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Meningioma

Updated : April 22, 2024





Background

Meningioma is the most prevalent primary tumor of the central nervous system, accounting for around 37.6% of all CNS tumors.50% of benign brain tumors are meningiomas. Development of meningioma occurs in the meningeal layers of the spinal cord or the brain. WHO has divided these tumors into 3 classes.

Most meningiomas are benign and fall under grade 1 of severity. Only 1%-3% of meningiomas covert into malignant tumors, and they have a 5-year survival rate ranging between 32%-64%.

Some factors greatly contribute to the incidence of meningioma; these include:

  • Neurofibromatosis type II or some other genetic abnormalities
  • Radiation exposure
  • Hormone therapy
  • Family history

The clinical presentation of a meningioma depends on its location and size. Consequently, some people may be asymptomatic while others may exhibit neurological abnormalities. The gold standard radiological examination for identifying meningioma is performing an MRI on the brain.

Typically, asymptomatic, slow-growing meningiomas are treated with observation and routine imaging. However, for rapidly developing tumors, large tumors, or patients with symptoms, surgery is the best treatment option.

Epidemiology

According to data from 2015, the incidence of brain tumors worldwide was approximately 10.82 per 100,00 individuals annually. This figure was lower at 8.3 per 100,000 individuals between 2010-2014, so the numbers are on the rise.

Patients have a median age of 66 years at diagnosis, and females are more than twice likely to develop it than males. Histologically verified meningiomas accounted for 37.6% of all primary tumors of the central nervous system and 50% of all benign primary tumors of the central nervous system.

It affects between 1.8 and 13.1 per 100,000 people annually. More than 170,000 persons have been diagnosed with meningioma in the United States, where the prevalence is 97,5 per 100,000.

Older individuals between the age range of 75-84 are the most likely to develop meningioma with an incidence of 37.74 per 100,000 individuals. Children between the age of 0-19 years are rarely affected. Around 1% of meningiomas are related with neurofibromatosis type 2.

According to the grading system established by the World Health Organization, the recurrence rate according to stage is as follows:

  • Grade 1: 80%-81%
  • Grade 2: 17%-18%
  • Grade 3: 1.7%

The 10-year recurrence rate of meningioma can be as high as 20%. Higher-grade meningiomas have been reported to have a high recurrence rate. In grade 3 meningioma, the recurrence rate ranges between 50 and 94%. In contrast, the recurrence rate in grades 1 and 2 respectively range between 7%-25% and 29%-52%.

Anatomy

Pathophysiology

Meningioma generally arises from meningothelial arachnoid cap cells. The majority of meningiomas are idiopathic, and benign with slow growth. Malignant meningiomas frequently demonstrate several chromosomal alterations. More genetic alterations are connected with greater tumor grade and rapid growth.

In sporadic meningiomas, chromosome 22 mutations in neurofibromatosis type 2 is one of the most common risk factors. Other documented chromosomal mutations in meningiomas include 1p, 6q, 14q, and 18q.

Etiology

The majority of meningiomas are sporadic, however some have been linked to specific disorders and risk factors.

Certain factors can significantly increase the risk of meningioma incidence. These include:

  • Radiotherapy
  • Radiation
  • Exogenous hormone exposure
  • HRT
  • Breast Cancer
  • Use of oral contraceptive

Progesterone receptors are present in as much as 72% of cancers. According to studies, their size fluctuates throughout the luteal stage of the menstrual cycle and also during pregnancy. The increased frequency among women is attributable to hormonal factors.

The chance of developing meningioma is also significantly increased if an individual has any direct relatives who suffered from certain genetic syndromes such as:

  • Li-Fraumeni syndrome
  • Cowden disease
  • Gorlin syndrome
  • von Hippel Lindau disease
  • Neurofibromatosis type II

Genetics

Prognostic Factors

The 5-year progression-free survival rate for grade 1 is 95.7%, for grade 2 it is 81.8%, and for grade 3 it is 46.7%, while the 10-year survival rate for grade 1 is 90.4.7% without any progression of disease and for grade 2 it is 69.4%.

The common predictors of progression-free survival are:

  • Degree of resection
  • A mitotic index below 5%
  • Low histological grade
  • Tumor size of 6 cm or below
  • Extent of tumor multiplicity
  • Tumor location

Recurrence is related to the histological grade; grade 1 has a 4.9 percent recurrence rate, grade 2 has an 18.4% recurrence rate, and grade 3 has a 27.3% recurrence rate.

The 10-year survival rate for malignant meningiomas has been rising as a result of new therapy options. Partial tumor excision yields a 5-year survival rate of 85% for benign meningiomas, but only 58 percent for malignant meningiomas.

Meningioma tumor grade is a major predictive factor for individuals getting radiation therapy following surgery. Grades 1 and 2 were linked to a greater 3-year overall survival rate. After radiation, the 5-year overall survival rate for grade 1 meningiomas is around 81%, compared to 53% for grades 2 and 3.

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK560538/

Meningioma

Updated : April 22, 2024




Meningioma is the most prevalent primary tumor of the central nervous system, accounting for around 37.6% of all CNS tumors.50% of benign brain tumors are meningiomas. Development of meningioma occurs in the meningeal layers of the spinal cord or the brain. WHO has divided these tumors into 3 classes.

Most meningiomas are benign and fall under grade 1 of severity. Only 1%-3% of meningiomas covert into malignant tumors, and they have a 5-year survival rate ranging between 32%-64%.

Some factors greatly contribute to the incidence of meningioma; these include:

  • Neurofibromatosis type II or some other genetic abnormalities
  • Radiation exposure
  • Hormone therapy
  • Family history

The clinical presentation of a meningioma depends on its location and size. Consequently, some people may be asymptomatic while others may exhibit neurological abnormalities. The gold standard radiological examination for identifying meningioma is performing an MRI on the brain.

Typically, asymptomatic, slow-growing meningiomas are treated with observation and routine imaging. However, for rapidly developing tumors, large tumors, or patients with symptoms, surgery is the best treatment option.

According to data from 2015, the incidence of brain tumors worldwide was approximately 10.82 per 100,00 individuals annually. This figure was lower at 8.3 per 100,000 individuals between 2010-2014, so the numbers are on the rise.

Patients have a median age of 66 years at diagnosis, and females are more than twice likely to develop it than males. Histologically verified meningiomas accounted for 37.6% of all primary tumors of the central nervous system and 50% of all benign primary tumors of the central nervous system.

It affects between 1.8 and 13.1 per 100,000 people annually. More than 170,000 persons have been diagnosed with meningioma in the United States, where the prevalence is 97,5 per 100,000.

Older individuals between the age range of 75-84 are the most likely to develop meningioma with an incidence of 37.74 per 100,000 individuals. Children between the age of 0-19 years are rarely affected. Around 1% of meningiomas are related with neurofibromatosis type 2.

According to the grading system established by the World Health Organization, the recurrence rate according to stage is as follows:

  • Grade 1: 80%-81%
  • Grade 2: 17%-18%
  • Grade 3: 1.7%

The 10-year recurrence rate of meningioma can be as high as 20%. Higher-grade meningiomas have been reported to have a high recurrence rate. In grade 3 meningioma, the recurrence rate ranges between 50 and 94%. In contrast, the recurrence rate in grades 1 and 2 respectively range between 7%-25% and 29%-52%.

Meningioma generally arises from meningothelial arachnoid cap cells. The majority of meningiomas are idiopathic, and benign with slow growth. Malignant meningiomas frequently demonstrate several chromosomal alterations. More genetic alterations are connected with greater tumor grade and rapid growth.

In sporadic meningiomas, chromosome 22 mutations in neurofibromatosis type 2 is one of the most common risk factors. Other documented chromosomal mutations in meningiomas include 1p, 6q, 14q, and 18q.

The majority of meningiomas are sporadic, however some have been linked to specific disorders and risk factors.

Certain factors can significantly increase the risk of meningioma incidence. These include:

  • Radiotherapy
  • Radiation
  • Exogenous hormone exposure
  • HRT
  • Breast Cancer
  • Use of oral contraceptive

Progesterone receptors are present in as much as 72% of cancers. According to studies, their size fluctuates throughout the luteal stage of the menstrual cycle and also during pregnancy. The increased frequency among women is attributable to hormonal factors.

The chance of developing meningioma is also significantly increased if an individual has any direct relatives who suffered from certain genetic syndromes such as:

  • Li-Fraumeni syndrome
  • Cowden disease
  • Gorlin syndrome
  • von Hippel Lindau disease
  • Neurofibromatosis type II

The 5-year progression-free survival rate for grade 1 is 95.7%, for grade 2 it is 81.8%, and for grade 3 it is 46.7%, while the 10-year survival rate for grade 1 is 90.4.7% without any progression of disease and for grade 2 it is 69.4%.

The common predictors of progression-free survival are:

  • Degree of resection
  • A mitotic index below 5%
  • Low histological grade
  • Tumor size of 6 cm or below
  • Extent of tumor multiplicity
  • Tumor location

Recurrence is related to the histological grade; grade 1 has a 4.9 percent recurrence rate, grade 2 has an 18.4% recurrence rate, and grade 3 has a 27.3% recurrence rate.

The 10-year survival rate for malignant meningiomas has been rising as a result of new therapy options. Partial tumor excision yields a 5-year survival rate of 85% for benign meningiomas, but only 58 percent for malignant meningiomas.

Meningioma tumor grade is a major predictive factor for individuals getting radiation therapy following surgery. Grades 1 and 2 were linked to a greater 3-year overall survival rate. After radiation, the 5-year overall survival rate for grade 1 meningiomas is around 81%, compared to 53% for grades 2 and 3.

https://www.ncbi.nlm.nih.gov/books/NBK560538/

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