Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models

Dorea P. Jenkins; Brittany Turner-Ivey; Jody Fromm Longo; Steven L. Carroll

doi:10.3791/65740

Identificación, diagnóstico y clasificación de tumores malignos de la vaina de los nervios perifé...

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Cancer Research

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JoVE Journal Cancer Research

Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models

Identificación, diagnóstico y clasificación de tumores malignos de la vaina de los nervios periféricos en modelos de ratón modificados genéticamente

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08:57 min

May 17, 2024

DOI: 10.3791/65740-v

Dorea P. Jenkins¹, Brittany Turner-Ivey^1,2, Jody Fromm Longo¹, Steven L. Carroll^1,2

¹Department of Pathology and Laboratory Medicine,Medical University of South Carolina, ²Hollings Cancer Center,Medical University of South Carolina

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Overview

This study focuses on neurofibromatosis type one and the associated cancers, specifically plexiform neurofibromas and malignant peripheral nerve sheath tumors. Using a genetically-engineered mouse model, P0-GGF-beta-3, the research aims to better understand the pathology of these tumors.

Key Study Components

Area of Science

Neuroscience
Oncology
Genetic Engineering

Background

Neurofibromatosis type one is associated with various tumors.
Plexiform neurofibromas and malignant peripheral nerve sheath tumors are common in affected individuals.
Access to patient-derived tumor samples is limited, complicating research.
Genetically-engineered mouse models are essential for studying disease mechanisms.

Purpose of Study

To assess the pathology of nervous system neoplasms in mouse models.
To determine if these models accurately reflect human tumor pathology.
To develop methodologies for studying these tumors.

Methods Used

Histologic techniques for tumor assessment.
Defined pathologic criteria for tumor classification.
Culture methodologies for tumor study.
Use of genetically-engineered mouse model P0-GGF-beta-3.

Main Results

The methodology allows for accurate assessment of tumor pathology.
Findings contribute to understanding the similarities between mouse models and human tumors.
Challenges in identifying peripheral nerve tumors were highlighted.
Potential for improved diagnosis and treatment strategies.

Conclusions

Genetically-engineered mouse models are valuable for studying neurofibromatosis-related tumors.
Accurate modeling can lead to better understanding and treatment of these cancers.
Continued research is essential for advancing knowledge in this area.

Frequently Asked Questions

What is neurofibromatosis type one?

Neurofibromatosis type one is a genetic disorder that causes tumors to grow on nerves.

Why are genetically-engineered mouse models important?

They allow researchers to study disease mechanisms and test potential treatments in a controlled environment.

What are plexiform neurofibromas?

Plexiform neurofibromas are a type of tumor that can occur in individuals with neurofibromatosis type one.

What challenges do researchers face in studying these tumors?

Limited access to patient-derived samples makes it difficult to study the tumors directly.

How does this study contribute to cancer research?

It provides methodologies for accurately assessing tumor pathology, which can improve diagnosis and treatment.

Hemos desarrollado una metodología para evaluar si las neoplasias del sistema nervioso en ratones modificados genéticamente recapitulan con precisión la patología de sus homólogos humanos. Aquí, aplicamos estas técnicas histológicas, criterios patológicos definidos y metodologías de cultivo a neurofibromas y tumores malignos de la vaina de los nervios periféricos que surgen en el modelo de ratón P 0-GGFβ3.

Nuestro grupo se centra en la neurofibromatosis tipo uno y en la comprensión de la patología de los cánceres asociados a la enfermedad, los neurofibromas plexiformes y los tumores malignos de la vaina de los nervios periféricos. Para ello, empleamos varias técnicas, incluido el uso de un modelo de ratón modificado genéticamente, P0GGF beta tres, Nuestro laboratorio y otros que estudian los neurofibromas plexiformes y los tumores malignos de la vaina de los nervios periféricos se enfrentan a retos similares. El acceso limitado a las muestras tumorales derivadas de los pacientes dificulta nuestro trabajo.

Como resultado, la creación y caracterización de modelos de ratón modificados genéticamente es crucial para la investigación en curso sobre los mecanismos de la enfermedad y el desarrollo potencial de tratamientos. La identificación de los tumores de los nervios periféricos puede ser complicada y, a menudo, se diagnostican erróneamente como otros tipos de cáncer. Para evitar esto, hemos desarrollado un protocolo detallado que utiliza la histología, la inmunohistoquímica y la clasificación para identificar y clasificar con precisión estos tumores.

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