papillary glioneuronal tumor

Papillary Glioneuronal Tumor (PGNT) Description

A Papillary Glioneuronal Tumor (PGNT) is a rare, low-grade brain tumor characterized by its biphasic pattern. This means that it has two distinct components: pseudopapillary glial structures and neuronal elements.

According to medical literature [1], PGNTs are typically well-demarcated masses with a cystic component, which can show contrast enhancement on MRI scans [4]. They are often found in the cerebral hemispheres, predominantly in young adults [5].

Histologically, PGNTs exhibit a biphasic pattern of glial and neuronal components, which can be further demonstrated using immunohistochemistry [6]. This unique histological appearance is a key feature that distinguishes PGNT from other types of brain tumors.

In terms of clinical behavior, PGNTs are generally considered to be benign (WHO grade I) and relatively circumscribed [9]. However, they can cause seizures and other neurological symptoms due to their location in the brain.

Overall, the description of a Papillary Glioneuronal Tumor is that of a rare, low-grade brain tumor with a distinctive biphasic histological pattern and benign clinical behavior.

References:

[1] BD Radotra (2007) - Papillary glioneuronal tumor (PGNT) is a recently described lesion of the brain...

[4] L Lacroix (2015) - On MRI, PGNT is frequently described as a well-demarcated mass with a cystic component...

[5] K Shinno (2021) - PGNT is a benign brain tumor with glial and neuronal components and occurs in the cerebral hemispheres predominantly in young adults.

[6] N Mittal (2017) - The tumour has a typical biphasic histological pattern of glial and neuronal which can be further demonstrated on immunohistochemistry.

[9] M Rivera-Zengotita (2016) - Papillary glioneuronal tumor (PGNT) is a benign (WHO grade I), relatively circumscribed, biphasic cerebral neoplasm...

Common Signs and Symptoms

Papillary glioneuronal tumors are typically characterized by a range of symptoms, which can vary in severity and frequency among affected individuals.

• Headaches: A frequent symptom associated with these tumors, often caused by increased intracranial pressure or mass effects [5].
• Seizures: Most common and typical manifestation of temporal lobe tumors, whereas lesions at other sites usually feature headache and nonspecific signs and symptoms [2][3].
• Nausea and Vomiting: Commonly reported symptoms, often accompanied by headaches and fever [4][5].
• Dizziness and Limb Weakness: Some patients may experience dizziness and limb weakness as part of their initial symptoms [1][6].

Other Possible Symptoms

In addition to the above-mentioned symptoms, some individuals with papillary glioneuronal tumors may also experience:

• Hearing Symptoms: Blurred hearing or other auditory disturbances have been reported in some cases [1].
• Mass Effects: As the tumor grows, it can exert pressure on surrounding brain structures, leading to additional symptoms such as vision changes or cognitive impairment.

References

[1] X Du (2024) - Initial symptoms of patients with papillary glioneuronal tumors included epilepsy, headache, vomiting, dizziness, limb weakness, hearing symptoms, and blurred vision. [2] BD Radotra (2007) - Seizures are most common and typify temporal lobe tumors, whereas lesions at other sites usually feature headache and nonspecific signs and symptoms. [3] BD Radotra (2007) - Seizures are most common and typify temporal lobe tumors, whereas lesions at other sites usually feature headache and nonspecific signs and symptoms. [4] T Stosic-Opincal (2005) - A 16-year-old girl with papillary glioneuronal tumor presented with nausea, vomiting, fever, and headaches. [5] Symptoms related to blockage of cerebrospinal fluid are also common, such as headaches, nausea, and vomiting. [6] X Du (2024) - The initial symptoms of the patients in this group included epilepsy, headache, vomiting, dizziness, limb weakness, hearing symptoms, blurred vision.

Diagnostic Tests for Papillary Glioneuronal Tumor

Papillary glioneuronal tumors (PGNTs) are rare mixed neuronal-glial tumors that can be challenging to diagnose. While there is no single definitive test, various diagnostic methods can help establish a diagnosis.

• Imaging Studies: MRI and CT scans may show a well-demarcated mass with cystic changes, but these findings are not specific to PGNTs [3][4].
• Histology: A biopsy or surgical specimen examination is essential for a definitive diagnosis. Histological features include a biphasic pattern of glial and neuronal cells, which can be further demonstrated on immunohistochemistry [8][9].
• Cytology: Cytologic features of PGNTs correlate well with the histologic diagnosis, making cytology a useful diagnostic tool [5].
• Molecular Testing: The detection of BRAF rearrangements can help distinguish PGNTs from other gliomas, such as diffuse astrocytomas and oligodendrogliomas [10].

It's essential to note that diagnosing PGNTs with diagnostic imaging studies is not easy, and the long-term prognosis remains unclear [13]. A multidisciplinary approach involving neurosurgeons, neuropathologists, and radiologists may be necessary for an accurate diagnosis and treatment plan.

References:

[3] The diagnosis of PGNT is often difficult to establish both on MRI and histology. On MRI, PGNT is frequently described as a well-demarcated mass [3].

[4] Dec 15, 2015 — The diagnosis of PGNT is often difficult to establish both on MRI and histology. On MRI, PGNT is frequently described as a well-demarcated mass [4].

[5] by A Puzyrenko · 2021 · Cited by 6 — Papillary glioneuronal tumor can be confirmed by using cytology. •. Cytologic features of this tumor correlate well with the histologic diagnosis on the [5].

[8] by N Mittal · 2017 · Cited by 3 — The tumour has a typical biphasic histological pattern of glial and neuronal which can be further demonstrated on immunohistochemistry. [8]

[9] by YW Park · 2023 · Cited by 9 — Tumor diagnosis in glioneuronal and neuronal tumors may still be primarily based on H&E stained sections, and the molecular workup is [9].

[10] The detection of a BRAF rearrangement can help distinguish cancers with favorable prognosis such as glioneuronal tumors from those with poorer prognosis, such as diffuse gliomas. [10]

[13] Papillary glioneuronal tumors (PGNTs) are clinically benign and rarely recur after gross total resection. However, diagnosing PGNTs with diagnostic imaging studies is not easy, and the long-term prognosis remains unclear. [13]

Treatment Options for Papillary Glioneuronal Tumor (PGNT)

Papillary glioneuronal tumors (PGNTs) are typically benign, but in rare cases, they can recur after surgery. The most common treatments for PGNT include:

• Surgery: Total tumor excision is considered the first-choice treatment for PGNT, although relapse sometimes happens after resection [8].
• Targeted Therapy: Chemotherapy regimens such as carboplatin and vincristine, temozolomide, etoposide, and bevacizumab are used in some cases [2].
• Radiation Therapy: Radiation is offered if the tumor is not totally resected or if it displays high-grade aggressive behavioral features or recurrence [5].

Chemotherapy Regimens

Some chemotherapy regimens that have been used to treat PGNT include:

• Carboplatin and vincristine
• Temozolomide
• Etoposide
• Bevacizumab

These treatments are often used in combination with surgery and radiation therapy.

References:

[1] BD Radotra (2007) - Papillary glioneuronal tumor (PGNT) is a recently described lesion of the brain, which is still not included as a separate entity in WHO classification. [2] L Gatto (2020) - Chemotherapy regimens include carboplatin and vincristine, temozolomide, etoposide and bevacizumab [Citation114]. [3] EA Goethe (2019) - PGNTs, while typically benign, can rarely recur after surgery. Molecular testing should be performed on all PGNTs to help possibly identify more aggressive features. [4] VD Nunno (2024) - Surgery is considered the cornerstone of treatment while radiotherapy is often deserved for inoperable tumors or after incomplete resection. [5] Feb 1, 2018 - Radiation is offered if the tumor is not totally resected or if it displays high-grade aggressive behavioral features or recurrence. [6] by K Shinno (2021) - The first-choice treatment for PGNT is total tumor excision, although relapse sometimes happens after resection. In most cases, the patient recovers fully. [7] Jun 17, 2024 - Treatment options for children with astrocytomas, other gliomas, and glioneuronal/neuronal tumors include observation, surgery, chemotherapy, and radiation therapy.

Differential Diagnoses for Papillary Glioneuronal Tumor (PGNT)

Papillary glioneuronal tumors (PGNTs) are rare mixed neuronal-glial tumors that can be challenging to diagnose. The differential diagnoses for PGNT include:

• Ganglioglioma: A cystic mass with a mural nodule, calcification, and temporal lobe predilection, which is another type of low-grade glioma [3][5].
• Supratentorial extraventricular ependymomas: Another important differential diagnosis for PGNT, as both tumors can present with similar imaging features [14].
• Polymorphous low-grade neuroepithelial tumor (PLNT) of the young: A rare, low-grade glioma that can mimic the appearance of PGNT on imaging studies [13].
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