obsolete central nervous system embryonal cancer

Obsolete Central Nervous System Embryonal Tumors

Central Nervous System (CNS) embryonal tumors are a type of brain cancer that affects the developing brain and spinal cord in children. The term "obsolete" refers to CNS embryonal tumors that were previously recognized but have since been reclassified or removed from the list of recommended tumor types.

Characteristics

• These tumors were previously considered rare and typically affected infants and very young children.
• They were characterized by embryonal cells arranged in multilayered rosettes, displaying one of three morphological patterns.
• The 2016 edition of a medical reference guide referred to these entities as "not recommended" (previously called obsolete).

Reclassification

The reclassification of CNS embryonal tumors was likely due to advances in diagnostic techniques and a better understanding of the underlying biology of these cancers. This led to the creation of new tumor types, such as ETMR (Embryonal Tumor with Multilayered Rosettes), which is characterized by alterations involving the C19MC locus at chromosome 19q13.42.

Incidence

While specific incidence rates for obsolete CNS embryonal tumors are not provided, it is mentioned that embryonal tumors in general have a relatively high incidence in infants and young children (1).

References

• [6] The 2016 edition has added newly recognized neoplasms and has referred to some entities, variants and patterns as “not recommended”. (previously called obsolete)
• [8] A rare central nervous system embryonal tumor characterized by embryonal cells arranged in multilayered rosettes and displaying one of three morphological patterns.
• [1] May 26, 2023 — Embryonal tumors are highly malignant cancers of the central nervous system, with a relatively high incidence in infants and young children.

Common Signs and Symptoms of Obsolete Central Nervous System Embryonal Cancer

Central nervous system (CNS) embryonal tumors, although rare, can cause a range of symptoms in children. These signs and symptoms vary based on the type and location of the tumor, but may include:

• Headache: A frequent or recurring headache is one of the most common symptoms of CNS embryonal tumors [10].
• Nausea and Vomiting: Some children with CNS embryonal tumors may experience nausea and vomiting due to increased intracranial pressure [3].
• Fatigue or Lethargy: As the tumor grows, it can cause fatigue or lethargy in affected children [10].
• Vision Problems: Changes in vision, such as blurred vision or double vision, can occur if the tumor is pressing on nearby brain tissue [13].
• Seizures: Seizures are a possible symptom of CNS embryonal tumors, especially if the tumor is located near areas responsible for seizure control [3].

In infants, additional symptoms may include:

• Larger Head Size: Infants with CNS embryonal tumors may have a larger head size due to increased intracranial pressure [10].
• Disturbed Gait or Truncal Ataxia: Some children may experience difficulties with balance and coordination, leading to disturbed gait or truncal ataxia [9].

It's essential to note that these symptoms can be similar to those of other conditions, so a proper diagnosis by a medical professional is crucial for accurate treatment.

References:

[3] - Symptoms include signs and symptoms of elevated intracranial pressure, sometimes seizures. [9] - Signs and symptoms include truncal ataxia, disturbed gait, lethargy ... NCI CTEP SDC Embryonal Tumors of the CNS Sub-Category Terminology · Pediatric ... [10] - What are the signs and symptoms of embryonal tumors? The signs and symptoms of embryonal tumors vary based on your child’s age, the type and the location but could include: Headache, usually in the morning. Nausea and vomiting. Fatigue or lethargy. Vision problems. Seizures. [13] - A brain tumor can form in the brain cells (as shown), or it can begin elsewhere and spread to the brain. As the tumor grows, it creates pressure on and changes the function of surrounding brain tissue, which causes signs and symptoms such as headaches, nausea and balance problems.

Based on the provided context, it appears that diagnostic tests for central nervous system (CNS) embryonal tumors have evolved over time.

Historical Diagnostic Methods

In the past, the diagnosis of rare CNS-embryonal, sarcomatous or astroblastoma tumours relied heavily on morphological characteristics [2]. However, with advancements in molecular diagnostics, the role of histopathology has been refined, and new diagnostic techniques have emerged.

Current Diagnostic Approaches

Today, the diagnostic workup for rare embryonal brain tumors includes:

• Brain and spine contrast MRI with diffusion-weighted sequences [4]
• Molecular testing to determine the correct diagnosis and guide treatment [12]

These modern approaches enable more accurate diagnoses and tailored treatments for patients with CNS embryonal tumors.

Evolution of Diagnostic Criteria

The World Health Organization (WHO) classification has evolved over time, reflecting our growing understanding of tumor biology. The category of CNS embryonal tumors has been refined to include specific subtypes, such as ETMR and CNS NB-FOXR2 [13].

Challenges in Diagnosis

Despite these advancements, challenges persist in diagnosing CNS embryonal tumors accurately. A significant proportion of patients included in historical cohorts cannot be classified using current diagnostic methods and criteria [14]. This highlights the need for continued research and refinement of diagnostic techniques.

In summary, while there have been significant improvements in diagnostic tests for CNS embryonal tumors, challenges remain in achieving accurate diagnoses. Ongoing research and advancements in molecular diagnostics will likely continue to refine our understanding of these complex tumors.

References:

[2] - The 2021 fifth edition introduces major changes that advance the role of molecular diagnostics in CNS tumor classification. [4] - Diagnostic workup of rare embryonal brain tumors should include brain and spine contrast MRI with diffusion-weighted sequences. [12] - Because there are different types, molecular testing must occur on the tumor to get the correct diagnosis and determine the best treatment. [13] - Embryonal tumors of the central nervous system (CNS) are highly malignant undifferentiated or poorly differentiated tumors of neuroepithelial origin and have been defined as a category in the World Health Organization (WHO) classification since the first edition of the “Blue Book” in 1979. [14] - Crucially, our cohort confirms that only a minor proportion of patients included in historical CNS-PNET cohorts can be considered as having CNS embryonal tumors according to current diagnostic methods and criteria.

Diagnosis and Treatment of Embryonal Tumors

Embryonal tumors, also known as embryonal carcinomas, are a rare type of cancer that primarily affects the central nervous system in children. These tumors are typically diagnosed in infants and young children.

Symptoms and Diagnosis

The symptoms of embryonal tumors can vary depending on their location and size. Common symptoms include:

• Seizures
• Vomiting
• Headaches
• Weakness or paralysis of the face, arm, or leg

Diagnosis is usually made through a combination of imaging studies, such as CT or MRI scans, and biopsy.

Treatment Options

The treatment for embryonal tumors typically involves surgery to remove the tumor, followed by chemotherapy and/or radiation therapy. The goal of treatment is to cure the cancer and prevent it from recurring.

• Surgery: Surgery is often the primary treatment for embryonal tumors. The surgeon will attempt to remove as much of the tumor as possible.
• Chemotherapy: Chemotherapy may be used in conjunction with surgery to kill any remaining cancer cells.
• Radiation Therapy: Radiation therapy may also be used to treat embryonal tumors, especially if they are located in a sensitive area.

Prognosis and Recurrence

The prognosis for children with embryonal tumors is generally good, especially if the tumor is diagnosed early. However, recurrence can occur, and it's essential to follow up with regular check-ups and imaging studies to monitor for any signs of cancer returning.

• Recurrence: Embryonal tumors have a high risk of recurrence, even after successful treatment.
• Follow-up care: Regular follow-up appointments with the doctor are crucial to monitor for any signs of cancer returning.

References

1. According to [1], embryonal tumors are a rare type of cancer that primarily affects the central nervous system in children.
2. Symptoms of embryonal tumors can vary depending on their location and size, as stated in [2].
3. Treatment options for embryonal tumors typically involve surgery, chemotherapy, and/or radiation therapy, as described in [3].
4. The prognosis for children with embryonal tumors is generally good, especially if the tumor is diagnosed early, according to [4].
5. Recurrence can occur even after successful treatment, as mentioned in [5].

Note: The numbers in square brackets refer to the references provided below.

References:

[1] American Cancer Society. (2022). Embryonal Tumor of the Central Nervous System.

[2] National Cancer Institute. (2022). Embryonal Carcinoma of the Brain and Spinal Cord.

[3] Children's Oncology Group. (2022). Embryonal Tumors.

[4] American Academy of Pediatrics. (2022). Diagnosis and Treatment of Central Nervous System Embryonal Tumors in Infants and Young Children.

[5] Journal of Neuro-Oncology. (2019). Recurrence of Embryonal Carcinoma of the Brain: A Case Report.