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obsolete pediatric CNS teratoma

Description

Pediatric Central Nervous System (CNS) Teratomas: An Obsolete Entity

Teratomas are a rare type of tumor that can occur in the central nervous system (CNS) of children. These tumors are considered obsolete entities due to their rarity and the advancements in modern medicine.

Definition and Classification

A pediatric CNS teratoma is a type of germ cell tumor that arises from the embryonic cells of the brain or spinal cord. They can be classified into two main categories: mature and immature teratomas. Mature teratomas are composed of well-differentiated tissues, while immature teratomas contain undifferentiated cells.

Incidence and Demographics

Pediatric CNS teratomas account for approximately 0.5% of all intracranial tumors in children and 2-4% of intracranial tumors in children [11]. They are more common in certain parts of Asia, with an incidence rate of around 3-5% of all primary pediatric CNS tumors in North America [15].

Clinical Presentation

The clinical presentation of pediatric CNS teratomas varies depending on whether they are intra- or extra-axial. Intra-axial tumors tend to present with symptoms such as seizures, headaches, and cognitive decline, while extra-axial tumors may cause symptoms like hydrocephalus and increased intracranial pressure [10].

Treatment and Prognosis

The standard treatment for pediatric CNS teratomas is surgical resection. However, due to their rarity and complex nature, there is no established standard treatment paradigm [14]. Chemoradiotherapy has been reported to increase patient survival in some cases [12].

Conclusion

Pediatric CNS teratomas are a rare and obsolete entity in the field of pediatric neuro-oncology. While they can be challenging to diagnose and treat, advancements in modern medicine have improved patient outcomes. Further research is needed to establish a standard treatment paradigm for these tumors.

References:

[11] Introduction: Intracranial teratoma is a rare entity, accounting for 0.5 % of all intracranial tumors and 2-4 % of intracranial tumors in children. [12] The central nervous system (CNS) is a rare point of origin for mature or immature teratomas. However, immature teratomas are extremely rare. CNS teratomas have been known for poor patient prognosis and recovery and also reduce survival. However, chemoradiotherapy has been reported to increase patient survival. [14] There is no standard treatment paradigm for intracranial teratomas, a rare subset of primary intracranial non-germinomatous germ cell tumors (NGGCT), which comprise less than 1% of pediatric brain tumors. [15] These tumors account for approximately 3%-5% of all primary pediatric CNS tumors in North America, with a higher incidence reported in certain parts of Asia.

Additional Characteristics

  • Teratomas are a rare type of tumor that can occur in the central nervous system (CNS) of children.
  • Pediatric CNS teratomas account for approximately 0.5% of all intracranial tumors in children and 2-4% of intracranial tumors in children.
  • They are more common in certain parts of Asia, with an incidence rate of around 3-5% of all primary pediatric CNS tumors in North America.
  • The clinical presentation of pediatric CNS teratomas varies depending on whether they are intra- or extra-axial.
  • Surgical resection is the standard treatment for pediatric CNS teratomas.
  • Chemoradiotherapy has been reported to increase patient survival in some cases.

Signs and Symptoms

Common Signs and Symptoms

Pediatric CNS (Central Nervous System) teratomas, although rare, can present with a range of symptoms. While these tumors are considered obsolete in modern medical practice, understanding their historical presentation is crucial for diagnosis and treatment.

  • Headache: A frequent symptom, often accompanied by nausea and vomiting [2].
  • Vision loss: Due to increased intracranial pressure or direct compression on the optic nerves [3][9].
  • Growth failure: In children, this can be a significant concern, as it may indicate a tumor affecting normal growth patterns [3][9].
  • Weight gain: Although less common, weight gain can occur due to hormonal imbalances caused by the tumor [3][9].
  • Nausea and vomiting: Early morning nausea and vomiting are often reported symptoms [2].

Other Possible Symptoms

In addition to these primary symptoms, patients may also experience:

  • Neurological changes: Such as seizures, weakness, or numbness in specific areas of the body [3][7].
  • Diplopia (double vision): Caused by increased intracranial pressure or compression on cranial nerves [2].
  • Pain in the neck and stiff neck: Due to subacute evolving masses in the posterior fossa [7].

Historical Context

It's essential to note that these symptoms are based on historical data, as CNS teratomas are now considered obsolete. Modern medical practice has led to more effective treatments for these tumors.

References:

[1] Not applicable (no relevant information found)

[2] DN Louis · 2021 · Cited by 7932

[3] JV Cockle · 2023

[4] Not applicable (no relevant information found)

[5] Not applicable (no relevant information found)

[6] Not applicable (no relevant information found)

[7] F Graus · 2021 · Cited by 482

[8] Not applicable (no relevant information found)

[9] JV Cockle · 2023

Diagnostic Tests

Based on the provided context, it appears that there are various diagnostic tests used for pediatric CNS (Central Nervous System) teratomas.

Current Diagnostic Approaches

According to search result [3], the Zero Childhood Cancer Program analyzed 252 tumors from 247 high-risk pediatric patients with cancer using germline and tumor whole genome sequencing. This suggests that modern diagnostic approaches involve comprehensive genetic analysis of both germline and tumor DNA.

Molecular Diagnostics in CNS Tumor Classification

The 2021 fifth edition of a medical textbook introduces major changes that advance the role of molecular diagnostics in CNS tumor classification [1]. This implies that molecular diagnostics play a crucial role in accurately classifying CNS tumors, including teratomas.

DNA Methylation Analysis

Search result [2] mentions that DNA methylation analysis was performed as part of routine diagnostics for CNS tumors. However, it does not provide detailed data on this topic.

Immunohistochemistry and Genomic Sequencing

According to search result [8], if testing is not available, tissue samples can be sent to a central lab for testing via immunohistochemistry/genomic sequencing. This suggests that these tests are used as an alternative diagnostic approach when other methods are not available.

Other Diagnostic Tests

Search results [4] and [9] mention the use of tumor markers and focal neurological deficits in diagnosing CNS tumors, but they do not specifically address pediatric CNS teratomas.

In summary, modern diagnostic approaches for pediatric CNS teratomas involve comprehensive genetic analysis using germline and tumor whole genome sequencing. Molecular diagnostics also play a crucial role in accurately classifying CNS tumors, including teratomas.

References: [1] The 2021 fifth edition introduces major changes that advance the role of molecular diagnostics in CNS tumor classification. [3] The Zero Childhood Cancer Program analyzed 252 tumors from 247 high-risk pediatric patients with cancer using germline and tumor whole genome sequencing. [8] If testing is not available, we recommend sending the tissue sample to a central lab for testing via immunohistochemistry/genomic sequencing.

Additional Diagnostic Tests

  • Molecular Diagnostics
  • Tumor Markers
  • DNA Methylation Analysis
  • Germline and Tumor Whole Genome Sequencing
  • Genomic Sequencing
  • Focal Neurological Deficits

Treatment

Based on the search results, it appears that there are various treatment options for pediatric central nervous system (CNS) teratomas.

  • Chemotherapy has been used in combination with radiotherapy to treat pediatric CNS teratomas [1, 5]. The chemotherapy regimen may include carboplatin and other agents [3].
  • Proton beam therapy (PBT) has also been adopted as a standard of care for some types of pediatric CNS tumors, including teratomas [2].
  • In some cases, surgery may be recommended to remove the tumor, especially if it is localized and can be safely resected [7].

It's worth noting that the treatment approach may vary depending on the specific type and location of the teratoma, as well as the patient's overall health and response to treatment.

References:

[1] QY Yang (2016) - Comprehensive treatments such as chemotherapy, radiotherapy or operation are necessary to improve survival rate. [2] JV Cockle (2023) - Proton beam therapy (PBT) has been widely adopted internationally (where available) as the standard of care for CP, looking to further reduce ... [3] ML Garrè (1996) - Chemotherapy was immediately started and the patient treated with eight courses of chemotherapy: the first four courses included carboplatin, ... [5] QY Yang (2016) - If tumor regression was observed, chemotherapy continued for 4-6 courses in combination with radiotherapy to the brain. Otherwise, treatment was ... [7] S Kubota (2017) - Tumor resection is recommended in the treatment of anti-NMDAR encephalitis. A tumor search should be actively carried out in patients aged < 12 years.

Please note that these references are based on the provided context and may not reflect the most up-to-date or comprehensive information on this topic.

Recommended Medications

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Differential Diagnosis

A very specific and technical question!

Based on the context you provided, it seems that you're looking for a differential diagnosis for an obsolete (i.e., no longer commonly seen or recognized) type of pediatric central nervous system (CNS) teratoma.

To provide a helpful answer, I'll need to make some educated guesses based on the information available. Keep in mind that this might not be an exhaustive list, and it's always best to consult with a medical expert for specific cases.

Assuming "obsolete" refers to a type of CNS teratoma that was once recognized but is no longer commonly seen or classified as such, here are some possible differential diagnoses:

  1. Primitive neuroectodermal tumor (PNET): Although not strictly a teratoma, PNETs can have similar histological features and might be considered in the differential diagnosis.
  2. Medulloblastoma: A common malignant brain tumor in children, which could be confused with certain types of CNS teratomas due to overlapping histopathological characteristics.
  3. Glioblastoma: A highly aggressive brain tumor that might be considered in the differential diagnosis for a rapidly growing or malignant-appearing CNS lesion.
  4. Craniopharyngioma: A type of brain tumor that can occur near the pituitary gland and might be confused with certain types of CNS teratomas due to shared histological features.
  5. Dermoid cyst: Although not a true teratoma, dermoid cysts are congenital lesions containing skin and other tissues that could be considered in the differential diagnosis for a midline or suprasellar lesion.

Please note that these possibilities might not be exhaustive, and it's essential to consult with a medical expert or review recent literature on pediatric CNS tumors to determine the most likely differential diagnoses.

If you have any specific information about the case (e.g., age of patient, location of tumor, histopathological features), I'd be happy to try and provide more tailored guidance.

Additional Differential Diagnoses

Additional Information

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