polymorphous low grade neuroepithelial tumour of the young

Polymorphous Low Grade Neuroepithelial Tumor of the Young (PLNTY): A Rare Epileptogenic Tumor

PLNTY is a rare and recently described type of brain tumor that primarily affects children and young adults. It is characterized by its heterogeneous histological appearance, with both diffuse and compact growth patterns [2]. The tumor exhibits oligodendroglioma-like cellular components and is often associated with cortical dysplasia [12].

Molecular analysis has identified frequent genetic abnormalities involving the BRAF proto-oncogene or fibroblast growth factor receptor 2 (FGFR2) and FGFR3 genes in PLNTY cases [12, 13]. These genetic alterations are thought to contribute to the tumor's development and progression.

PLNTY is often diagnosed in individuals with a history of drug-resistant epilepsy, and it is considered one of the low-grade neuroepithelial tumors according to the World Health Organization 2021 classification of brain tumors [10].

Key Features:

• Heterogeneous histological appearance
• Oligodendroglioma-like cellular components
• Association with cortical dysplasia
• Frequent genetic abnormalities involving BRAF, FGFR2, and FGFR3 genes
• Often diagnosed in individuals with drug-resistant epilepsy

References:

[1] Acta Neuropathol 2017;133:417 [2] by G Broggi · 2021 · Cited by 20 [10] by VR Gupta · 2019 · Cited by 38 [12] by Y Chen · 2020 · Cited by 54 [13] by AH Palejwala · 2022 · Cited by 14

Based on the search results, here are the signs and symptoms of Polymorphous Low Grade Neuroepithelial Tumor of the Young (PLNTY):

• Mental confusion: PLNTY can cause mental confusion, which is a decrease in cognitive function, making it difficult to think clearly or make decisions [1].
• Reduced consciousness: Patients with PLNTY may experience reduced consciousness, which can range from mild to severe and can affect their ability to respond to stimuli [1].
• Dyskinesia: Dyskinesia, a movement disorder characterized by involuntary movements, can occur in the upper right limb of patients with PLNTY [1].
• Gaze deviation: Patients may experience gaze deviation towards one side, which can be a sign of neurological involvement [3].
• Seizures: Seizures have been reported as a symptom of PLNTY, particularly in children and adolescents [4].
• Confusion, twitching limbs, deviated mouth: A 14-year-old girl with PLNTY was referred to the hospital with symptoms of confusion, twitching limbs, and deviated mouth [4].
• Calcification: PLNTY tumors are often calcified, which can be observed on imaging studies [2].

It's worth noting that these symptoms can vary in severity and may not be present in all cases. A definitive diagnosis of PLNTY requires a combination of clinical evaluation, imaging studies, and histopathological examination.

References:

[1] by G Broggi · 2021 · Cited by 20 [2] Dec 3, 2021 [3] by C Cerron-Vela · 2024 [4] by Y Chen · 2020 · Cited by 54

Diagnostic Tests for Polymorphous Low Grade Neuroepithelial Tumor of the Young (PLNTY)

Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a rare and unique brain tumor that requires accurate diagnosis to differentiate it from other similar tumors. The following diagnostic tests are crucial in diagnosing PLNTY:

• Histopathological Examination: A detailed examination of microscopic sections of the tumor specimen is essential to confirm the presence of PLNTY [7]. Microscopic sections were studied which revealed the characteristic features of PLNTY, such as infiltrating "oligodendrocyte-like" cells [10].
• Molecular Testing: Correlating histology and results from molecular testing is critical to correctly diagnosing PLNTY, as its histologic appearance is similar to other tumors [1][3]. Molecular testing helps in identifying the genetic alterations associated with PLNTY.
• Imaging Studies: Imaging studies such as MRI and CT scans are essential in evaluating the extent of tumor spread and its location within the brain. PLNTY tumors have multiple characteristic imaging features, including well-circumscribed masses, typically in the temporal lobe [5].
• Genetic Alterations Analysis: Genetic alterations analysis is crucial in identifying the genetic mutations associated with PLNTY. This helps in confirming the diagnosis of PLNTY and differentiating it from other similar tumors.

References:

[1] by AH Palejwala · 2022 · Cited by 14 — Correlating histology and results from molecular testing is critical to correctly diagnosing PLNTY, as its histologic appearance is similar to ...

[3] by AH Palejwala · 2022 · Cited by 14 — Correlating histology and results from molecular testing is critical to correctly diagnosing PLNTY, as its histologic appearance is similar to ...

[5] by JC Benson · 2020 · Cited by 36 — Although scantly described, PLNTY tumors have multiple characteristic imaging features: well-circumscribed masses, typically in the temporal lobe...

[7] by V Joshi · 2023 · Cited by 4 — The collected tumor specimen was sent for detailed histopathological examination and molecular testing. Microscopic sections were studied which revealed the characteristic features of PLNTY...

[10] by D Armocida · 2023 · Cited by 12 — Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a low-grade tumor comprised of infiltrating “oligodendrocyte-like” cells...

Treatment Options for Polymorphous Low-Grade Neuroepithelial Tumor of the Young (PLNTY)

Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a rare and benign brain tumor that primarily affects children and young adults. While it's not typically aggressive, treatment is still necessary to prevent potential complications.

Surgical Resection

The most effective treatment for PLNTY is surgical resection, which involves removing the tumor entirely. This approach has been shown to be curative and associated with a low recurrence rate [5]. In fact, one study found that surgical resection of the tumor resulted in complete recovery and no evidence of recurrence over a 15-month period [3].

Other Treatment Options

While surgery is the primary treatment for PLNTY, other options may also be considered on a case-by-case basis. These include:

• Targeted therapy: Some studies have explored the use of targeted therapies, such as MEK inhibitors (e.g., Selumetinib), to treat PLNTY [7]. However, more research is needed to confirm their efficacy.
• Chemotherapy and radiation therapy: In some cases, chemotherapy or radiation therapy may be used in conjunction with surgery to ensure complete removal of the tumor.

Importance of Early Detection

Early detection and treatment are crucial for preventing potential complications associated with PLNTY. If you suspect that you or a loved one has been diagnosed with this condition, consult with a qualified medical professional for guidance on the best course of action.

References:

[1] AH Palejwala et al. (2022) - PLNTY is a low-grade tumor comprised of infiltrating “oligodendrocyte-like” cells with frequent calcifications. [3] D Golub et al. (2023) - Clinically, the patient has shown no evidence of recurrence despite lack of adjuvant treatment over a 15-month period. [5] Polymorphous Low-Grade Neuroepithelial Tumor of the Young (PLNTY) - Surgical resection of the tumor is curative and is associated with a low recurrence rate. [7] HB Lindsay et al. (2023) - Currently available MEK inhibitors include Selumetinib, Trametinib, Cobimetinib, and Binimetinib; all are oral medications.

The differential diagnosis for Polymorphous Low Grade Neuroepithelial Tumor of the Young (PLNTY) includes:

• Dysembryoplastic Neuroepithelial Tumor (DNET): a cortically-based, "bubbly" wedge-shaped lesion that usually shows no contrast enhancement [8].
• Adult-type Diffuse Glioma (Oligodendroglioma, IDH mutant): a type of glioma that is typically found in adults and can present with similar characteristics to PLNTY [3].

It's worth noting that the differential diagnosis for PLNTY is still evolving as more research is conducted on this rare entity. However, these two conditions are currently considered to be among the most likely alternatives.

Additionally, PLNTY has been distinguished from other low-grade neuroepithelial tumors by its unique histological features, including oligodendroglioma-like cellular components and CD34 immunopositivity [6][7].