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{{Short description|Rare genetic disorder affecting albumin production}}
{{Infobox medical condition (new)
{{Medical condition|name=Analbuminaemia}}
| name            = Anaplastic astrocytoma
| synonyms        =
| image          = Anaplastic_astrocytoma_-_very_high_mag - cropped.jpg
| caption        = [[Micrograph]] of an anaplastic astrocytoma. [[H&E stain]].
| pronounce      =
| field          = [[Neurosurgery]]
| symptoms        =
| complications  =
| onset          =
| duration        =
| types          =
| causes          =
| risks          =
| diagnosis      =
| differential    =
| prevention      =
| treatment      =
| medication      =
| prognosis      =
| frequency      =
| deaths          =
}}{{Short description|Rare genetic disorder affecting albumin production}}
== Anaplastic Astrocytoma ==
'''Anaplastic astrocytoma''' is a rare [[WHO]] grade III type of [[astrocytoma]], which is a type of [[brain tumor]]. In the [[United States]], the annual incidence rate for anaplastic astrocytoma is 0.44 per 100,000 people.


'''Analbuminaemia''' is a rare [[genetic disorder]] characterized by the absence or severe reduction of [[albumin]] in the blood plasma. Albumin is a vital protein produced by the [[liver]], playing a crucial role in maintaining the [[oncotic pressure]] necessary for proper distribution of body fluids between body tissues and the bloodstream. This condition is inherited in an [[autosomal recessive]] manner.
== Signs and Symptoms ==
Initial presenting symptoms most commonly include [[headache]], depressed [[mental status]], focal [[neurological deficit]]s, and/or [[seizure]]s. The growth rate and mean interval between onset of symptoms and diagnosis is approximately 1.5–2 years but is highly variable, being intermediate between that of [[low-grade astrocytoma]]s and [[glioblastoma]]s. Seizures are less common among patients with anaplastic astrocytomas compared to low-grade lesions.


==Pathophysiology==
== Causes ==
Albumin is the most abundant protein in human plasma, accounting for about 60% of the total plasma protein. It is essential for maintaining the [[colloid osmotic pressure]] of the blood, which prevents the leakage of fluid from the blood vessels into the tissues. In individuals with analbuminaemia, the absence of albumin leads to a compensatory increase in other plasma proteins, such as [[globulins]], to maintain oncotic pressure. However, this compensation is often inadequate, leading to various clinical manifestations.
Most high-grade [[glioma]]s occur sporadically or without identifiable cause. However, a small proportion (less than 5%) of individuals with [[malignant astrocytoma]] have a definite or suspected hereditary predisposition. The main hereditary predispositions include [[neurofibromatosis type I]], [[Li-Fraumeni syndrome]], [[hereditary nonpolyposis colorectal cancer]], and [[tuberous sclerosis]]. Anaplastic astrocytomas have also been associated with previous exposure to [[vinyl chloride]] and high doses of [[radiation therapy]] to the [[brain]].


==Clinical Features==
== Pathology ==
Patients with analbuminaemia may present with a range of symptoms, although some individuals remain asymptomatic. Common clinical features include:
Anaplastic astrocytomas are classified as high-grade gliomas ([[WHO]] grade III-IV), which are pathologically undifferentiated gliomas that carry a poor clinical prognosis. Unlike [[glioblastoma]]s ([[WHO]] grade IV), anaplastic astrocytomas lack [[vascular proliferation]] and [[necrosis]] on pathological evaluation. Compared to grade II tumors, anaplastic astrocytomas are more cellular, exhibit more atypia, and display increased mitotic activity.
* [[Edema]]: Due to the reduced oncotic pressure, fluid may accumulate in the interstitial spaces, leading to swelling, particularly in the lower extremities.
* [[Hyperlipidemia]]: Elevated levels of lipids in the blood are often observed, as albumin plays a role in lipid transport.
* [[Fatigue]] and [[muscle weakness]]: These symptoms may result from the body's inability to maintain proper fluid and electrolyte balance.
* [[Hypotension]]: Low blood pressure can occur due to the reduced blood volume.


==Diagnosis==
== Treatment ==
The diagnosis of analbuminaemia is typically made through laboratory tests that reveal low or undetectable levels of albumin in the blood. Genetic testing can confirm the diagnosis by identifying mutations in the [[albumin gene]] (ALB) responsible for the condition.
The standard initial treatment involves [[surgical resection]] to remove as much of the tumor as possible without worsening neurological deficits. [[Radiation therapy]] has been shown to prolong survival and is a standard component of treatment. There is no proven benefit to adjuvant [[chemotherapy]] or supplementing other treatments for this type of tumor. Although [[temozolomide]] is effective for treating recurrent anaplastic astrocytoma, its role as an adjuvant to radiation therapy has not been fully tested.


==Management==
Quality of life after treatment depends heavily on the affected area of the brain. In many cases, patients with anaplastic astrocytoma may experience various types of [[paralysis]], [[speech disorder]]s, difficulties in [[executive function]], and altered [[sensory perception]]. Most cases of paralysis and speech difficulties can be rehabilitated with [[speech therapy]], [[occupational therapy]], [[physical therapy]], and [[vision therapy]].
There is no specific treatment for analbuminaemia. Management focuses on alleviating symptoms and preventing complications. This may include:
* Dietary modifications to manage hyperlipidemia.
* Diuretics to reduce edema.
* Monitoring and managing blood pressure.


==Prognosis==
== Prognosis ==
The prognosis for individuals with analbuminaemia varies. Some individuals may lead relatively normal lives with minimal symptoms, while others may experience significant complications. Regular monitoring and supportive care are essential to manage the condition effectively.
The age-standardized 5-year relative survival rate for anaplastic astrocytoma is 23.6%. Patients with this tumor are significantly more likely to succumb to the disease compared to matched members of the general population. Prognosis varies across age groups, particularly during the first three years post-diagnosis. Older adults are at a significantly higher risk of mortality within the first year post-diagnosis compared to young adults (aged 15 to 40), but after three years, this difference is markedly reduced.


==Genetics==
Typical median survival for anaplastic astrocytoma is 2–3 years. Secondary progression to [[glioblastoma multiforme]] is common. Factors associated with improved survival in anaplastic astrocytoma patients include [[radiation therapy]], younger age, female sex, treatment after the year 2000, and [[surgery]].
Analbuminaemia is inherited in an [[autosomal recessive]] pattern, meaning that an individual must inherit two copies of the mutated gene, one from each parent, to be affected. Carriers, who have only one copy of the mutation, typically do not show symptoms.
{{Nervous tissue tumors}}
 
[[Category:Brain tumor]]
==Related pages==
== Anaplastic astrocytoma ==
* [[Albumin]]
* [[Hypoalbuminemia]]
* [[Genetic disorder]]
* [[Autosomal recessive inheritance]]
 
==Gallery==
<gallery>
<gallery>
File:Autosomal recessive - en.svg|Diagram illustrating autosomal recessive inheritance.
File:Anaplastic_astrocytoma_-_very_high_mag_-_cropped.jpg
</gallery>
</gallery>
[[Category:Genetic disorders]]
[[Category:Rare diseases]]
[[Category:Blood disorders]]

Latest revision as of 00:57, 17 February 2025

Anaplastic astrocytoma
Synonyms
Pronounce
Field Neurosurgery
Symptoms
Complications
Onset
Duration
Types
Causes
Risks
Diagnosis
Differential diagnosis
Prevention
Treatment
Medication
Prognosis
Frequency
Deaths


Rare genetic disorder affecting albumin production


Anaplastic Astrocytoma[edit]

Anaplastic astrocytoma is a rare WHO grade III type of astrocytoma, which is a type of brain tumor. In the United States, the annual incidence rate for anaplastic astrocytoma is 0.44 per 100,000 people.

Signs and Symptoms[edit]

Initial presenting symptoms most commonly include headache, depressed mental status, focal neurological deficits, and/or seizures. The growth rate and mean interval between onset of symptoms and diagnosis is approximately 1.5–2 years but is highly variable, being intermediate between that of low-grade astrocytomas and glioblastomas. Seizures are less common among patients with anaplastic astrocytomas compared to low-grade lesions.

Causes[edit]

Most high-grade gliomas occur sporadically or without identifiable cause. However, a small proportion (less than 5%) of individuals with malignant astrocytoma have a definite or suspected hereditary predisposition. The main hereditary predispositions include neurofibromatosis type I, Li-Fraumeni syndrome, hereditary nonpolyposis colorectal cancer, and tuberous sclerosis. Anaplastic astrocytomas have also been associated with previous exposure to vinyl chloride and high doses of radiation therapy to the brain.

Pathology[edit]

Anaplastic astrocytomas are classified as high-grade gliomas (WHO grade III-IV), which are pathologically undifferentiated gliomas that carry a poor clinical prognosis. Unlike glioblastomas (WHO grade IV), anaplastic astrocytomas lack vascular proliferation and necrosis on pathological evaluation. Compared to grade II tumors, anaplastic astrocytomas are more cellular, exhibit more atypia, and display increased mitotic activity.

Treatment[edit]

The standard initial treatment involves surgical resection to remove as much of the tumor as possible without worsening neurological deficits. Radiation therapy has been shown to prolong survival and is a standard component of treatment. There is no proven benefit to adjuvant chemotherapy or supplementing other treatments for this type of tumor. Although temozolomide is effective for treating recurrent anaplastic astrocytoma, its role as an adjuvant to radiation therapy has not been fully tested.

Quality of life after treatment depends heavily on the affected area of the brain. In many cases, patients with anaplastic astrocytoma may experience various types of paralysis, speech disorders, difficulties in executive function, and altered sensory perception. Most cases of paralysis and speech difficulties can be rehabilitated with speech therapy, occupational therapy, physical therapy, and vision therapy.

Prognosis[edit]

The age-standardized 5-year relative survival rate for anaplastic astrocytoma is 23.6%. Patients with this tumor are significantly more likely to succumb to the disease compared to matched members of the general population. Prognosis varies across age groups, particularly during the first three years post-diagnosis. Older adults are at a significantly higher risk of mortality within the first year post-diagnosis compared to young adults (aged 15 to 40), but after three years, this difference is markedly reduced.

Typical median survival for anaplastic astrocytoma is 2–3 years. Secondary progression to glioblastoma multiforme is common. Factors associated with improved survival in anaplastic astrocytoma patients include radiation therapy, younger age, female sex, treatment after the year 2000, and surgery.

Tumours of the nervous system
Endocrine
Sellar:
Other:
CNS
PNS:
Note: Not all brain tumors are of nervous tissue, and not all nervous tissue tumors are in the brain (see brain metastasis).

Anaplastic astrocytoma[edit]

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