtype of ependymoma...