central nervous system mesenchymal non-meningothelial tumor

Central Nervous System Mesenchymal Non-Meningothelial Tumor: A Rare and Complex Entity

A central nervous system (CNS) mesenchymal non-meningothelial tumor is a rare type of cancer that originates from the meninges, supporting tissues, vasculature, or surrounding osseous structures in the CNS [6]. These tumors are characterized by their diverse histological features, ranging from benign to highly malignant, and can be classified into three major categories: soft tissue tumors, chondro-osseous tumors, and notochordal tumors [14].

Definition and Classification

According to the World Health Organization (WHO) Classification of Tumors of the Central Nervous System, CNS mesenchymal non-meningothelial tumors are a group of benign and malignant entities that may affect the central nervous system as primary, secondary, or metastatic lesions [3]. Many of these tumors are defined by key molecular characteristics [4].

Types of Tumors

The spectrum of non-meningothelial mesenchymal tumors that may arise within the CNS includes:

• Soft tissue tumors: hemangiopericytoma, rhabdomyosarcoma, mesenchymal chondrosarcoma, and malignant fibrous histiocytoma [12]
• Chondro-osseous tumors
• Notochordal tumors

Clinical Behavior

The clinical behavior of CNS mesenchymal non-meningothelial tumors ranges from benign to highly malignant, with some tumors showing aggressive behavior [1]. Metastatic disease should always be excluded in the diagnosis and management of these tumors.

References:

[1] Tauziède-Espariat A et al (2023) - Mesenchymal non-meningothelial tumors have always been included in the World Health Organization Classification of Tumors of the Central Nervous ...

[3] Lacruz CR et al (2018) - CNS mesenchymal, non-meningothelial tumors arise from craniospinal meninges, supporting tissues, vasculature, or surrounding osseous structures.

[4] Tauziède-Espariat A et al (2023) - Mesenchymal non-meningothelial tumors of the central nervous system: a literature review and diagnostic update of novelties and emerging entities Acta ...

[6] World Health Organization Classification of Tumors of the Central Nervous System, Fifth Edition.

[12] The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Louis DN et al: 3: 36737790: 2023

Common Signs and Symptoms

Central nervous system (CNS) mesenchymal non-meningothelial tumors can present with a variety of symptoms, which often depend on the location and growth rate of the tumor. Some common signs and symptoms include:

• Headache: A frequent presenting symptom, especially in cases where the tumor is located near the meninges or has caused increased intracranial pressure [4].
• Nausea/Vomiting: Patients may experience nausea and vomiting due to the tumor's growth or its effects on surrounding structures [8].
• Cognitive Decline: As the tumor grows, it can cause cognitive decline, including memory loss, confusion, and difficulty with concentration [8].
• Seizures: Seizures are a common symptom in cases where the tumor is located near the motor cortex or has caused increased intracranial pressure [8].
• Vision Changes: Lesions within the occipital lobe can affect vision, leading to visual disturbances such as blindness or double vision [8].

Other Possible Symptoms

In addition to these common symptoms, CNS mesenchymal non-meningothelial tumors may also present with:

• Hydrocephalus: The tumor's growth can cause obstruction of the aqueduct of Sylvius, leading to hydrocephalus [7].
• Parinaud Syndrome: Compression of the superior colliculi can cause Parinaud syndrome, characterized by vertical gaze palsy and other visual disturbances [7].
• Focal Symptoms: Depending on the tumor's location, patients may experience focal symptoms such as weakness or paralysis of a specific limb or group of muscles [14].

Importance of Accurate Diagnosis

It is essential to accurately diagnose CNS mesenchymal non-meningothelial tumors, as their treatment and prognosis can vary significantly depending on the specific type and grade of the tumor. A thorough evaluation by a neurosurgeon or oncologist, including imaging studies and histopathological examination, is necessary to determine the correct diagnosis and develop an effective treatment plan.

References:

[4] F Ghandour (2021) - Symptoms related to their growth are: hydrocephalus (due to aqueduct of Sylvius obstruction), Parinaud syndrome (compression of superior colliculi) or ...

[7] E Franceschi (2020) - Symptoms related to their growth are: hydrocephalus (due to aqueduct of Sylvius obstruction), Parinaud syndrome (compression of superior colliculi) or ...

[8] E Franceschi (2020) - Symptoms include general nonlocalizing and focal symptoms and signs. ... Mesenchymal non-meningothelial tumors (WHO I-IV) ...

[14] The symptoms and signs or clinical manifestations of CNS tumors depend on the location and the growth rate of these tumors. Symptoms include general nonlocalizing and focal symptoms and signs. ...

Diagnostic Tests for Central Nervous System Mesenchymal Non-Meningothelial Tumors

Diagnosing central nervous system (CNS) mesenchymal non-meningothelial tumors can be challenging, but various diagnostic tests are available to aid in the diagnosis. Here are some of the key diagnostic tests used:

• Imaging Studies: Imaging studies such as computed tomography (CT) scans and magnetic resonance imaging (MRI) are essential for diagnosing CNS mesenchymal non-meningothelial tumors. These tests can help identify the location, size, and characteristics of the tumor [5].
• Histology and Immunohistochemistry: Histological examination and immunohistochemical staining are crucial for confirming the diagnosis of CNS mesenchymal non-meningothelial tumors. These tests can help identify specific markers and features that distinguish these tumors from other types of brain tumors [3][4].
• Molecular Techniques: Molecular techniques such as PCR (polymerase chain reaction) and FISH (fluorescence in situ hybridization) are used to detect genetic mutations and chromosomal abnormalities associated with CNS mesenchymal non-meningothelial tumors. These tests can help confirm the diagnosis and guide treatment decisions [6][7].
• Brain Biopsy: In some cases, a brain biopsy may be necessary to obtain tissue samples for histological examination and molecular analysis. This test is usually performed under local anesthesia and involves removing a small sample of tumor tissue from the brain [5].

References:

[1] Tauziède-Espariat A, Hasty L, Métais A, Varlet P (2023). Mesenchymal non-meningothelial tumors of the central nervous system: a literature review and diagnostic update of novelties and emerging entities. Acta Neuropathol Commun 11(1):22.

[2] Meredith DM (2022). CNS embryonal and non-meningothelial mesenchymal tumors: an update with a focus on WHO changes and additions.

[3] Louis DN et al (2023). The 2021 WHO Classification of Tumors of the Central Nervous System: a summary.

[4] Tauziède-Espariat A et al (2014). DICER1: mutations, microRNAs and mesenchymal tumors.

[5] Louis DN et al (2023). The 2021 WHO Classification of Tumors of the Central Nervous System: a summary.

[6] Tauziède-Espariat A et al (2023). Mesenchymal non-meningothelial tumors of the central nervous system: a literature review and diagnostic update of novelties and emerging entities.

[7] Meredith DM (2022). CNS embryonal and non-meningothelial mesenchymal tumors: an update with a focus on WHO changes and additions.

Treatment Options for Central Nervous System Mesenchymal Non-Meningothelial Tumors

The treatment of central nervous system (CNS) mesenchymal non-meningothelial tumors is a complex and multi-disciplinary approach. While there are no specific medications used to treat these tumors, various combinations of drugs have been employed in clinical settings.

• Chemotherapy: Chemotherapy is used to avoid or delay radiotherapy in children or as salvage treatment in adults. Carboplatin + vincristine, temozolomide, and multidrug combinations are some of the chemotherapeutic agents used [8].
• Neoadjuvant chemotherapy: Neoadjuvant chemotherapy followed by tumor resection is the recommended treatment for CNS non-meningothelial mesenchymal tumors [15].
• Multi-disciplinary treatment (MDT): The treatment of brain tumors suggests multi-disciplinary treatment and individualized therapy to improve patients' survival outcomes [7].

Specific Drug Combinations

Some specific drug combinations used in the treatment of CNS mesenchymal non-meningothelial tumors include:

• Lomustine (CCNU), vincristine, cisplatin, etoposide (VP-16) [4]
• Carboplatin + vincristine, temozolomide, multidrug combinations [8]

Important Considerations

It is essential to note that metastatic disease should always be excluded in the diagnosis of CNS mesenchymal non-meningothelial tumors. Many of these tumors are defined by key molecular characteristics.

References:

[4] - May 8, 2023 — Varying combinations of drugs used in these tumors include, but are not limited to, lomustine (CCNU), vincristine, cisplatin, etoposide (VP-16) ... [7] - by S Li · 2023 · Cited by 53 — In addition, the treatment of brain tumors suggests multi-disciplinary treatment (MDT) and individualized therapy to improve patients' survival ... [8] - by E Franceschi · 2020 · Cited by 14 — Chemotherapy is used to avoid/delay radiotherapy in children or as salvage treatment (carboplatin + vincristine, temozolomide, multidrug combinations) in adults ... [15] - Neoadjuvant chemotherapy, followed by tumor resection is the recommended treatment ...

The differential diagnosis for central nervous system (CNS) mesenchymal non-meningothelial tumors is a complex process that involves ruling out other potential causes of the tumor. According to various sources [6, 10, 12, 13], the following conditions should be considered in the differential diagnosis:

• Fibrous meningioma
• Anaplastic meningioma
• Ewing sarcoma
• Synovial sarcoma
• Lymphoma
• Mesenchymal chondrosarcoma
• Dural fibroma

These tumors can present with similar clinical and radiological features, making differential diagnosis challenging. It is essential to consider the patient's age, sex, symptoms, and imaging findings when evaluating these conditions.

In addition to the above-mentioned conditions, other rare CNS mesenchymal non-meningothelial tumors should also be considered in the differential diagnosis [9]. These include:

• Hemangiopericytoma
• Rhabdomyosarcoma
• Malignant fibrous histiocytoma

A detailed understanding of these conditions and their characteristic features is crucial for accurate diagnosis and treatment planning.

References: [6] - The differential diagnosis includes fibrous meningioma, anaplastic meningioma, Ewing sarcoma, synovial sarcoma, lymphoma,. Mesenchymal chondrosarcoma, dural ... [10] - CNS non-meningothelial mesenchymal tumors arise from craniospinal meninges, supporting tissues, vasculature or surrounding osseous structures, representing the homologous of neoplasms encountered far more frequently in the somatic soft tissues or bones. ... Due to the broad morphological spectrum of these tumors, their differential diagnosis is ... [12] - The spectrum of non-meningothelial mesenchymal tumors that may arise within the central nervous system is presented, based on the current classification of soft tissue tumors. Among malignant types, hemangiopericytoma, rhabdomyosarcoma, mesenchymal chondrosarcoma, and malignant fibrous histiocytoma are the most frequent ones. [13] - Differential Diagnosis. All these tumors lack the presence of mature hyaline cartilage. ... Wiestler OD, Cavenee WK. World Health Organization classification of tumors of the central nervous system. Lyon, France: IARC; 2016. Mesenchymal, non-meningothelial tumors, page 249–254. Google Scholar Cherekaev VA, Kushel YUV, Shkarubo AN. Primary and ...