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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 tumor; Pineal gland tumor; Brain tumor


==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.<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><ref name=intropineoblastoma2>Pinealoblastoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pinealoblastoma. Accessed on December 1, 2015</ref>
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=intropineoblastoma2>Pinealoblastoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pinealoblastoma. Accessed on December 1, 2015</ref>
*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>]]
====Gallery====
<gallery>
Image:Gross pathology of pineoblastoma.jpg|<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>
 
</gallery>


===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-wright & Flexner-Winterstein rosettes
*Homer-Wright & Flexner-Winterstein rosettes
*Fleurettes
*Fleurettes
====Gallery====
<gallery>
Image:Microscopic image of pineoblastoma 1.jpg|<sub>Pathology specimen of a pineoblastoma (HE stain, x200 magnification).<ref name=microimage1>Microscopic images of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015</ref></sub>
Image:Microscopic image of pineoblastoma 2.jpg|<sub>Paraffin sections show fragments of a densely hypercellular tumor. Tumor cells have small round, oval, and angulated hyperchromatic nuclei and delicate processes which show strong immunostaining for neurofilament protein (NFP). Tumor cells are arranged in diffuse sheets. Prominent Homer-Wright rosettes are observed in several areas. Scattered mitotic figures are identified. No areas of necrosis are observed.<ref name=micropb2image>Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file [http://radiopaedia.org/cases/pineoblastoma-3 here]). Creative Commons BY-SA-NC</ref></sub>
</gallery>
===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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*[[MIB1|MIB-1]]
*[[MIB1|MIB-1]]
*[[Ki-67 (Biology)|Ki-67]]
*[[Ki-67 (Biology)|Ki-67]]
====Gallery====
<gallery>
Image:800px-Pineoblastoma neurofilament.jpg|<sub>Immunohistochemical stain of a pineoblastoma demonstrating positivity to neurofilament.<ref name=microimage1>Microscopic images of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015</ref></sub>
Image:800px-Pineoblastoma gfap.jpg|<sub>Immunohistochemical stain of a pineoblastoma demonstrating positivity to GFAP.<ref name=microimage1>Microscopic images of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015</ref></sub>
</gallery>


==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]]
*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]] of the pineal gland
*[[Astrocytoma|Astrocytoma of the pineal gland]]
*[[Meningioma]] near pineal gland
*[[Meningioma|Meningioma near pineal gland]]
*[[Intracerebral metastases|Pineal metastasis]]
*[[Intracerebral metastases|Pineal metastasis]]
*[[Cavernoma]] in pineal region
*[[Cavernoma|Cavernoma in pineal region]]
*[[Aneurysm]] in pineal region
*[[Aneurysm|Aneurysm in pineal region]]
*[[Glioblastoma multiforme]]
*[[Medulloblastoma]]


==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 the children and young adult population.<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>
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===
When evaluating a patient for pineoblastoma, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough family and past medical history review.
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===
*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>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>
*[[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>
*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]])
*Deficiency in upward-gaze
*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>


==CT==
===MRI===
*Head CT scan may be diagnostic of pineoblastoma.
*[[Brain]] [[Magnetic resonance imaging|MRI]] 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]].<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>
*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>
 
===Gallery===
<gallery>
Image:Pineoblastoma ct image 1.jpg|<sub>Pineal calcification: exploded calcification of tumors of pineal cell origin, whereas engulfed calcification by germinomas.<ref name=ctimage1>Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file [http://radiopaedia.org/cases/pineal-tumour-calcification-illustration here]). Creative Commons BY-SA-NC</ref></sub>
Image:Pineoblastoma ct image 2.jpg|<sub>Single head CT image demonstrates a soft tissue mass in the region of the pineal gland with eccentric calcification (anterior) and evidence of hydrocephalus.<ref name=ctiomage2>Image courtesy of Dr. Michael Sargent. Radiopaedia (original file [http://radiopaedia.org/cases/pineoblastoma-2 here]). Creative Commons BY-SA-NC</ref></sub>
Image:Pineoblastoma ct image 3.jpg|<sub>Pre-contrast CT scan demonstrating moderate internal obstructive hydrocephalus, due to a large, partially calcified, dense mass which appears to be centered upon the pineal gland. The quadrigeminal plate appears anteriorly displaced and the aqueduct obliterated. It does not appear to have an intimate relationship with the tentorium.<ref name=ctimage2pb>Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file [http://radiopaedia.org/cases/pineoblastoma-3 here]). Creative Commons BY-SA-NC</ref></sub>
 


 
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==MRI==
*Brain MRI may be diagnostic of pineoblastoma.
*Features on 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>
 
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| 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

Most recent articles on Pineoblastoma

Most cited articles on Pineoblastoma

Review articles on Pineoblastoma

Articles on Pineoblastoma in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Pineoblastoma

Images of Pineoblastoma

Photos of Pineoblastoma

Podcasts & MP3s on Pineoblastoma

Videos on Pineoblastoma

Evidence Based Medicine

Cochrane Collaboration on Pineoblastoma

Bandolier on Pineoblastoma

TRIP on Pineoblastoma

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

NHS PRODIGY Guidance

FDA on Pineoblastoma

CDC on Pineoblastoma

Books

Books on Pineoblastoma

News

Pineoblastoma in the news

Be alerted to news on Pineoblastoma

News trends on Pineoblastoma

Commentary

Blogs on Pineoblastoma

Definitions

Definitions of Pineoblastoma

Patient Resources / Community

Patient resources on Pineoblastoma

Discussion groups on Pineoblastoma

Patient Handouts on Pineoblastoma

Directions to Hospitals Treating Pineoblastoma

Risk calculators and risk factors for Pineoblastoma

Healthcare Provider Resources

Symptoms of Pineoblastoma

Causes & Risk Factors for Pineoblastoma

Diagnostic studies for Pineoblastoma

Treatment of Pineoblastoma

Continuing Medical Education (CME)

CME Programs on Pineoblastoma

International

Pineoblastoma en Espanol

Pineoblastoma en Francais

Business

Pineoblastoma in the Marketplace

Patents on Pineoblastoma

Experimental / Informatics

List of terms related to Pineoblastoma

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

Gross Pathology

On gross pathology, pineoblastoma is characterized by solid, large poorly defined masses.[3]

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.[4]

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]

Epidemiology and Demographics

Prevalence

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

Complications

Common complications of pineoblastoma include:[14][15]

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

Neurological

Laboratory Diagnosis

There are no specific laboratory findings for pineocytoma. However, the following findings are of significant

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

  • Isointense to hypointense to adjacent brain

T2

T1 with gadolinium contrast [T1 C+ (Gd)]

  • Vivid heterogenous enhancement

Diffuse weighted imaging/Apparent diffusion coefficient [DWI/ADC]

  • Restricted diffusion due to dense cellular packing
  • ADC values are typically 400-800 mm2/s

Other Imaging Findings

Other imaging studies for pineoblastoma include magnetic resonance spectroscopy (MR spectroscopy), which demonstrates:[19]

Other Diagnostic Studies

Stereotactic biopsy

Treatment

Management Options of Penial Gland tumors
CSF diversion
Surgical resection
Radiation
Stereotactic radiosurgery
  • SRS is increasingly being used to treat 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
  • 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. 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
  2. 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.
  3. 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
  4. Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  5. Microscopic features of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  6. 6.0 6.1 Histology of pineoblastoma. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-3. Accessed on December 1, 2015
  7. IHC of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  8. 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
  9. DDx of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  10. 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. 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.
  12. General features of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  13. 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. 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
  15. 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. 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
  17. 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
  18. 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
  19. MR spectroscopy of pineoblastoma. Dr Mohammad A. ElBeialy. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-6. Accessed on December 1, 2015


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