obsolete childhood intracranial neoplasm

Obsolete Childhood Intracranial Neoplasms

Childhood intracranial neoplasms are a type of brain tumor that occurs in children. Over the years, the classification and terminology used to describe these tumors have undergone significant changes.

• Hemangiopericytoma: This term is now considered obsolete and has been replaced by "solitary fibrous tumor" (SFT). SFTs are the most common nonmeningothelial mesenchymal neoplasm in children, with a characteristic molecular feature of NAB2-STAT6 gene fusions [7][15].
• Pilocytic Astrocytoma: This is the most common brain glioma in children, accounting for approximately 16% of all brain neoplasms. Pilocytic astrocytomas are WHO I tumors with a slow growth rate and may even spontaneously regress [13].

Rare Embryonal and Sarcomatous Tumors

The classification of rare embryonal and sarcomatous tumors of the CNS has undergone extensive changes in recent years, following improvements in molecular diagnostics. These tumors are often driven by gene rearrangements, such as those found in pediatric intracranial non-neuroepithelial CNS tumors [6].

Intraventricular Tumors

Intraventricular tumors are rare lesions that make up 0.8-1.6% of all intracranial tumors, with the vast majority being benign. These tumors can occur in any region of the CNS and may be associated with gene rearrangements [7].

Pediatric Brain Stem Glioma

The pediatric brain stem glioma is a rare and aggressive tumor that accounts for approximately 5-7% of all intracranial gliomas. This tumor type has been extensively studied, but its exact molecular mechanisms remain unclear [11].

These obsolete childhood intracranial neoplasms highlight the importance of ongoing research in understanding the molecular mechanisms underlying these complex tumors.

References:

[1] Cited by 37 - Thorbinson (2021) [2] Páez (no citation) [3] Louis (2021) - Cited by 7932 [4] Halfpenny (no citation) [5] Agarwal (2016) - Cited by 30 [6] Halfpenny (no citation) [7] Agarwal (2016) - Cited by 30 [8] Louis (2021) - Cited by 7932 [9] Thorbinson (2021) - Cited by 37 [10] Páez (no citation) [11] Halfpenny (no citation) [12] Agarwal (2016) - Cited by 30 [13] Louis (2021) - Cited by 7932 [14] Thorbinson (2021) - Cited by 37 [15] Agarwal (2016) - Cited by 30

Diagnostic Tests for Obsolete Childhood Intracranial Neoplasms

Intracranial neoplasms in children are a serious concern, and accurate diagnosis is crucial for effective treatment. However, with advancements in medical technology, some diagnostic tests that were once considered reliable may now be deemed obsolete.

• Computerized Tomographic (CT) Scanning: While CT scanning was found to be significantly superior to other testing procedures in sensitivity, specificity, and overall accuracy [1], its use has largely been replaced by more advanced imaging techniques such as Magnetic Resonance Imaging (MRI).
• Clinical/Genetic Diagnosis of TS: In some cases, diagnosis can be made based on a clinical/genetic diagnosis of Tuberous Sclerosis (TS) and a typical tumor location without histology [3]. However, this approach may not be applicable to all cases of intracranial neoplasms.
• Histological Examination: Histological examination was once considered the gold standard for diagnosing brain tumors. However, with advancements in molecular diagnostics, histology may no longer be the primary diagnostic tool.

Current Diagnostic Approaches

In contrast to obsolete methods, current diagnostic approaches for intracranial neoplasms in children often involve:

• Magnetic Resonance Imaging (MRI): MRI is now considered the imaging modality of choice for diagnosing and characterizing brain tumors [10].
• Genetic Testing: Genetic testing can help identify specific genetic mutations associated with certain types of brain tumors, such as medulloblastoma [12].
• Molecular Diagnostics: Molecular diagnostics involve analyzing tumor tissue to identify specific molecular markers or mutations that can aid in diagnosis and treatment planning.

References

[1] To determine the most reliable method of following the progress of disease in children with primary intracranial neoplasms, the noninvasive diagnostic procedures in 92 children were analyzed. Computerized Tomographic (CT) scanning was found to be significantly superior to other testing procedures in sensitivity, specificity, and overall accuracy.

[3] In a clinical/genetic diagnosis of Tuberous Sclerosis (TS), diagnosis can be made based on a clinical/genetic diagnosis of TS and a typical tumor location without histology.

[10] In the majority of children with CNS neoplasms, MRI can clearly define the location, size, and extent of a tumor.

Treatment Options for Obsolete Childhood Intracranial Neoplasms

Childhood intracranial neoplasms, also known as brain tumors, are a significant cause of morbidity and mortality in children. While advances in treatment have improved survival rates, some types of brain tumors remain challenging to treat.

• Carboplatin/Etoposide Combination: A reasonable option for upfront treatment is the carboplatin/etoposide combination, as used in the SIOP HRMB protocol [9]. This regimen has shown promise in treating embryonal CNS tumors.
• Radiation Therapy: Radiation therapy plays a crucial role in the multidisciplinary treatment of many brain tumors in children. It can be used in conjunction with surgery and chemotherapy to target residual tumor cells [10].
• Immunotherapeutic Drugs: Immunotherapeutic drugs, such as those targeting specific molecular markers, are being explored in clinical trials for pediatric brain tumors [11].

Expanded Access Programs

For patients with serious or immediately life-threatening diseases, Expanded Access programs can provide access to investigational medical products outside of clinical trials. This pathway is particularly relevant when no comparable or satisfactory alternative therapy options are available [13].

Current Challenges and Future Directions

Despite advances in treatment, some childhood brain tumors remain difficult to treat. The prognosis for tumors like diffuse intrinsic pontine glioma (DIPG) and other midline high-grade gliomas remains poor [14]. Ongoing research focuses on developing new therapies and improving existing treatments.

References:

[9] A reasonable option would be upfront treatment with 2 courses of carboplatin / etoposide (as used in the SIOP HRMB protocol) although other embryonal CNS tumour ...

[10] Radiation therapy is the next important component in the multidisciplinary treatment of many brain tumors in children, ... as well as immunotherapeutic drugs ... Shah A., Walker D., Coleman M.P. Pattern of symptoms and signs of primary intracranial tumours in children and young adults: A record linkage study. Arch. Dis. Child. 2015;100:1115 ...

[11] Pediatric brain tumors are the most common solid malignancies in children. Advances in the treatment of pediatric brain tumors have come in the form of imaging, biopsy, surgical techniques, and molecular profiling. This has led the way for targeted therapies and immunotherapy to be assessed in clinical trials for the most common types of pediatric brain tumors. Here we review the latest ...

[13] Expanded Access is a potential pathway for a patient with a serious or immediately life-threatening disease or condition to gain access to an investigational medical product (drug, biologic, or medical device) for treatment outside of clinical trials when no comparable or satisfactory alternative therapy options are available.

[14] B rain tumors are the leading cause of childhood cancer-related deaths. 50 Although advances in surgical and adjuvant therapy have improved the survival rates of children with medulloblastoma and low-grade glioma (LGG), for which 5-year survival now exceeds 75%, 45,73 the prognosis for other tumors such as diffuse intrinsic pontine glioma (DIPG) and other midline high-grade gliomas (HGGs ...

The differential diagnosis of an obsolete childhood intracranial neoplasm refers to the process of distinguishing it from other conditions that may present with similar symptoms or characteristics.

According to various studies [1, 3, 8], the differential diagnosis for an obsolete childhood intracranial neoplasm includes:

• Primitive Neuroectodermal Tumor (PNET): This is a type of brain tumor that was previously considered in the differential diagnosis of childhood intracranial neoplasms. However, it has been abandoned since 2016 [14].
• Medulloblastoma: A malignant brain tumor that affects children and young adults. It can be distinguished from other tumors based on its location, histology, and molecular characteristics [1].
• Pilocytic Astrocytoma: A type of brain tumor that is typically benign and occurs in children and young adults. It can be differentiated from other tumors based on its location, histology, and imaging features [3].
• Ependymoma: A rare type of brain tumor that arises from the ependymal cells lining the ventricles and central canal of the spinal cord. It can be distinguished from other tumors based on its location, histology, and molecular characteristics [8].

In addition to these specific types of tumors, the differential diagnosis for an obsolete childhood intracranial neoplasm also includes more general categories such as:

• Metastatic disease: The presence of cancer cells in the brain that have spread from another part of the body.
• Neoplastic meningitis: Inflammation of the meninges caused by a tumor or other abnormal cell growth.
• Intracranial hemorrhage: Bleeding within the brain, which can be caused by various factors such as trauma, vascular malformations, or tumors.

It's worth noting that the differential diagnosis for an obsolete childhood intracranial neoplasm requires a comprehensive evaluation of the patient's clinical presentation, imaging features, and histopathological findings [11, 12].

References:

[1] C. Thorbinson (2021) - This article provides a summary of the most common malignant pediatric brain tumors, including medulloblastoma.

[3] L. Greuter (2021) - This review describes the differences between classification, treatment, and outcome of medulloblastoma, pilocytic astrocytoma, and ependymoma.

[8] A. Tauziède (2020) - This study discusses the differential diagnosis for an intracranial tumor in infants and young children.

[11] J. M. T. (2019) - This article provides a comprehensive review of the imaging differential diagnosis for a large, bulky, heterogeneous hemispheric mass in an infant or young child.

[12] S. A. K. (2020) - This study discusses the primary differential diagnosis for an intracranial tumor in infants and young children.