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Pathology - Oligodendroglioma
Uncommon noncancerous growth originating from oligodendrocytes.
Typically impacts individuals in their middle years.
The pathology is a well-defined, slow-growing gray mass typically containing cysts. It commonly occurs in the white matter of the cerebral hemispheres, particularly in the frontal lobe.
Microscopic: Uniform cell sheets with spherical nuclei and clear cytoplasm resembling a fried egg; often contains calcifications and enhanced vascularity.
Clinical Symptoms
Seizures, headaches, papilledema, and other indicators of elevated intracranial pressure.
Imaging reveals the presence of tumor calcification on a CT scan.
Therapy
Surgical removal, subsequent radiation, and chemotherapy.
The average survival time post-diagnosis ranges from 5 to 10 years.
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Pathology - Schwannoma
Typically noncancerous growths originating from Schwann cells.
Pathology: Gross Encapsulated masses, frequently with cystic regions, typically found in the cerebellopontine angle, where they might compress cranial nerve VIII (acoustic schwannoma).
Microscopic: Two patterns of growth:
(1) Antoni A consists of densely packed elongated cells with nuclei arranged in a palisade pattern; (2) Antoni B is characterized by a loose organization of cells with small microcysts.
Manifests symptoms related to nerve compression (cranial nerve VIII compression results in patients experiencing ipsilateral hearing loss, tinnitus, and vertigo), seizures, headaches, nausea and vomiting, and other indications of elevated intracranial pressure.
Therapy
Tumor removal surgery.
The prognosis is favorable.
Neurofibromatosis type 2 is linked to bilateral auditory schwannomas.
Pineal tumors typically affect young males aged 10 to 40. They exhibit Parinaud syndrome, which includes paralysis of upward gaze resulting from injury to the pretectal and superior colliculus, obstructive hydrocephalus due to compression of the aqueduct of Sylvius, and endocrine problems related to compression of the hypothalamus.
Typically noncancerous growths originating from Schwann cells.
Pathology: Gross Encapsulated masses, frequently with cystic regions, typically found in the cerebellopontine angle, where they might compress cranial nerve VIII (acoustic schwannoma).
Microscopic: Two patterns of growth:
(1) Antoni A consists of densely packed elongated cells with nuclei arranged in a palisade pattern; (2) Antoni B is characterized by a loose organization of cells with small microcysts.
Manifests symptoms related to nerve compression (cranial nerve VIII compression results in patients experiencing ipsilateral hearing loss, tinnitus, and vertigo), seizures, headaches, nausea and vomiting, and other indications of elevated intracranial pressure.
Therapy
Tumor removal surgery.
The prognosis is favorable.
Neurofibromatosis type 2 is linked to bilateral auditory schwannomas.
Pineal tumors typically affect young males aged 10 to 40. They exhibit Parinaud syndrome, which includes paralysis of upward gaze resulting from injury to the pretectal and superior colliculus, obstructive hydrocephalus due to compression of the aqueduct of Sylvius, and endocrine problems related to compression of the hypothalamus.
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Pathology- Medulloblastoma
Malignant tumor originating in the cerebellum, linked to a deletion on the short arm of chromosome 17 (17p-).
Primarily found in youngsters and represents 20% of all brain tumors in this age group.
The tumor is gray, well-defined, and situated at the midline of the cerebellum.
Microscopic examination reveals densely packed sheets of undifferentiated cells with numerous divisions, minimal cytoplasm, and intensely stained nuclei. The cells are frequently organized in a circular or pseudo-circular pattern around blood vessels.
Unstable walking; blockage of cerebrospinal fluid flow due to tumor compressing the fourth ventricle leading to obstructive hydrocephalus; seizures, headaches; nausea, vomiting; and other symptoms of elevated intracranial pressure.
Therapy Surgical intervention combined with radiation and chemotherapy.
The 5-year survival rate is 75% with complete removal of the tumor plus radiation therapy.
Malignant tumor originating in the cerebellum, linked to a deletion on the short arm of chromosome 17 (17p-).
Primarily found in youngsters and represents 20% of all brain tumors in this age group.
The tumor is gray, well-defined, and situated at the midline of the cerebellum.
Microscopic examination reveals densely packed sheets of undifferentiated cells with numerous divisions, minimal cytoplasm, and intensely stained nuclei. The cells are frequently organized in a circular or pseudo-circular pattern around blood vessels.
Unstable walking; blockage of cerebrospinal fluid flow due to tumor compressing the fourth ventricle leading to obstructive hydrocephalus; seizures, headaches; nausea, vomiting; and other symptoms of elevated intracranial pressure.
Therapy Surgical intervention combined with radiation and chemotherapy.
The 5-year survival rate is 75% with complete removal of the tumor plus radiation therapy.
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Pathology - Ependymoma
Tumor originating from the ependyma of the ventricular system.
Primarily found in youngsters, typically in the fourth ventricle, but can also develop in the spinal cord of adults.
Pathology: Solid, papillary masses protruding from the floor of the fourth ventricle.
The cells are uniform and round with nuclei distributed in a fibrillary stroma in a perivascular pseudorosette formation. Tumor cells frequently contain blepharoplasts, which are rods located near the nucleus and represent basal ciliary bodies.
Obstructive hydrocephalus occurs when a tumor blocks the flow of cerebrospinal fluid by compressing the fourth ventricle.
Clinical Symptoms and Signs
Seizures, headaches, nausea, vomiting, and other symptoms indicating elevated intracranial pressure.
Therapies
Surgical removal is challenging due to the close proximity of brainstem nuclei.
The prognosis is unfavorable with an average survival time of 4 years.
Tumor originating from the ependyma of the ventricular system.
Primarily found in youngsters, typically in the fourth ventricle, but can also develop in the spinal cord of adults.
Pathology: Solid, papillary masses protruding from the floor of the fourth ventricle.
The cells are uniform and round with nuclei distributed in a fibrillary stroma in a perivascular pseudorosette formation. Tumor cells frequently contain blepharoplasts, which are rods located near the nucleus and represent basal ciliary bodies.
Obstructive hydrocephalus occurs when a tumor blocks the flow of cerebrospinal fluid by compressing the fourth ventricle.
Clinical Symptoms and Signs
Seizures, headaches, nausea, vomiting, and other symptoms indicating elevated intracranial pressure.
Therapies
Surgical removal is challenging due to the close proximity of brainstem nuclei.
The prognosis is unfavorable with an average survival time of 4 years.
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Pathology - Neuroblastoma
Neoplasm originating from neural precursor cells; linked to N-myc oncogene amplification and deletions in the short arm of chromosome 1 (1p-).
Most frequently observed in young children, although does occasionally happen in adults.
Pathology: Typically originates in the adrenal medulla but can also develop in the sympathetic chain, pelvis, neck, or brain. It varies in size, may have well-defined borders, and can exhibit cyst formation or necrosis.
Microscopic examination reveals sheets of tiny cells with dark nuclei and little cytoplasm, typically organized in Homer-Wright pseudorosettes. These cells contain neurosecretory granules that store catecholamines.
The typical scenario involves a child under 2 years old presenting with a significant abdominal mass, high blood pressure, and weight loss. Additional symptoms consist of ecchymosis and proptosis (eye protrusion).
Older children might show signs of cancer spreading to the bone, liver, or lungs, which can cause symptoms including bone pain, respiratory issues, or gastrointestinal problems.
Laboratory results show elevated 24-hour urinary VMA and metanephrine levels, as well as higher plasma and urinary catecholamine levels.
Therapy
Combination of surgical removal and chemotherapy.
The prognosis varies depending on the patient's age and the stage of cancer at diagnosis. Younger patients and those with lower cancer stages tend to have a more favorable prognosis.
Neoplasm originating from neural precursor cells; linked to N-myc oncogene amplification and deletions in the short arm of chromosome 1 (1p-).
Most frequently observed in young children, although does occasionally happen in adults.
Pathology: Typically originates in the adrenal medulla but can also develop in the sympathetic chain, pelvis, neck, or brain. It varies in size, may have well-defined borders, and can exhibit cyst formation or necrosis.
Microscopic examination reveals sheets of tiny cells with dark nuclei and little cytoplasm, typically organized in Homer-Wright pseudorosettes. These cells contain neurosecretory granules that store catecholamines.
The typical scenario involves a child under 2 years old presenting with a significant abdominal mass, high blood pressure, and weight loss. Additional symptoms consist of ecchymosis and proptosis (eye protrusion).
Older children might show signs of cancer spreading to the bone, liver, or lungs, which can cause symptoms including bone pain, respiratory issues, or gastrointestinal problems.
Laboratory results show elevated 24-hour urinary VMA and metanephrine levels, as well as higher plasma and urinary catecholamine levels.
Therapy
Combination of surgical removal and chemotherapy.
The prognosis varies depending on the patient's age and the stage of cancer at diagnosis. Younger patients and those with lower cancer stages tend to have a more favorable prognosis.
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Pathology - Neurofibromatosis
Autosomal dominant disorder resulting from a mutation in the neurofibromatosis type 1 (NF1) gene, a tumor suppressor gene situated on chromosome 17.
Pathology
Neurofibroma is a type of tumor made up of spindle cells that can be found in the dermis, peripheral nerves, or major nerve trunks. Cutaneous neurofibromas appear as skin nodules and may lead to darkening of the skin above them.
Clinical Neurofibromas can result in neurological symptoms depending on their position on various nerves, for example, gliomas on the optic nerve might cause vision abnormalities. Patients may have Lisch nodules (pigmented nodules of the iris) or Cafe-au-lait spots (pigmented macules on the skin) upon examination. Bending and tilting of long bones such as the tibia or radial bone, or the development of scoliosis, can lead to symptoms resembling arthritis. Hypertension is a prevalent condition.
Therapy
Surgery is performed to remove neurofibromas if they are causing disfigurement or neurological problems. Hypertension is treated if necessary.
Neurofibromatosis type 2 is an autosomal dominant disorder caused by a mutation in the NF2 gene on chromosome 22. It is less common than neurofibromatosis type 1 and is characterized by bilateral auditory schwannomas, numerous meningiomas, and other neoplasms.
Autosomal dominant disorder resulting from a mutation in the neurofibromatosis type 1 (NF1) gene, a tumor suppressor gene situated on chromosome 17.
Pathology
Neurofibroma is a type of tumor made up of spindle cells that can be found in the dermis, peripheral nerves, or major nerve trunks. Cutaneous neurofibromas appear as skin nodules and may lead to darkening of the skin above them.
Clinical Neurofibromas can result in neurological symptoms depending on their position on various nerves, for example, gliomas on the optic nerve might cause vision abnormalities. Patients may have Lisch nodules (pigmented nodules of the iris) or Cafe-au-lait spots (pigmented macules on the skin) upon examination. Bending and tilting of long bones such as the tibia or radial bone, or the development of scoliosis, can lead to symptoms resembling arthritis. Hypertension is a prevalent condition.
Therapy
Surgery is performed to remove neurofibromas if they are causing disfigurement or neurological problems. Hypertension is treated if necessary.
Neurofibromatosis type 2 is an autosomal dominant disorder caused by a mutation in the NF2 gene on chromosome 22. It is less common than neurofibromatosis type 1 and is characterized by bilateral auditory schwannomas, numerous meningiomas, and other neoplasms.
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Pathology - Osteogenesis Imperfecta
Inherited mutations lead to a lack of type 1 collagen synthesis, affecting bone, teeth, ears, eyes, and skin. Inheritance is primarily autosomal dominant, although there are occasional instances of autosomal recessive inheritance. There are four varieties, with type II being the most severe and typically leading to neonatal mortality.
Bone shows cortical thinning with trabecular thinning resembling osteoporosis.
Multiple fractures resulting from minor trauma; blue sclerae caused by the transparency of connective tissue over choroids; hearing loss due to aberrant middle ear bones; dental abnormalities.
Therapy
Utilizing pneumatic bracing to prevent trauma.
The prognosis varies depending on the type of disease and might range from mortality in infancy to a typical lifespan.
Achondroplasia is the outcome of an autosomal dominant mutation in the fibroblast growth factor receptor 3 (FGFR3) gene on chromosome 4, leading to irregularities in cartilage calcification and restructuring. It presents clinically as dwarfism, with small limbs and a normal trunk, and is defined by narrow epiphyseal plates and short, thick bones.
Inherited mutations lead to a lack of type 1 collagen synthesis, affecting bone, teeth, ears, eyes, and skin. Inheritance is primarily autosomal dominant, although there are occasional instances of autosomal recessive inheritance. There are four varieties, with type II being the most severe and typically leading to neonatal mortality.
Bone shows cortical thinning with trabecular thinning resembling osteoporosis.
Multiple fractures resulting from minor trauma; blue sclerae caused by the transparency of connective tissue over choroids; hearing loss due to aberrant middle ear bones; dental abnormalities.
Therapy
Utilizing pneumatic bracing to prevent trauma.
The prognosis varies depending on the type of disease and might range from mortality in infancy to a typical lifespan.
Achondroplasia is the outcome of an autosomal dominant mutation in the fibroblast growth factor receptor 3 (FGFR3) gene on chromosome 4, leading to irregularities in cartilage calcification and restructuring. It presents clinically as dwarfism, with small limbs and a normal trunk, and is defined by narrow epiphyseal plates and short, thick bones.
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Pathology - Osteopetrosis
abnormal bone resorption as a result of osteoclast activity dysfunction.
There are other varieties, such as a less severe autosomal dominant form and a deadly autosomal recessive variety.
Pathology
Gross: Dense, thick bones; widened ends of bones (Erlenmeyer flask appearance).
Microscopic: Absence of hematopoietic marrow or trabeculae; persistence of primary spongiosa, calcified cartilage generated during endochondral bone formation.
Clinical Signs and Symptoms
Multiple fractures; infections; hepatosplenomegaly (from extramedullary hematopoiesis); cranial nerve palsies (blindness, deafness, facial paralysis); and various neuropathies (from nerve compression brought on by restricted neural foramina).
Treatment
corticosteroids and bone marrow transplantation as treatments.
A condition known as McCune-Albright syndrome affects young girls. Cafe-au-lait spots, small stature, early puberty (or other endocrine hyperfunctions like acromegaly or hyperthyroidism), and polyostotic fibrous dysplasia (fibrous replacement of medullary bone in various sites) are some of its manifestations.
abnormal bone resorption as a result of osteoclast activity dysfunction.
There are other varieties, such as a less severe autosomal dominant form and a deadly autosomal recessive variety.
Pathology
Gross: Dense, thick bones; widened ends of bones (Erlenmeyer flask appearance).
Microscopic: Absence of hematopoietic marrow or trabeculae; persistence of primary spongiosa, calcified cartilage generated during endochondral bone formation.
Clinical Signs and Symptoms
Multiple fractures; infections; hepatosplenomegaly (from extramedullary hematopoiesis); cranial nerve palsies (blindness, deafness, facial paralysis); and various neuropathies (from nerve compression brought on by restricted neural foramina).
Treatment
corticosteroids and bone marrow transplantation as treatments.
A condition known as McCune-Albright syndrome affects young girls. Cafe-au-lait spots, small stature, early puberty (or other endocrine hyperfunctions like acromegaly or hyperthyroidism), and polyostotic fibrous dysplasia (fibrous replacement of medullary bone in various sites) are some of its manifestations.
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Pathology - Epidural Hematoma and Subdural Hematoma
Epidural hematoma is typically the result of the rupture of the middle meningeal artery, middle meningeal vein, or dural sinus, generally due to a skull fracture.
Subdural hematoma is the result of the rupture of bridge veins situated between the cerebrum and venous sinuses in the dura, typically caused by head trauma.
Epidural hematoma: Blood buildup between the dura mater and the skull causing compression on the brain.
Subdural hematoma is the accumulation of blood between the dura mater and arachnoid membrane. The bleeding is self-limited, but the hematoma might expand due to osmotic water movement. Resolution may occur with the formation of granulation tissue, leading to a chronic subdural hematoma.
Epidural anesthesia leads to unconsciousness, a clear period of awareness, headache, changes in mental function, seizures, specific neurological impairments, and ultimately a state of unconsciousness. Imaging findings: Head CT reveals a biconcave disk that does not extend across suture lines.
Subdural symptoms include headache, altered mental status, and evidence of cerebral compression. These clinical manifestations develop gradually, typically emerging hours to weeks after the injury. Imaging: Head CT reveals a crescent-shaped disk that extends across suture lines.
Treatment.
Surgically draining blood and correcting coagulopathy.
Epidural hematoma is typically the result of the rupture of the middle meningeal artery, middle meningeal vein, or dural sinus, generally due to a skull fracture.
Subdural hematoma is the result of the rupture of bridge veins situated between the cerebrum and venous sinuses in the dura, typically caused by head trauma.
Epidural hematoma: Blood buildup between the dura mater and the skull causing compression on the brain.
Subdural hematoma is the accumulation of blood between the dura mater and arachnoid membrane. The bleeding is self-limited, but the hematoma might expand due to osmotic water movement. Resolution may occur with the formation of granulation tissue, leading to a chronic subdural hematoma.
Epidural anesthesia leads to unconsciousness, a clear period of awareness, headache, changes in mental function, seizures, specific neurological impairments, and ultimately a state of unconsciousness. Imaging findings: Head CT reveals a biconcave disk that does not extend across suture lines.
Subdural symptoms include headache, altered mental status, and evidence of cerebral compression. These clinical manifestations develop gradually, typically emerging hours to weeks after the injury. Imaging: Head CT reveals a crescent-shaped disk that extends across suture lines.
Treatment.
Surgically draining blood and correcting coagulopathy.
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Pathology - Hydrocephalus
Hydrocephalus is caused by the buildup of excess cerebrospinal fluid in the skull, which can be due to blockages in CSF flow caused by tumors or inflammation, or by the excessive production of CSF by tumors in the choroid plexus.
Pathology: Ventricular dilation observed on gross examination.
There are four types of hydrocephalus: (1) Internal hydrocephalus: excess cerebrospinal fluid (CSF) is found exclusively in the ventricular system; (2) External hydrocephalus: excess CSF is found only in the subarachnoid space; (3) Communicating hydrocephalus: CSF flows freely between the ventricles and subarachnoid space; (4) Noncommunicating hydrocephalus: there is an obstruction in the flow of CSF between the ventricles and subarachnoid space.
Clinical Symptoms and Signs
May manifest as skull enlargement in adults, seizures, headaches, vision problems, nausea, vomiting, and other symptoms of elevated intracranial pressure.
Therapy
Placement of ventriculoperitoneal shunt; extraction of blockage or choroid plexus tumor.
Hydrocephalus ex vacuo is the enlargement of the brain's ventricles due to a higher volume of cerebrospinal fluid caused by a reduction in brain tissue, typically due to conditions like infarction or Alzheimer's disease.
Normal-pressure hydrocephalus is a clinical condition identified by the presence of magnetic gait, urine incontinence, and dementia. Brain imaging shows ventricular enlargement. A lumbar puncture shows a normal opening pressure and offers both diagnostic and therapeutic advantages, as symptoms improve when cerebrospinal fluid is removed during the procedure. The definitive treatment involves the insertion of a ventriculoperitoneal shunt.
Hydrocephalus is caused by the buildup of excess cerebrospinal fluid in the skull, which can be due to blockages in CSF flow caused by tumors or inflammation, or by the excessive production of CSF by tumors in the choroid plexus.
Pathology: Ventricular dilation observed on gross examination.
There are four types of hydrocephalus: (1) Internal hydrocephalus: excess cerebrospinal fluid (CSF) is found exclusively in the ventricular system; (2) External hydrocephalus: excess CSF is found only in the subarachnoid space; (3) Communicating hydrocephalus: CSF flows freely between the ventricles and subarachnoid space; (4) Noncommunicating hydrocephalus: there is an obstruction in the flow of CSF between the ventricles and subarachnoid space.
Clinical Symptoms and Signs
May manifest as skull enlargement in adults, seizures, headaches, vision problems, nausea, vomiting, and other symptoms of elevated intracranial pressure.
Therapy
Placement of ventriculoperitoneal shunt; extraction of blockage or choroid plexus tumor.
Hydrocephalus ex vacuo is the enlargement of the brain's ventricles due to a higher volume of cerebrospinal fluid caused by a reduction in brain tissue, typically due to conditions like infarction or Alzheimer's disease.
Normal-pressure hydrocephalus is a clinical condition identified by the presence of magnetic gait, urine incontinence, and dementia. Brain imaging shows ventricular enlargement. A lumbar puncture shows a normal opening pressure and offers both diagnostic and therapeutic advantages, as symptoms improve when cerebrospinal fluid is removed during the procedure. The definitive treatment involves the insertion of a ventriculoperitoneal shunt.
