Pineoblastoma: Difference between revisions
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{{CMG}}{{AE}}{{SR}} {{ADG}} | |||
{{CMG}}{{AE}}{{SR}} | {{SI}} | ||
{{SK}} Pineoblastomas; Pinealoblastoma; Pinealoblastomas; PB; Pineal parenchymal tumor | |||
{{SK}} Pineoblastomas; Pinealoblastoma; Pinealoblastomas; PB; Pineal parenchymal | |||
==Overview== | ==Overview== | ||
Pineoblastoma is a rare, [[malignant]] pineal parenchymal tumor. It is a [[supratentorial]] midline [[primitive neuroectodermal tumor]]. It is considered as a ''WHO grade IV tumor'' according to the WHO classification of tumors of the central nervous system. | Pineoblastoma is a rare, [[malignant]] pineal parenchymal tumor. It is a [[supratentorial]] midline [[primitive neuroectodermal tumor]]. It is considered as a ''WHO grade IV tumor'' according to the WHO classification of tumors of the [[central nervous system]]. | ||
==Pathophysiology== | ==Pathophysiology== | ||
===Pathogenesis=== | ===Pathogenesis=== | ||
Pineoblastoma originates from the neuroectodermal cells. It is the least differentiated pineal gland tumors, with [[pineocytoma]] and [[pineal parenchymal tumour with intermediate differentiation]] representing better differentiated tumors along the same spectrum.<ref name=pathopb1>Pathology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | Pineoblastoma originates from the [[Neuroectoderm|neuroectodermal cells]]. It is the least differentiated [[pineal gland]] [[tumors]], with [[pineocytoma]] and [[pineal parenchymal tumour with intermediate differentiation]] representing better differentiated [[tumors]] along the same spectrum.<ref name="pathopb1">Pathology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | ||
===Associated Conditions=== | ===Associated Conditions=== | ||
Pineoblastoma may occur in patients with hereditary uni- or bilateral [[retinoblastoma]]. When retinoblastoma patients present with pineoblastoma, this is characterized as "trilateral retinoblastoma".<ref name= | *Pineoblastoma may occur in patients with [[hereditary]] uni- or bilateral [[retinoblastoma]]. | ||
*When [[retinoblastoma]] patients present with pineoblastoma, this is characterized as "trilateral [[retinoblastoma]]".<ref name="pmid22802019">{{cite journal |vauthors=Rodjan F, de Graaf P, Brisse HJ, Göricke S, Maeder P, Galluzzi P, Aerts I, Alapetite C, Desjardins L, Wieland R, Popovic MB, Diezi M, Munier FL, Hadjistilianou T, Knol DL, Moll AC, Castelijns JA |title=Trilateral retinoblastoma: neuroimaging characteristics and value of routine brain screening on admission |journal=J. Neurooncol. |volume=109 |issue=3 |pages=535–44 |date=September 2012 |pmid=22802019 |pmc=3434888 |doi=10.1007/s11060-012-0922-4 |url=}}</ref> | |||
===Gross Pathology=== | ===Gross Pathology=== | ||
On gross pathology, pineoblastoma is characterized by solid, large poorly defined masses.<ref name=pathopb2>Radiographic features of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | On [[gross pathology]], pineoblastoma is characterized by solid, large poorly defined masses.<ref name="pathopb2">Radiographic features of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | ||
[[File:Gross pathology of pineoblastoma.jpg|center|thumb|<sub>An autopsy specimen showing a rather large pineal tumor. It was a pineoblastoma composed of highly cellular sheets of anaplastic cells with irregular hyperchromatic nuclei and brisk mitotic activity – resembling medulloblastoma and retinoblastoma.<ref name="grossimage1">Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file [http://radiopaedia.org/cases/pineoblastoma-gross-pathology here]). Creative Commons BY-SA-NC</ref></sub>]] | |||
===Microscopic Pathology=== | ===Microscopic Pathology=== | ||
On microscopic histopathological analysis, pineoblastoma is characterized by:<ref name=pathopb1>Pathology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref><ref name=micropathpb1>Microscopic features of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015</ref><ref name=micropb2>Histology of pineoblastoma. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-3. Accessed on December 1, 2015</ref> | On microscopic [[Histopathological|histopathological analysis]], pineoblastoma is characterized by:<ref name="pathopb1">Pathology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref><ref name="micropathpb1">Microscopic features of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015</ref><ref name="micropb2">Histology of pineoblastoma. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-3. Accessed on December 1, 2015</ref> | ||
*Hypercellular | *Hypercellular appearance | ||
*Tightly packed small round blue cells (high nuclear to cytoplasmic ratio) | *Tightly packed small round blue cells (high [[nuclear]] to [[cytoplasmic]] ratio) | ||
*Oval and angulated hyperchromatic nuclei with [[atypia]] | *Oval and angulated hyperchromatic [[nuclei]] with [[atypia]] | ||
*[[Mitoses]] | *[[Mitoses]] | ||
*Homer- | *Homer-Wright & Flexner-Winterstein rosettes | ||
*Fleurettes | *Fleurettes | ||
===Immunohistochemistry=== | ===Immunohistochemistry=== | ||
Pineoblastoma is demonstrated by positivity to [[tumor markers]] such as:<ref name=micropb2>Histology of pineoblastoma. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-3. Accessed on December 1, 2015</ref><ref name=ihcpb1>IHC of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015</ref> | Pineoblastoma is demonstrated by positivity to [[tumor markers]] such as:<ref name="micropb2">Histology of pineoblastoma. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-3. Accessed on December 1, 2015</ref><ref name="ihcpb1">IHC of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015</ref> | ||
*[[GFAP]] | *[[GFAP]] | ||
*[[Neurofilament]] | *[[Neurofilament]] | ||
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==Differentiating Pineoblastoma from other Diseases== | ==Differentiating Pineoblastoma from other Diseases== | ||
Pineoblastoma must be differentiated from:<ref name=differeialpb1nt>Differential diagnoses of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref><ref name=ddxpb1>DDx of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015</ref> | Pineoblastoma must be differentiated from:<ref name="differeialpb1nt">Differential diagnoses of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref><ref name="ddxpb1">DDx of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015</ref> | ||
*[[Pineocytoma]] | *[[Pineocytoma]] | ||
*[[Pineal parenchymal tumor with intermediate differentiation]] | *[[Pineal parenchymal tumor with intermediate differentiation]] | ||
* | *Papillary tumor of the pineal region | ||
*Pineal germinoma | *[[Pineal germinoma]] | ||
*Pineal embryonal carcinoma | *[[Pineal embryonal carcinoma]] | ||
*Pineal choriocarcinoma | *[[Pineal choriocarcinoma]] | ||
*Pineal yolk sac carcinoma (endodermal sinus tumor) | *[[Pineal yolk sac carcinoma]] ([[endodermal sinus tumor]]) | ||
*Pineal teratoma | *[[Pineal teratoma]] | ||
*[[Pineal gland cyst|Pineal cyst]] | *[[Pineal gland cyst|Pineal cyst]] | ||
*[[Astrocytoma | *[[Astrocytoma|Astrocytoma of the pineal gland]] | ||
*[[Meningioma | *[[Meningioma|Meningioma near pineal gland]] | ||
*[[Intracerebral metastases|Pineal metastasis]] | *[[Intracerebral metastases|Pineal metastasis]] | ||
*[[Cavernoma | *[[Cavernoma|Cavernoma in pineal region]] | ||
*[[Aneurysm | *[[Aneurysm|Aneurysm in pineal region]] | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
===Prevalence=== | ===Prevalence=== | ||
Pineoblastoma constitutes approximately 0.1% of the intracranial neoplasms.<ref name="pmid25210636">{{cite journal| author=Palled S, Kalavagunta S, Beerappa Gowda J, Umesh K, Aal M, Abdul Razack TP et al.| title=Tackling a recurrent pinealoblastoma. | journal=Case Rep Oncol Med | year= 2014 | volume= 2014 | issue= | pages= 135435 | pmid=25210636 | doi=10.1155/2014/135435 | pmc=PMC4158562 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25210636 }} </ref> | * Pineoblastoma constitutes approximately 0.1% of the [[Intracranial cavity|intracranial]] [[neoplasms]].<ref name="pmid25210636">{{cite journal| author=Palled S, Kalavagunta S, Beerappa Gowda J, Umesh K, Aal M, Abdul Razack TP et al.| title=Tackling a recurrent pinealoblastoma. | journal=Case Rep Oncol Med | year= 2014 | volume= 2014 | issue= | pages= 135435 | pmid=25210636 | doi=10.1155/2014/135435 | pmc=PMC4158562 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25210636 }} </ref> | ||
* Pineoblastoma together with [[germ cell tumors]] are the most common [[Pineal gland|pineal]] [[tumors]] in children.<ref name="Alexiou2012">{{cite journal|last1=Alexiou|first1=George A|title=Management of pineal region tumours in children|journal=Journal of Solid Tumors|volume=2|issue=2|year=2012|issn=1925-4075|doi=10.5430/jst.v2n2p15}}</ref> | |||
===Age=== | ===Age=== | ||
Pineoblastoma is a disease that tends to affect | Pineoblastoma is a disease that tends to affect children and young adults.<ref name="intropineoblastoma1">General features of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015</ref> | ||
===Gender=== | ===Gender=== | ||
Pineoblastoma affects men and women equally.<ref name=epidemiopb1>Epidemiology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | Pineoblastoma affects men and women equally.<ref name="epidemiopb1">Epidemiology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | ||
==Natural History, Complications and Prognosis== | ==Natural History, Complications and Prognosis== | ||
===Natural History=== | ===Natural History=== | ||
Pineoblastoma is the most agressive pineal parenchymal tumor. If left untreated, patients with pineoblastoma may progress to develop [[seizures]], [[obstructive hydrocephalus]], local recurrence, and CSF metastasis.<ref name=naturalpb1>Clinical presentation of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | * Pineoblastoma is the most agressive [[Pineal parenchymal tumor with intermediate differentiation|pineal parenchymal tumor]]. | ||
* If left untreated, patients with pineoblastoma may progress to develop [[seizures]], [[obstructive hydrocephalus]], local recurrence, and [[cerebrospinal fluid]] ([[CSF]]) [[metastasis]].<ref name="naturalpb1">Clinical presentation of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | |||
===Complications=== | ===Complications=== | ||
Common complications of pineoblastoma include:<ref name=naturalpb1>Clinical presentation of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref><ref name="pmid21184689">{{cite journal| author=Stoiber EM, Schaible B, Herfarth K, Schulz-Ertner D, Huber PE, Debus J et al.| title=Long term outcome of adolescent and adult patients with pineal parenchymal tumors treated with fractionated radiotherapy between 1982 and 2003--a single institution's experience. | journal=Radiat Oncol | year= 2010 | volume= 5 | issue= | pages= 122 | pmid=21184689 | doi=10.1186/1748-717X-5-122 | pmc=PMC3019157 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21184689 }} </ref> | Common complications of pineoblastoma include:<ref name="naturalpb1">Clinical presentation of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref><ref name="pmid21184689">{{cite journal| author=Stoiber EM, Schaible B, Herfarth K, Schulz-Ertner D, Huber PE, Debus J et al.| title=Long term outcome of adolescent and adult patients with pineal parenchymal tumors treated with fractionated radiotherapy between 1982 and 2003--a single institution's experience. | journal=Radiat Oncol | year= 2010 | volume= 5 | issue= | pages= 122 | pmid=21184689 | doi=10.1186/1748-717X-5-122 | pmc=PMC3019157 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21184689 }} </ref> | ||
*[[Obstructive hydrocephalus]] | *[[Obstructive hydrocephalus]] | ||
*Local recurrence | *Local recurrence | ||
*[[CSF]] metastasis | *[[CSF]] [[metastasis]] | ||
===Prognosis=== | ===Prognosis=== | ||
Prognosis is generally poor, and the 5-year survival rate of patients with pineoblastoma is approximately 58%.<ref name=prognosispb1>Treatment and prognosis of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | [[Prognosis]] is generally poor, and the [[Five year survival rate|5-year survival rate]] of patients with pineoblastoma is approximately 58%.<ref name="prognosispb1">Treatment and prognosis of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | ||
==History and Symptoms== | ==History and Symptoms== | ||
===History=== | ===History=== | ||
The clinical presentation of pineoblastoma is mainly from the [[obstructive hydrocephalus]] secondary to compression of the [[tectum]] of the [[midbrain]] and obstruction of the [[Cerebral aqueduct|aqueduct]].<ref name="naturalpb1" /> | |||
===Symptoms=== | ===Symptoms=== | ||
* | *[[Symptoms]] of pineoblastoma include:<ref name="pmid25210636">{{cite journal| author=Palled S, Kalavagunta S, Beerappa Gowda J, Umesh K, Aal M, Abdul Razack TP et al.| title=Tackling a recurrent pinealoblastoma. | journal=Case Rep Oncol Med | year= 2014 | volume= 2014 | issue= | pages= 135435 | pmid=25210636 | doi=10.1155/2014/135435 | pmc=PMC4158562 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25210636 }} </ref><ref name="naturalpb1">Clinical presentation of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | ||
:*[[Headache]]s | :*[[Headache]]s | ||
:*[[Nausea]] | :*[[Nausea]] | ||
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==Physical Examination== | ==Physical Examination== | ||
Compression of the superior colliculi can lead to a characteristic gaze palsy, known as [[Parinaud syndrome]]. Common physical examination findings of pineoblastoma include:<ref name="pmid25210636">{{cite journal| author=Palled S, Kalavagunta S, Beerappa Gowda J, Umesh K, Aal M, Abdul Razack TP et al.| title=Tackling a recurrent pinealoblastoma. | journal=Case Rep Oncol Med | year= 2014 | volume= 2014 | issue= | pages= 135435 | pmid=25210636 | doi=10.1155/2014/135435 | pmc=PMC4158562 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25210636 }} </ref><ref name=naturalpb1>Clinical presentation of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | * Compression of the superior colliculi can lead to a characteristic upward gaze palsy, known as [[Parinaud syndrome]]. | ||
* Common physical examination findings of pineoblastoma include:<ref name="pmid25210636">{{cite journal| author=Palled S, Kalavagunta S, Beerappa Gowda J, Umesh K, Aal M, Abdul Razack TP et al.| title=Tackling a recurrent pinealoblastoma. | journal=Case Rep Oncol Med | year= 2014 | volume= 2014 | issue= | pages= 135435 | pmid=25210636 | doi=10.1155/2014/135435 | pmc=PMC4158562 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25210636 }} </ref><ref name="naturalpb1">Clinical presentation of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | |||
===HEENT=== | ===HEENT=== | ||
*Bulging soft spots ([[fontanelle]]s) | *Bulging soft spots ([[fontanelle]]s) | ||
*Eyes that are constantly looking down ([[sunsetting sign]]) | *Eyes that are constantly looking down ([[sunsetting sign]]) | ||
* | *Upward [[gaze palsy]] | ||
*Pupillary light-near dissociation (pupils respond to near stimuli but not light) | *[[Light-near dissociation|Pupillary light-near dissociation]] ([[pupils]] respond to near stimuli but not light) | ||
*Convergence-retraction [[nystagmus]] | *Convergence-retraction [[nystagmus]] | ||
*[[Papilledema]] | *[[Papilledema]] | ||
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*[[Nerve palsy|Cranioneuropathies]] | *[[Nerve palsy|Cranioneuropathies]] | ||
:*[[abducens nerve palsy|VI<sup>th</sup> nerve palsy]] | :*[[abducens nerve palsy|VI<sup>th</sup> nerve palsy]] | ||
===Laboratory Diagnosis=== | |||
There are no specific laboratory findings for pineocytoma. However, the following findings are of significant | |||
*Both [[serum]] and [[CSF]] should be assayed for [[alpha-fetoprotein]] and [[Human chorionic gonadotropin|beta human chorionic gonadotropin]] ([[beta-hCG]]) to help diagnose a [[germ cell tumor]]. | |||
*[[Immunohistochemistry]] may be of value in detecting these markers or [[placental]] [[alkaline phosphatase]]. | |||
===CT Scan=== | |||
*Head [[Computed tomography|CT scan]] may be diagnostic of pineoblastoma. | |||
*Findings on [[Computed tomography|CT scan]] suggestive of pineoblastoma include a mass with a solid component that tends to be slightly hyperdense compared to adjacent brain due to high cellularity. [[Calcification]] is present that is peripherally disperse or "exploded", similar to [[pineocytoma]].<ref name="CTpb1">CT findings of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | |||
== | ===MRI=== | ||
* | *[[Brain]] [[Magnetic resonance imaging|MRI]] may be diagnostic of pineoblastoma. | ||
* | *Features on [[Magnetic resonance imaging|MRI]] suggestive of pineoblastoma include:<ref name="MRIscanpb1">Radiographic features MRI of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | ||
{| style="border: 0px; font-size: 90%; margin: 3px; width:1000px align=center" | |||
| valign="top" | | |||
{| style="border: 0px; font-size: 90%; margin: 3px; width:1000px" | |||
|valign=top| | |||
|+ | |+ | ||
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|MRI component}} | ! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|MRI component}} | ||
! style="background: #4479BA; width: 700px;" | {{fontcolor|#FFF|Findings}} | ! style="background: #4479BA; width: 700px;" | {{fontcolor|#FFF|Findings}} | ||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | | | style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" | | ||
T1 | T1 | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
*Isointense to hypointense to adjacent brain | *Isointense to hypointense to adjacent [[brain]] | ||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align=center| | | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align="center" | | ||
T2 | T2 | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
*Isointense to adjacent brain | *Isointense to adjacent [[brain]] | ||
*Areas of [[cyst]] formation or [[necrosis]] may be present | *Areas of [[cyst]] formation or [[necrosis]] may be present | ||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align=center| | | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align="center" | | ||
T1 with gadolinium contrast | T1 with gadolinium contrast [T1 C+ (Gd)] | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
*Vivid heterogenous enhancement | *Vivid heterogenous enhancement | ||
|- | |- | ||
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align=center| | | style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align="center" | | ||
Diffuse weighted imaging/Apparent diffusion coefficient | Diffuse weighted imaging/Apparent diffusion coefficient [DWI/ADC] | ||
| style="padding: 5px 5px; background: #F5F5F5;" | | | style="padding: 5px 5px; background: #F5F5F5;" | | ||
*Restricted diffusion due to dense cellular packing | *Restricted diffusion due to dense cellular packing | ||
*ADC values are typically 400-800 mm2/s | *ADC values are typically 400-800 mm2/s | ||
|} | |} | ||
===Other Imaging Findings=== | |||
Other imaging studies for pineoblastoma include [[Nuclear magnetic resonance spectroscopy|magnetic resonance spectroscopy]] (MR [[spectroscopy]]), which demonstrates:<ref name="otherimagefindingsmrspectrocscopypineoblastoma1">MR spectroscopy of pineoblastoma. Dr Mohammad A. ElBeialy. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-6. Accessed on December 1, 2015</ref> | |||
*Elevation of the [[choline]] and lipid [[lactate]] peaks | |||
*Depression of the neural markers ([[N-Acetylaspartate|N-acetyl aspartate (NAA)]] and [[creatine|creatine (Cr)]] | |||
*Prominent [[glutamate]] and [[taurine]] peaks at 3.4 ppm with shot TE signal voxel MR [[spectroscopy]] | |||
=== Other Diagnostic Studies === | |||
'''Stereotactic biopsy''' | |||
*A direct, visually guided [[biopsy]] of the [[pineal gland]] mass with open or neuroendoscopic [[surgery]] has been preferred due to concerns about injury to the [[Cerebral veins|deep cerebral veins]]. | |||
*An open procedure also allows [[CSF]] to be obtained for | |||
**[[Tumor marker]] studies | |||
**Permits direct visualization of the third ventricle for staging purposes | |||
**Sllows a third [[ventriculostomy]] to be performed for [[CSF]] diversion if needed. | |||
*The diagnostic yield of [[stereotactic]] [[biopsy]] ranges from 94 to 100 percent. | |||
*If the [[biopsy]] is nondiagnostic, equivocal, or suggests a [[benign tumor]] such as mature [[teratoma]] or [[meningioma]], [[surgery]] is recommended to establish a definitive [[diagnosis]] or to identify focal areas of [[malignant]] disease | |||
==Treatment== | ==Treatment== | ||
The predominant therapy for pineoblastoma is [[surgical resection]]. Adjunctive [[chemotherapy]] and [[radiation]] may be required.<ref name=prognosispb1>Treatment and prognosis of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | *The predominant therapy for pineoblastoma is [[surgical resection]]. Adjunctive [[chemotherapy]] and [[radiation]] may be required.<ref name="Alexiou2012">{{cite journal|last1=Alexiou|first1=George A|title=Management of pineal region tumours in children|journal=Journal of Solid Tumors|volume=2|issue=2|year=2012|issn=1925-4075|doi=10.5430/jst.v2n2p15}}</ref><ref name="prognosispb1">Treatment and prognosis of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015</ref> | ||
*The main goal of open [[surgery]] on pineoblastoma is the complete [[tumor]] removal with minimal [[morbidity]], whenever possible. However, even if gross total [[excision]] cannot be achieved, establishment of an accurate [[diagnosis]], maximal cytoreduction, and restoration of the [[CSF]] pathway may be achieved. | |||
{| class="wikitable" | |||
|+ | |||
! colspan="2" |Management Options of Penial Gland tumors | |||
|- | |||
|'''CSF diversion''' | |||
| | |||
* The optimal surgical strategy to treat acute [[hydrocephalus]] in patients with [[Pineal gland tumor|pineal tumors]] is uncertain. | |||
*[[CSF]] diversion ([[ventriculoperitoneal shunt]] (VP shunt) or [[Ventriculostomy|third ventriculostomy]] may be necessary in [[symptomatic]] patients, although debulking surgery may obviate the need for this procedure | |||
* When [[CSF]] diversion is necessary, [[endoscopic]] [[Third ventricle|third]] [[ventriculostomy]] can be carried out at the same time as the [[biopsy]] and is preferred over [[Ventriculoperitoneal shunt|VP shunts]], which can be complicated by [[infection]], shunt malfunction, [[subdural hematoma]], and rarely, [[tumor]] seeding | |||
|- | |||
|'''Surgical resection''' | |||
| | |||
* Some series report long-term survival with surgery alone, even in patients with pineoblastomas. | |||
* Indeed, for pineoblastomas, gross total [[surgical resection]] appears to correlate with improved [[Survival rate|survival]]. | |||
* Patients with [[symptomatic]] recurrent [[pineocytomas]] should also be considered for [[surgical resection]] of the lesion | |||
|- | |||
|'''Radiation''' | |||
| | |||
* Postoperative [[Radiotherapy|adjuvant radiotherapy]] (RT) is frequently (but not universally) recommended, and local control is dose-dependent. | |||
* The [[incidence]] of [[leptomeningeal]] recurrence is low. | |||
* The [[Five year survival rate|five-year survival rates]] were 86 and 49 percent for pineocytomas and non-pineocytoma PPTs, respectively. | |||
* Adjuvant RT is not universally recommended after gross total [[resection]] of a pineocytoma | |||
|- | |||
|'''Stereotactic radiosurgery''' | |||
| | |||
*[[Stereotactic radiosurgery]] (SRS) is emerging as a useful treatment alternative for pineocytomas, although experience is limited. | |||
* The precise [[Radiation therapy|radiation]] fields that are defined by [[MRI]] or [[CT-scans|CT]]-computerized treatment planning minimize damage to the surrounding [[brain]], and the risks of [[general anesthesia]] and [[craniotomy]] are avoided. | |||
* SRS is increasingly being used to treat [[Pineal gland|pineal]] region [[tumors]], either as an additional therapy after conventional treatments or as a primary treatment. | |||
* Due to the low rate of side effects, SRS may develop into an attractive alternative to [[microsurgery]] in newly diagnosed pineocytomas. In [[malignant]] PPTs, IRS may be routinely applied in a multimodality treatment schedule supplementary to conventional [[irradiation]]. | |||
|- | |||
|'''Chemotherapy as part of multimodality therapy''' | |||
| | |||
* The similarity of pineoblastomas to [[medulloblastomas]] in terms of their clinical behavior and tendency for [[leptomeningeal]] seeding has led to the use of similar [[chemotherapy]] regimens in patients with pineoblastoma as part of a multimodality approach. | |||
*[[Chemotherapy]] has been used to delay [[radiation therapy]] in very young children, for whom the long-term [[neurocognitive]] and developmental side effects of craniospinal [[irradiation]] (CSI) are a major concern. | |||
* The importance of [[radiation therapy]] as a component of the initial treatment of [[supratentorial]] [[primitive neuroectodermal tumors]] ([[Primitive neuroectodermal tumor|PNETs]]) is also supported by the German HIT-SKK87 and HIT-SKK92 protocols, as well as the Canadian pediatric brain tumor protocol | |||
|} | |||
*Gross total resection has been associated with improved [[Survival rate|survival]], similar to treatment with craniospinal [[irradiation]] and multi-agent [[chemotherapy]].<ref name="Alexiou2012">{{cite journal|last1=Alexiou|first1=George A|title=Management of pineal region tumours in children|journal=Journal of Solid Tumors|volume=2|issue=2|year=2012|issn=1925-4075|doi=10.5430/jst.v2n2p15}}</ref> | |||
*Children under the age of 36 months with pineoblastoma should be treated with multi-agent [[chemotherapy]] for 12 to 24 months with the goal of delaying [[Radiation therapy|radiation]] past the age of 36 months. Craniospinal irradiation before this age of 3 has been associated with significant [[cognitive]] and [[neuroendocrine]] sequelae. | |||
*Subtotal [[tumor]] [[resection]] in children under 5 years of age is associated with markedly worsened patient survival. According to the Children's Oncology Group trials, these tumors require craniospinal irradiation (with local tumor doses of at least 50 Gy) and adjuvant [[chemotherapy]]. When [[carboplatin]] and [[vincristine]] were administered during craniospinal irradiation followed by 6 months of non-intensive non-[[cisplatin]] chemotherapy, it significantly reduces [[tumor]] progression. | |||
*Patients with pineoblastoma will develop [[hydrocephalus]] in majority of the cases and they will require [[CSF]] diversion. [[Ventriculoperitoneal shunt|Ventriculo-peritoneal (V-P) shunt]] placement is associated with low [[morbidity]] and [[mortality rate]]. However, shunt malfunction in this population is as high as 20%. In addition, [[tumor]] [[metastasis]] through a [[Cerebral shunt|CSF shunt]] has been reported. Endoscopic [[Third ventricle|third]] [[ventriculostomy]] (ETVC) is an alternative option, which also permits a [[biopsy]] of the tumor in the same procedure. Ahn et al. reported that the biopsy samples, obtained in the [[Lateral ventricles|lateral ventricle]] or [[Pineal gland|pineal region]], were more favorable towards a successful diagnosis than those in the [[thalamus]] or [[Midbrain tectum|tectal region]]. Neuroendoscopic [[biopsy]] procedures have been proven safe with low [[Complication (medicine)|complication]] rates.<ref name="Alexiou2012">{{cite journal|last1=Alexiou|first1=George A|title=Management of pineal region tumours in children|journal=Journal of Solid Tumors|volume=2|issue=2|year=2012|issn=1925-4075|doi=10.5430/jst.v2n2p15}}</ref> | |||
==References== | ==References== | ||
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Latest revision as of 06:49, 6 July 2020
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2] Aditya Ganti M.B.B.S. [3]
WikiDoc Resources for Pineoblastoma |
Articles |
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Media |
Powerpoint slides on Pineoblastoma |
Evidence Based Medicine |
Clinical Trials |
Ongoing Trials on Pineoblastoma at Clinical Trials.gov Trial results on Pineoblastoma Clinical Trials on Pineoblastoma at Google
|
Guidelines / Policies / Govt |
US National Guidelines Clearinghouse on Pineoblastoma NICE Guidance on Pineoblastoma
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Definitions |
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Patient resources on Pineoblastoma Discussion groups on Pineoblastoma Patient Handouts on Pineoblastoma Directions to Hospitals Treating Pineoblastoma Risk calculators and risk factors for Pineoblastoma
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Synonyms and keywords: Pineoblastomas; Pinealoblastoma; Pinealoblastomas; PB; Pineal parenchymal tumor
Overview
Pineoblastoma is a rare, malignant pineal parenchymal tumor. It is a supratentorial midline primitive neuroectodermal tumor. It is considered as a WHO grade IV tumor according to the WHO classification of tumors of the central nervous system.
Pathophysiology
Pathogenesis
Pineoblastoma originates from the neuroectodermal cells. It is the least differentiated pineal gland tumors, with pineocytoma and pineal parenchymal tumour with intermediate differentiation representing better differentiated tumors along the same spectrum.[1]
Associated Conditions
- Pineoblastoma may occur in patients with hereditary uni- or bilateral retinoblastoma.
- When retinoblastoma patients present with pineoblastoma, this is characterized as "trilateral retinoblastoma".[2]
Gross Pathology
On gross pathology, pineoblastoma is characterized by solid, large poorly defined masses.[3]

Microscopic Pathology
On microscopic histopathological analysis, pineoblastoma is characterized by:[1][5][6]
- Hypercellular appearance
- Tightly packed small round blue cells (high nuclear to cytoplasmic ratio)
- Oval and angulated hyperchromatic nuclei with atypia
- Mitoses
- Homer-Wright & Flexner-Winterstein rosettes
- Fleurettes
Immunohistochemistry
Pineoblastoma is demonstrated by positivity to tumor markers such as:[6][7]
Differentiating Pineoblastoma from other Diseases
Pineoblastoma must be differentiated from:[8][9]
- Pineocytoma
- Pineal parenchymal tumor with intermediate differentiation
- Papillary tumor of the pineal region
- Pineal germinoma
- Pineal embryonal carcinoma
- Pineal choriocarcinoma
- Pineal yolk sac carcinoma (endodermal sinus tumor)
- Pineal teratoma
- Pineal cyst
- Astrocytoma of the pineal gland
- Meningioma near pineal gland
- Pineal metastasis
- Cavernoma in pineal region
- Aneurysm in pineal region
Epidemiology and Demographics
Prevalence
- Pineoblastoma constitutes approximately 0.1% of the intracranial neoplasms.[10]
- Pineoblastoma together with germ cell tumors are the most common pineal tumors in children.[11]
Age
Pineoblastoma is a disease that tends to affect children and young adults.[12]
Gender
Pineoblastoma affects men and women equally.[13]
Natural History, Complications and Prognosis
Natural History
- Pineoblastoma is the most agressive pineal parenchymal tumor.
- If left untreated, patients with pineoblastoma may progress to develop seizures, obstructive hydrocephalus, local recurrence, and cerebrospinal fluid (CSF) metastasis.[14]
Complications
Common complications of pineoblastoma include:[14][15]
- Obstructive hydrocephalus
- Local recurrence
- CSF metastasis
Prognosis
Prognosis is generally poor, and the 5-year survival rate of patients with pineoblastoma is approximately 58%.[16]
History and Symptoms
History
The clinical presentation of pineoblastoma is mainly from the obstructive hydrocephalus secondary to compression of the tectum of the midbrain and obstruction of the aqueduct.[14]
Symptoms
Physical Examination
- Compression of the superior colliculi can lead to a characteristic upward gaze palsy, known as Parinaud syndrome.
- Common physical examination findings of pineoblastoma include:[10][14]
HEENT
- Bulging soft spots (fontanelles)
- Eyes that are constantly looking down (sunsetting sign)
- Upward gaze palsy
- Pupillary light-near dissociation (pupils respond to near stimuli but not light)
- Convergence-retraction nystagmus
- Papilledema
Neurological
Laboratory Diagnosis
There are no specific laboratory findings for pineocytoma. However, the following findings are of significant
- Both serum and CSF should be assayed for alpha-fetoprotein and beta human chorionic gonadotropin (beta-hCG) to help diagnose a germ cell tumor.
- Immunohistochemistry may be of value in detecting these markers or placental alkaline phosphatase.
CT Scan
- Head CT scan may be diagnostic of pineoblastoma.
- Findings on CT scan suggestive of pineoblastoma include a mass with a solid component that tends to be slightly hyperdense compared to adjacent brain due to high cellularity. Calcification is present that is peripherally disperse or "exploded", similar to pineocytoma.[17]
MRI
- Brain MRI may be diagnostic of pineoblastoma.
- Features on MRI suggestive of pineoblastoma include:[18]
MRI component | Findings |
---|---|
T1 |
|
T2 |
|
T1 with gadolinium contrast [T1 C+ (Gd)] |
|
Diffuse weighted imaging/Apparent diffusion coefficient [DWI/ADC] |
|
Other Imaging Findings
Other imaging studies for pineoblastoma include magnetic resonance spectroscopy (MR spectroscopy), which demonstrates:[19]
- Elevation of the choline and lipid lactate peaks
- Depression of the neural markers (N-acetyl aspartate (NAA) and creatine (Cr)
- Prominent glutamate and taurine peaks at 3.4 ppm with shot TE signal voxel MR spectroscopy
Other Diagnostic Studies
Stereotactic biopsy
- A direct, visually guided biopsy of the pineal gland mass with open or neuroendoscopic surgery has been preferred due to concerns about injury to the deep cerebral veins.
- An open procedure also allows CSF to be obtained for
- Tumor marker studies
- Permits direct visualization of the third ventricle for staging purposes
- Sllows a third ventriculostomy to be performed for CSF diversion if needed.
- The diagnostic yield of stereotactic biopsy ranges from 94 to 100 percent.
- If the biopsy is nondiagnostic, equivocal, or suggests a benign tumor such as mature teratoma or meningioma, surgery is recommended to establish a definitive diagnosis or to identify focal areas of malignant disease
Treatment
- The predominant therapy for pineoblastoma is surgical resection. Adjunctive chemotherapy and radiation may be required.[11][16]
- The main goal of open surgery on pineoblastoma is the complete tumor removal with minimal morbidity, whenever possible. However, even if gross total excision cannot be achieved, establishment of an accurate diagnosis, maximal cytoreduction, and restoration of the CSF pathway may be achieved.
Management Options of Penial Gland tumors | |
---|---|
CSF diversion |
|
Surgical resection |
|
Radiation |
|
Stereotactic radiosurgery |
|
Chemotherapy as part of multimodality therapy |
|
- Gross total resection has been associated with improved survival, similar to treatment with craniospinal irradiation and multi-agent chemotherapy.[11]
- Children under the age of 36 months with pineoblastoma should be treated with multi-agent chemotherapy for 12 to 24 months with the goal of delaying radiation past the age of 36 months. Craniospinal irradiation before this age of 3 has been associated with significant cognitive and neuroendocrine sequelae.
- Subtotal tumor resection in children under 5 years of age is associated with markedly worsened patient survival. According to the Children's Oncology Group trials, these tumors require craniospinal irradiation (with local tumor doses of at least 50 Gy) and adjuvant chemotherapy. When carboplatin and vincristine were administered during craniospinal irradiation followed by 6 months of non-intensive non-cisplatin chemotherapy, it significantly reduces tumor progression.
- Patients with pineoblastoma will develop hydrocephalus in majority of the cases and they will require CSF diversion. Ventriculo-peritoneal (V-P) shunt placement is associated with low morbidity and mortality rate. However, shunt malfunction in this population is as high as 20%. In addition, tumor metastasis through a CSF shunt has been reported. Endoscopic third ventriculostomy (ETVC) is an alternative option, which also permits a biopsy of the tumor in the same procedure. Ahn et al. reported that the biopsy samples, obtained in the lateral ventricle or pineal region, were more favorable towards a successful diagnosis than those in the thalamus or tectal region. Neuroendoscopic biopsy procedures have been proven safe with low complication rates.[11]
References
- ↑ 1.0 1.1 Pathology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
- ↑ Rodjan F, de Graaf P, Brisse HJ, Göricke S, Maeder P, Galluzzi P, Aerts I, Alapetite C, Desjardins L, Wieland R, Popovic MB, Diezi M, Munier FL, Hadjistilianou T, Knol DL, Moll AC, Castelijns JA (September 2012). "Trilateral retinoblastoma: neuroimaging characteristics and value of routine brain screening on admission". J. Neurooncol. 109 (3): 535–44. doi:10.1007/s11060-012-0922-4. PMC 3434888. PMID 22802019.
- ↑ Radiographic features of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
- ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
- ↑ Microscopic features of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
- ↑ 6.0 6.1 Histology of pineoblastoma. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-3. Accessed on December 1, 2015
- ↑ IHC of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
- ↑ Differential diagnoses of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
- ↑ DDx of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
- ↑ 10.0 10.1 10.2 Palled S, Kalavagunta S, Beerappa Gowda J, Umesh K, Aal M, Abdul Razack TP; et al. (2014). "Tackling a recurrent pinealoblastoma". Case Rep Oncol Med. 2014: 135435. doi:10.1155/2014/135435. PMC 4158562. PMID 25210636.
- ↑ 11.0 11.1 11.2 11.3 Alexiou, George A (2012). "Management of pineal region tumours in children". Journal of Solid Tumors. 2 (2). doi:10.5430/jst.v2n2p15. ISSN 1925-4075.
- ↑ General features of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
- ↑ Epidemiology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
- ↑ 14.0 14.1 14.2 14.3 14.4 Clinical presentation of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
- ↑ Stoiber EM, Schaible B, Herfarth K, Schulz-Ertner D, Huber PE, Debus J; et al. (2010). "Long term outcome of adolescent and adult patients with pineal parenchymal tumors treated with fractionated radiotherapy between 1982 and 2003--a single institution's experience". Radiat Oncol. 5: 122. doi:10.1186/1748-717X-5-122. PMC 3019157. PMID 21184689.
- ↑ 16.0 16.1 Treatment and prognosis of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
- ↑ CT findings of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
- ↑ Radiographic features MRI of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
- ↑ MR spectroscopy of pineoblastoma. Dr Mohammad A. ElBeialy. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-6. Accessed on December 1, 2015