Ganglioglioma

Last updated
Ganglioglioma
Other namesGangliocytoma
Dysplastic cerebellar gangliocytoma.jpg
Dysplastic cerebellar ganglioglioma (Lhermitte–Duclos disease)
Specialty Neuro-oncology
Usual onsetUsually childhood to young adulthood [1]
TreatmentPrimarily surgery
Prognosis >90% five-year survival rate among children [2]

A ganglioglioma (or gangliocytoma) is a rare, slow-growing primary central nervous system (CNS) tumor which most frequently occurs in the temporal lobes of children and young adults. [3] They are mixed cell tumors containing both neural ganglionic cells and neural glial cell components. [4]

Contents

Classification

Gangliogliomas are generally benign WHO grade I tumors; the presence of anaplastic changes in the glial component is considered to represent WHO grade III (anaplastic ganglioglioma). Criteria for WHO grade II have been suggested, but are not established. Malignant transformation of spinal ganglioglioma has been seen in only a select few cases. Poor prognostic factors for adults with gangliogliomas include older age at diagnosis, male sex, and malignant histologic features.

Histopathology

Histologically, ganglioglioma is composed of both neoplastic glial and ganglion cells which are disorganized, variably cellular, and non-infiltrative. Occasionally, it may be challenging to differentiate ganglion cell tumors from an infiltrating glioma with entrapped neurons. The presence of neoplastic ganglion cells forming abnormal clusters, the presence of binucleation and dysmorphic neurons are helpful clues favoring ganglioglioma over glioma. The glial component of ganglioglioma includes fibrillary astrocytes with varying degrees of cellular atypia. The neoplastic neuronal components are often clustered or irregularly oriented. Fibrovascular stroma confined to the neuronal component, perivascular lymphocytic infiltrates, and small foci of calcification are common, as is immunopositivity for synaptophysin, neuron-specific enolase, and chromogranin A. Elevated Ki-67 and p53 labeling index is associated with more aggressive tumor behavior in both children and adults with gangliogliomas. The rare occurrence of malignant transformation is confined to the glial cell population, and is characterized by increased cellularity and mitotic activity, endothelial proliferation, and necrosis.

Diagnosis

Computed Tomography (CT) is generally not a recommended modality for diagnosis and evaluation of spinal cord tumors. Evaluation with Magnetic Resonance (MR) most commonly demonstrates a circumscribed solid or mixed solid and cystic mass spanning a long segment of the cord with hypointense T1 signal and hyperintense T2 signal in the solid component. Enhancement patterns are highly variable, ranging from minimal to marked, and may be solid, rim, or nodular. Adjacent cord edema and syringomyelia and peritumoral cysts may be present in addition to reactive scoliosis.

It is nearly impossible to differentiate ganglioglioma from other more common intramedullary neoplasms based on imaging alone. Astrocytoma and ependymoma are more familiar intramedullary tumors which share many similar features to ganglioglioma, including T2 hyperintensity, enhancement, tumoral cysts, and cord edema. Poorly defined margins may be more suggestive of astrocytoma, while a central location in the spinal cord, hemorrhage, and hemosiderin staining are often seen with ependymoma. Hemangioblastoma and paraganglioma are less usual intramedullary tumors, but since they are more frequently encountered than ganglioglioma, they should also be included in the differential diagnosis.

Treatment

Definitive treatment for ganglioglioma requires gross total surgical resection, and a good prognosis is generally expected when this is achieved. However, indistinct tumor margins and the desire to preserve normal spinal cord tissue, motor and sensory function may preclude complete resection of tumor. According to a series by Lang et al., reviewing several patients with resected spinal cord ganglioglioma, the 5- and 10-year survival rates after total resection were 89% and 83%, respectively. [5] In that study, patients with spinal cord ganglioglioma had a 3.5-fold higher relative risk of tumor recurrence compared to patients with supratentorial ganglioglioma. It has been recognized that postoperative results correlate closely with preoperative neurological status as well as the ability to achieve complete resection.

With the exception of WHO grade III anaplastic ganglioglioma, radiation therapy is generally regarded to have no role in the treatment of ganglioglioma. In fact, radiation therapy may induce malignant transformation of a recurrent ganglioglioma several years later. Adjuvant chemotherapy is also typically reserved for anaplastic ganglioglioma, but has been used anecdotally in partially resected low grade spinal cord gangliogliomas which show evidence of disease progression.

See also

Related Research Articles

<span class="mw-page-title-main">Brain tumor</span> Neoplasm in the brain

A brain tumor occurs when abnormal cells form within the brain. There are two main types of tumors: malignant (cancerous) tumors and benign (non-cancerous) tumors. These can be further classified as primary tumors, which start within the brain, and secondary tumors, which most commonly have spread from tumors located outside the brain, known as brain metastasis tumors. All types of brain tumors may produce symptoms that vary depending on the size of the tumor and the part of the brain that is involved. Where symptoms exist, they may include headaches, seizures, problems with vision, vomiting and mental changes. Other symptoms may include difficulty walking, speaking, with sensations, or unconsciousness.

<span class="mw-page-title-main">Nervous tissue</span> Main component of the nervous system

Nervous tissue, also called neural tissue, is the main tissue component of the nervous system. The nervous system regulates and controls body functions and activity. It consists of two parts: the central nervous system (CNS) comprising the brain and spinal cord, and the peripheral nervous system (PNS) comprising the branching peripheral nerves. It is composed of neurons, also known as nerve cells, which receive and transmit impulses, and neuroglia, also known as glial cells or glia, which assist the propagation of the nerve impulse as well as provide nutrients to the neurons.

<span class="mw-page-title-main">Glioma</span> Tumour of the glial cells of the brain or spine

A glioma is a type of tumor that starts in the glial cells of the brain or the spine. Gliomas comprise about 30 percent of all brain tumors and central nervous system tumours, and 80 percent of all malignant brain tumours.

Spinal tumors are neoplasms located in either the vertebral column or the spinal cord. There are three main types of spinal tumors classified based on their location: extradural and intradural. Extradural tumors are located outside the dura mater lining and are most commonly metastatic. Intradural tumors are located inside the dura mater lining and are further subdivided into intramedullary and extramedullary tumors. Intradural-intramedullary tumors are located within the dura and spinal cord parenchyma, while intradural-extramedullary tumors are located within the dura but outside the spinal cord parenchyma. The most common presenting symptom of spinal tumors is nocturnal back pain. Other common symptoms include muscle weakness, sensory loss, and difficulty walking. Loss of bowel and bladder control may occur during the later stages of the disease.

<span class="mw-page-title-main">Oligodendroglioma</span> Medical condition

Oligodendrogliomas are a type of glioma that are believed to originate from the oligodendrocytes of the brain or from a glial precursor cell. They occur primarily in adults but are also found in children.

<span class="mw-page-title-main">Ependymoma</span> Medical condition

An ependymoma is a tumor that arises from the ependyma, a tissue of the central nervous system. Usually, in pediatric cases the location is intracranial, while in adults it is spinal. The common location of intracranial ependymomas is the fourth ventricle. Rarely, ependymomas can occur in the pelvic cavity.

<span class="mw-page-title-main">Astrocytoma</span> Medical condition

Astrocytoma is a type of brain tumor. Astrocytomas originate from a specific kind of star-shaped glial cell in the cerebrum called an astrocyte. This type of tumor does not usually spread outside the brain and spinal cord and it does not usually affect other organs. After glioblastomas, astrocytomas are the second most common glioma and can occur in most parts of the brain and occasionally in the spinal cord.

The International Classification of Diseases for Oncology (ICD-O) is a domain-specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. This classification is widely used by cancer registries.

<span class="mw-page-title-main">Oligoastrocytoma</span> Medical condition

Oligoastrocytomas are a subset of brain tumors that present with an appearance of mixed glial cell origin, astrocytoma and oligodendroglioma. However, the term "Oligoastrocytoma" is now considered obsolete by the National Comprehensive Cancer Network stating "the term should no longer be used as such morphologically ambiguous tumors can be reliably resolved into astrocytomas and oligodendrogliomas with molecular testing."

<span class="mw-page-title-main">Gemistocyte</span> Swollen and reactive astrocyte

A gemistocyte is a swollen, reactive astrocyte.

Glial tumor is a general term for numerous tumors of the central nervous system, including astrocytomas, ependymal tumors, Oligodendroglioma, and primitive neuroectodermal tumors. The World Health Organization (WHO) classifies tumors into different categories according to severity and recurrence.The first tumor classified as grade I is called pilocytic astrocytoma and it is most commonly observed in children rather than adults. The next tumor is never common in the Dns called diffuse astrocytoma and it is considered a grade II, they are benign, or noncancerous, but can become malignant, meaning cancerous, as the tumor progresses. Grades III and grade IV are considered malignant astrocytomas. Anaplastic astrocytomas are considered by the WHO to be a grade III astrocytoma and Glioblastoma is a grade IV both are referred to high-grade glial tumors.

<span class="mw-page-title-main">WHO classification of tumours of the central nervous system</span>

The WHOclassification of tumours of the central nervous system is a World Health Organization Blue Book that defines, describes and classifies tumours of the central nervous system (CNS).

<span class="mw-page-title-main">Subependymoma</span> Relatively benign brain cancer involving ependymal cells

A subependymoma is a type of brain tumor; specifically, it is a rare form of ependymal tumor. They are usually in middle aged people. Earlier, they were called subependymal astrocytomas.

Neuro-oncology is the study of brain and spinal cord neoplasms, many of which are very dangerous and life-threatening. Among the malignant brain cancers, gliomas of the brainstem and pons, glioblastoma multiforme, and high-grade astrocytoma/oligodendroglioma are among the worst. In these cases, untreated survival usually amounts to only a few months, and survival with current radiation and chemotherapy treatments may extend that time from around a year to a year and a half, possibly two or more, depending on the patient's condition, immune function, treatments used, and the specific type of malignant brain neoplasm. Surgery may in some cases be curative, but, as a general rule, malignant brain cancers tend to regenerate and emerge from remission easily, especially highly malignant cases. In such cases, the goal is to excise as much of the mass and as much of the tumor margin as possible without endangering vital functions or other important cognitive abilities. The Journal of Neuro-Oncology is the longest continuously published journal in the field and serves as a leading reference to those practicing in the area of neuro-oncology.

<span class="mw-page-title-main">Fibrillary astrocytoma</span> Type of brain tumor

Fibrillary astrocytomas are a group of primary slow-growing brain tumors that typically occur in adults between the ages of 20 and 50.

<span class="mw-page-title-main">Anaplastic astrocytoma</span> Medical condition

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

Pediatric ependymomas are similar in nature to the adult form of ependymoma in that they are thought to arise from radial glial cells lining the ventricular system. However, they differ from adult ependymomas in which genes and chromosomes are most often affected, the region of the brain they are most frequently found in, and the prognosis of the patients. Children with certain hereditary diseases, such as neurofibromatosis type II (NF2), have been found to be more frequently afflicted with this class of tumors, but a firm genetic link remains to be established. Symptoms associated with the development of pediatric ependymomas are varied, much like symptoms for a number of other pediatric brain tumors including vomiting, headache, irritability, lethargy, and changes in gait. Although younger children and children with invasive tumor types generally experience less favorable outcomes, total removal of the tumors is the most conspicuous prognostic factor for both survival and relapse.

<span class="mw-page-title-main">Astroblastoma</span> Medical condition

Astroblastoma is a rare glial tumor derived from the astroblast, a type of cell that closely resembles spongioblastoma and astrocytes. Astroblastoma cells are most likely found in the supratentorial region of the brain that houses the cerebrum, an area responsible for all voluntary movements in the body. It also occurs significantly in the frontal lobe, parietal lobe, and temporal lobe, areas where movement, language creation, memory perception, and environmental surroundings are expressed. These tumors can be present in major brain areas not associated with the main cerebral hemispheres, including the cerebellum, optic nerve, cauda equina, hypothalamus, and brain stem.

<span class="mw-page-title-main">Palisade (pathology)</span> Microscopic view of single layer of cells

In histopathology, a palisade is a single layer of relatively long cells, arranged loosely perpendicular to a surface and parallel to each other. A rosette is a palisade in a halo or spoke-and-wheel arrangement, surrounding a central core or hub. A pseudorosette is a perivascular radial arrangement of neoplastic cells around a small blood vessel.

<span class="mw-page-title-main">Angiocentric glioma</span>

Angiocentric glioma (AG) refers to a rare neuroepithelial tumor when the superficial brain malignant cells enclose the brain vessels, commonly found in children and young adults. Initially identified in 2005 by Wang and his team from the University of Texas, AG was classified as Grade I by 2007 WHO Classification of Tumors of the Central Nervous System due to its benign clinical behavior, low proliferation index, and curative properties. AG primarily affects children and young adults at an average initial diagnosis age of 16 years old. Over 85% AG patients experience intractable seizures since childhood, especially partial epilepsy.

References

  1. "Ganglioglioma". St. Jude Children's Research Hospital . Retrieved March 8, 2023.
  2. "Ganglioglioma". St. Jude Children's Research Hospital . Retrieved March 9, 2023.
  3. Oppenheimer DC, Johnson MD, Judkins AR (2015). "Ganglioglioma of the Spinal Cord". Journal of Clinical Imaging Science. 5: 53. doi: 10.4103/2156-7514.166355 . PMC   4629305 . PMID   26605127.
  4. "Ganglioglioma | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 14 June 2019.[ permanent dead link ]
  5. Lang FF, Epstein FJ, Ransohoff J, Allen JC, Wisoff J, Abbott IR, Miller DC (December 1993). "Central nervous system gangliogliomas. Part 2: Clinical outcome". Journal of Neurosurgery. 79 (6): 867–873. doi:10.3171/jns.1993.79.6.0867. PMID   8246055.