intracranial primitive neuroectodermal tumor

Intracranial Primitive Neuroectodermal Tumor (PNET): A Rare and Aggressive Brain Cancer

Primitive neuroectodermal tumors (PNETs) are a group of highly malignant brain tumors that originate from primitive nerve cells. Intracranial PNETs, in particular, are rare and aggressive forms of this cancer that occur within the brain.

Characteristics:

• Rare occurrence: Intracranial PNETs account for only a small percentage of all brain tumors.
• Highly malignant: These tumors are known to be highly aggressive and have a poor prognosis.
• Affects children and young adults: Most cases of intracranial PNETs occur in children and young adults, although they can also affect older individuals.
• Can be misdiagnosed: Intracranial PNETs can be easily misdiagnosed as other types of brain tumors, such as meningiomas or central nervous system primitive neuroectodermal tumors (CNS-PNET).

Symptoms:

• Variable symptoms: The symptoms of intracranial PNETs can vary depending on the location and size of the tumor.
• Common symptoms: Common symptoms include headaches, seizures, weakness or numbness in the face or limbs, and changes in mental status.

Treatment:

• Surgery: Surgery is often the primary treatment for intracranial PNETs, although it may not be possible to completely remove the tumor.
• Chemotherapy and radiation therapy: Chemotherapy and radiation therapy are also used to treat intracranial PNETs, either alone or in combination with surgery.

Prognosis:

• Poor prognosis: Unfortunately, the prognosis for patients with intracranial PNETs is generally poor, although it can vary depending on the individual case.

References:

[1] (12) Abstract. Background: Primary intracranial Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare malignancies, which arise in children and adolescents, with only 9 cases reported in patients over 30 years of age.

[2] (13) Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) is a malignant small, round cell tumor arising from bone and soft tissue in children and young adults.

[3] (14) INTRODUCTION. Ewing’s sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) is a type of malignant small and blue round cell tumors. It usually occurs in children and young adults with 3/4 th of the cases arising from bone and rest 1/4 th from soft tissue.

Note: The above information is based on the search results provided, which are relevant to intracranial primitive neuroectodermal tumor (PNET).

Common Signs and Symptoms

Intracranial primitive neuroectodermal tumors (PNETs) can exhibit a range of symptoms, primarily due to increased pressure in the brain. Some common signs and symptoms include:

• Headaches: Morning headaches or headaches that go away after vomiting are often reported [3][6].
• Vomiting: Nausea and vomiting are frequent complaints, especially in the morning [7][8].
• Weakness or change in sensation: Weakness or a change in sensation on one side of the body can occur due to the tumor's location [5][8].
• Blurry vision: Blurry vision is another symptom that may be experienced by individuals with intracranial PNETs [3].

Additional Symptoms

Symptoms can vary depending on the location of the tumor. Some additional symptoms include:

• Irritability: Irritability or restlessness can be a sign of an intracranial PNET.
• Dizziness: Dizziness and other symptoms of intracranial hypertension are common in individuals with these tumors [6].
• Site-specific symptoms: Symptoms such as exophthalmos, epistaxis, nasal obstruction, and individual cranial neuropathies can occur depending on the tumor's location [4].

Aggressive Nature

It is essential to note that PNETs and pineoblastomas are aggressive tumors that tend to attach to parts of the brain controlling movement, thought, and sensation. Early detection and treatment are crucial for improving outcomes.

References:

[1] Context result 3 [2] Context result 6 [3] Context result 7 [4] Context result 8

Diagnostic Tests for Intracranial Primitive Neuroectodermal Tumor (PNET)

Intracranial PNETs are aggressive tumors that require prompt and accurate diagnosis to determine the best course of treatment. The following diagnostic tests can help identify this type of tumor:

• Imaging Studies: Magnetic Resonance Imaging (MRI) scans are primarily used to show the size and characteristics of the tumor, as well as the presence of any metastasis [6]. Computed Tomography (CT) scans may also be used to evaluate the extent of the disease.
• Tissue Biopsy: A tissue biopsy with cytogenetic and immunohistochemical studies is crucial in diagnosing peripheral primitive neuroectodermal tumors (pPNETs) [4].
• Neurological Exam: A thorough neurological exam, including tests of reflexes, muscle strength, eye and mouth movement, coordination, and other functions, can help assess the tumor's impact on brain function.
• Other Diagnostic Procedures: Additional procedures such as a CT scan, MRI, x-ray, bone scan, arteriogram, myelogram, spinal tap, PET, MRS, and others may be used to rule out other conditions or evaluate the extent of the disease [9].

Key Points

• Imaging studies, particularly MRI scans, are essential in evaluating the tumor's size and characteristics.
• A tissue biopsy with cytogenetic and immunohistochemical studies is crucial for accurate diagnosis.
• A thorough neurological exam can help assess the tumor's impact on brain function.

References

[4] Jan 3, 2024 — Tissue biopsy with cytogenetic and immunohistochemical studies is paramount in diagnosing peripheral primitive neuroectodermal tumors (pPNETs). [6] Magnetic resonance imaging (MRI) scans primarily are used; this imaging test can show the size and characteristics of the tumor, as well as the presence of any metastasis. [9] by A Behdad · 2010 · Cited by 84 — In summary, the current study supports the following conclusions regarding CNS PNET: (i) routine application of INI1 immunohistochemistry helps rule out AT/RT, ...

Chemotherapy plays a crucial role in treating intracranial primitive neuroectodermal tumors (PNETs).

• Chemotherapy regimens have significantly improved outcomes in patients with peripheral primitive neuroectodermal tumors (pPNETs) [3].
• Short intensive induction chemotherapy followed by tandem-HDCT in young children with CNS-PNET/pineoblastomas seems to be superior to the conventional treatment approach [5].
• Clinical trials testing new chemotherapy, targeted therapy, or immunotherapy drugs may also be available for treating PNETs [4].

Combination of therapies is often used

• Treatment typically includes a combination of surgery, radiation therapy, chemotherapy, and possibly shunt placement [1].
• These treatments include neurosurgery, radiation therapy, or chemotherapy. If your child has hydrocephalus another procedure, called ETV (endoscopic third ventriculostomy), may be performed [7].

New treatment options are being explored

• UCSF is testing a treatment that combines iobenguane and vorinostat for high-risk neuroblastoma, which may also be applicable to PNETs [6].
• Ensartinib, a tyrosine kinase inhibitor, is being studied as a potential treatment option for tumors like PNETs [6].

References:

[1] Several treatment options are available; treatment typically includes a combination of surgery, radiation therapy, chemotherapy, and possibly shunt placement. [3] Jan 3, 2024 — Chemotherapy regimens have significantly improved outcomes in patients with peripheral primitive neuroectodermal tumors (pPNETs). [4] Aug 20, 2024 — Clinical trials testing new chemotherapy, targeted therapy, or immunotherapy drugs may also be available. Treatments are decided by the ... [5] by C Friedrich · 2013 · Cited by 96 — Short intensive induction chemotherapy followed by tandem-HDCT in young children with CNS-PNET/pineoblastomas seems to be superior to the ... [6] Nov 5, 2024 — UCSF is testing a treatment that combines iobenguane and vorinostat for high-risk neuroblastoma. They are also studying ensartinib for tumors ... [7] These treatments include neurosurgery, radiation therapy, or chemotherapy. If your child has hydrocephalus another procedure, called ETV (endoscopic third ...

The differential diagnosis for intracranial primitive neuroectodermal tumors (PNETs) includes a wide range of conditions that can mimic the appearance and behavior of PNETs.

Key Differential Diagnoses:

• Anaplastic ependymoma
• Atypical teratoid rhabdoid tumor (ATRT)
• Other forms of highly proliferative malignant neuroectodermal tumors

These differential diagnoses are important to consider when evaluating patients with suspected intracranial PNETs, as they can have similar histological and clinical features.

Other Conditions to Consider:

• Teratoma with embryonic-type neuroectodermal elements
• Metastatic small cell carcinoma
• Rhabdomyosarcoma
• Ewing's sarcoma-primitive neuroectodermal tumor (ES-PNET)
• Medulloblastoma
• Small cell tumors of the central nervous system

These conditions can be challenging to distinguish from PNETs, and a thorough evaluation by a multidisciplinary team is often necessary to arrive at an accurate diagnosis.

Revised Classification:

It's worth noting that the World Health Organization (WHO) has revised its classification of central nervous system (CNS) tumors using molecular parameters. As a result, some tumors previously recognized as PNETs are now classified differently. Specifically, CNS embryonal tumors are now used to describe certain types of brain tumors.

References:

• [1] WHO Classification of Tumors of the Central Nervous System (2016)
• [2-4] Various case reports and studies on intracranial PNETs
• [5] Review article on differential diagnosis of small round cell tumors in the CNS

Note: The numbers in square brackets refer to the corresponding search results provided in the context.