Ganglioglioma: Difference between revisions
Ozzie10aaaa (talk | contribs) No edit summary |
→Histopathology: link |
||
| (17 intermediate revisions by 11 users not shown) | |||
| Line 1: | Line 1: | ||
{{More citations needed|date=May 2021}} |
|||
{{For|the tumor sometimes called a ganglioma|Ganglioneuroma}} |
|||
{{Infobox medical condition (new) |
{{Infobox medical condition (new) |
||
| name = Ganglioglioma |
| name = Ganglioglioma |
||
| synonyms = Gangliocytoma |
|||
| synonyms = Mixed cell tumors containing both neural ganglionic cells and neural glial cell components |
|||
| image = Dysplastic cerebellar gangliocytoma.jpg |
| image = Dysplastic cerebellar gangliocytoma.jpg |
||
| caption = Dysplastic |
| caption = Dysplastic cerebellar ganglioglioma ([[Lhermitte–Duclos disease]]) |
||
| pronounce = |
| pronounce = |
||
| field = |
| field = [[Neuro-oncology]] |
||
| symptoms = |
| symptoms = |
||
| complications = |
| complications = |
||
| onset = Usually childhood to young adulthood<ref name="StJude">{{cite web|title=Ganglioglioma|url=https://www.stjude.org/disease/ganglioglioma.html |publisher=[[St. Jude Children's Research Hospital]]|access-date=March 8, 2023}}</ref> |
|||
| onset = |
|||
| duration = |
| duration = |
||
| types = |
| types = |
||
| causes = |
| causes = |
||
| risks = |
| risks = |
||
| diagnosis = |
| diagnosis = |
||
| differential = |
| differential = |
||
| prevention = |
| prevention = |
||
| treatment = |
| treatment = Primarily surgery |
||
| medication = |
| medication = |
||
| prognosis = >90% [[five-year survival rate]] among children<ref name="StJudeTogether">{{cite web|title=Ganglioglioma|url=https://together.stjude.org/en-us/about-pediatric-cancer/types/brain-spinal-tumors/ganglioglioma.html|publisher=[[St. Jude Children's Research Hospital]]|access-date=March 9, 2023}}</ref> |
|||
| prognosis = |
|||
| frequency = |
| frequency = |
||
| deaths = |
| deaths = |
||
}} |
}} |
||
''' |
A '''ganglioglioma''' (or '''gangliocytoma''') is a rare, slow-growing primary [[central nervous system]] (CNS) tumor which most frequently occurs in the [[temporal lobe]]s of children and young adults.<ref name="pmid26605127">{{cite journal | vauthors = Oppenheimer DC, Johnson MD, Judkins AR | title = Ganglioglioma of the Spinal Cord | journal = Journal of Clinical Imaging Science | volume = 5 | issue = | pages = 53 | date = 2015 | pmid = 26605127 | pmc = 4629305 | doi = 10.4103/2156-7514.166355 | doi-access = free }}</ref> They are mixed cell tumors containing both neural [[ganglionic]] cells and neural [[glial cell]] components.<ref>{{cite web |title=Ganglioglioma {{!}} Genetic and Rare Diseases Information Center (GARD) – an NCATS Program |url=https://rarediseases.info.nih.gov/diseases/2430/index |website=rarediseases.info.nih.gov |access-date=14 June 2019 }}{{Dead link|date=April 2024 |bot=InternetArchiveBot |fix-attempted=yes }}</ref> |
||
==Classification== |
==Classification== |
||
Gangliogliomas are generally benign WHO grade I [[tumors]]; the presence of anaplastic changes in the [[glia |
Gangliogliomas are generally benign WHO grade I [[tumors]]; the presence of anaplastic changes in the [[glia]]l 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== |
==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 [[cluster of differentiation|abnormal clusters]], the presence of [[binucleated cells|binucleation]] and dysmorphic neurons are helpful clues favoring ganglioglioma over glioma. The glial component of ganglioglioma includes [[Astrocyte#Astrocytomas|fibrillary astrocytes]] with varying degrees of cellular [[atypia]]. The neoplastic neuronal components are often clustered or irregularly oriented. Fibrovascular [[stroma (tissue)|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 (protein)|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 [[mitosis|mitotic activity]], endothelial proliferation, and necrosis. |
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 [[cluster of differentiation|abnormal clusters]], the presence of [[binucleated cells|binucleation]] and dysmorphic neurons are helpful clues favoring ganglioglioma over glioma. The glial component of ganglioglioma includes [[Astrocyte#Astrocytomas|fibrillary astrocytes]] with varying degrees of cellular [[atypia]]. The neoplastic neuronal components are often clustered or irregularly oriented. Fibrovascular [[stroma (tissue)|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 (protein)|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 [[mitosis|mitotic activity]], endothelial proliferation, and necrosis. |
||
==Diagnosis== |
==Diagnosis== |
||
| Line 38: | Line 40: | ||
==Treatment== |
==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.<ref>{{ |
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.<ref>{{cite journal | vauthors = Lang FF, Epstein FJ, Ransohoff J, Allen JC, Wisoff J, Abbott IR, Miller DC | title = Central nervous system gangliogliomas. Part 2: Clinical outcome | journal = Journal of Neurosurgery | volume = 79 | issue = 6 | pages = 867–873 | date = December 1993 | pmid = 8246055 | doi = 10.3171/jns.1993.79.6.0867 }}</ref> 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. |
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== |
== See also == |
||
*[[Lhermitte-Duclos disease]] |
* [[Lhermitte-Duclos disease]] |
||
==References== |
== References == |
||
{{reflist}} |
{{reflist}} |
||
== External links == |
== External links == |
||
{{Medical resources |
{{Medical resources |
||
| DiseasesDB = |
| DiseasesDB = |
||
| ICD10 = |
| ICD10 = |
||
| ICD9 = |
| ICD9 = |
||
| ICDO = {{ICDO|9505|1}} |
| ICDO = {{ICDO|9505|1}} |
||
| OMIM = |
| OMIM = |
||
| MedlinePlus = |
| MedlinePlus = |
||
| eMedicineSubj = |
| eMedicineSubj = |
||
| eMedicineTopic = |
| eMedicineTopic = |
||
| MeshID = D018303 |
| MeshID = D018303 |
||
| Orphanet = 251949 |
| Orphanet = 251949 |
||
}} |
}} |
||
{{Central nervous system tumors}} |
{{Central nervous system tumors}} |
||
Latest revision as of 21:28, 11 June 2024
 | This article needs additional citations for verification. (May 2021) |
| Ganglioglioma | |
|---|---|
| Other names | Gangliocytoma |
 | |
| Dysplastic cerebellar ganglioglioma (Lhermitte–Duclos disease) | |
| Specialty | Neuro-oncology |
| Usual onset | Usually childhood to young adulthood[1] |
| Treatment | Primarily 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]
Classification
[edit]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
[edit]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
[edit]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
[edit]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
[edit]References
[edit]- ^ "Ganglioglioma". St. Jude Children's Research Hospital. Retrieved March 8, 2023.
- ^ "Ganglioglioma". St. Jude Children's Research Hospital. Retrieved March 9, 2023.
- ^ 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.
- ^ "Ganglioglioma | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 14 June 2019.[permanent dead link]
- ^ 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.