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==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.<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>


==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 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="intropineoblastoma2">Pinealoblastoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pinealoblastoma. Accessed on December 1, 2015</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>


====Gallery====
====Gallery====
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===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 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 appearance
*Hypercellular appearance
*Tightly packed small round blue cells (high nuclear to cytoplasmic ratio)
*Tightly packed small round blue cells (high nuclear to cytoplasmic ratio)
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===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]]
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===Age===
===Age===
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>
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. 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>


===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
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===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 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==
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===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>
*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 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===
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==CT==
==CT==
*Head CT scan may be diagnostic of pineoblastoma.
*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]].<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>
*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>


===Gallery===
===Gallery===
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==MRI==
==MRI==
*Brain MRI may be diagnostic of pineoblastoma.
*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>
*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>


{| style="border: 0px; font-size: 90%; margin: 3px; width:1000px align=center"
{| style="border: 0px; font-size: 90%; margin: 3px; width:1000px align=center"
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|+
|+
! 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}}
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T1
T1
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*Isointense to hypointense to adjacent brain
*Isointense to hypointense to adjacent brain
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|-
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*Areas of [[cyst]] formation or [[necrosis]] may be present
*Areas of [[cyst]] formation or [[necrosis]] may be present
|-
|-
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| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align="center" |
T1 with gadolinium contrast [T1 C+ (Gd)]
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
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|-
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| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align="center" |
Diffuse weighted imaging/Apparent diffusion coefficient [DWI/ADC]
Diffuse weighted imaging/Apparent diffusion coefficient [DWI/ADC]
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==Other Imaging Findings==
==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>
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
*Elevation of the [[choline]] and lipid [[lactate]] peaks
*Depression of the neural markers ([[N-Acetylaspartate|N-acetyl aspartate (NAA)]] and [[creatine|creatine (Cr)]]
*Depression of the neural markers ([[N-Acetylaspartate|N-acetyl aspartate (NAA)]] and [[creatine|creatine (Cr)]]
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==Treatment==
==Treatment==
*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 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.
*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.
*Gross total resection has been associated with improved 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>
*Gross total resection has been associated with improved 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>

Revision as of 01:18, 2 May 2019


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

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Synonyms and keywords: Pineoblastomas; Pinealoblastoma; Pinealoblastomas; PB; Pineal parenchymal tumor; Pineal gland tumor; Brain 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.[1]

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.[2]

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".[3]

Gross Pathology

On gross pathology, pineoblastoma is characterized by solid, large poorly defined masses.[4]

Gallery

  • 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.[5]

    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.[5]

Microscopic Pathology

On microscopic histopathological analysis, pineoblastoma is characterized by:[2][6][7]

  • 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

Gallery

  • Pathology specimen of a pineoblastoma (HE stain, x200 magnification).[8]

    Pathology specimen of a pineoblastoma (HE stain, x200 magnification).[8]

  • 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.[9]

    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.[9]

Immunohistochemistry

Pineoblastoma is demonstrated by positivity to tumor markers such as:[7][10]

Gallery

  • Immunohistochemical stain of a pineoblastoma demonstrating positivity to neurofilament.[8]

    Immunohistochemical stain of a pineoblastoma demonstrating positivity to neurofilament.[8]

  • Immunohistochemical stain of a pineoblastoma demonstrating positivity to GFAP.[8]

    Immunohistochemical stain of a pineoblastoma demonstrating positivity to GFAP.[8]

Differentiating Pineoblastoma from other Diseases

Pineoblastoma must be differentiated from:[11][12]

Epidemiology and Demographics

Prevalence

Pineoblastoma constitutes approximately 0.1% of the intracranial neoplasms.[13] Pineoblastoma together with germ cell tumors are the most common pineal tumors in children.[14]

Age

Pineoblastoma is a disease that tends to affect children and young adults.[1]

Gender

Pineoblastoma affects men and women equally.[15]

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 CSF metastasis.[16]

Complications

Common complications of pineoblastoma include:[16][17]

Prognosis

Prognosis is generally poor, and the 5-year survival rate of patients with pineoblastoma is approximately 58%.[18]

History and Symptoms

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.

Symptoms

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:[13][16]

HEENT

  • Bulging soft spots (fontanelles)
  • Eyes that are constantly looking down (sunsetting sign)
  • Deficiency in upward-gaze
  • Pupillary light-near dissociation (pupils respond to near stimuli but not light)
  • Convergence-retraction nystagmus
  • Papilledema

Neurological

CT

  • 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.[19]

Gallery

  • Pineal calcification: exploded calcification of tumors of pineal cell origin, whereas engulfed calcification by germinomas.[20]

    Pineal calcification: exploded calcification of tumors of pineal cell origin, whereas engulfed calcification by germinomas.[20]

  • Single head CT image demonstrates a soft tissue mass in the region of the pineal gland with eccentric calcification (anterior) and evidence of hydrocephalus.[21]

    Single head CT image demonstrates a soft tissue mass in the region of the pineal gland with eccentric calcification (anterior) and evidence of hydrocephalus.[21]

  • 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.[22]

    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.[22]

  • Axial brain CT image demonstrating a large hyperdense lobulated mass in the pineal region with peripheral foci of calcification and associated hydrocephalus. A VP shunt has recently been inserted (note the small amount of pneumocephalus). Hyperdense material is observed coating the frontal horns of the lateral ventricle and filling the floor of the third ventricle.[23]

    Axial brain CT image demonstrating a large hyperdense lobulated mass in the pineal region with peripheral foci of calcification and associated hydrocephalus. A VP shunt has recently been inserted (note the small amount of pneumocephalus). Hyperdense material is observed coating the frontal horns of the lateral ventricle and filling the floor of the third ventricle.[23]

MRI

  • Brain MRI may be diagnostic of pineoblastoma.
  • Features on MRI suggestive of pineoblastoma include:[24]
MRI component Findings

T1

  • Isointense to hypointense to adjacent brain

T2

  • Isointense to adjacent brain
  • Areas of cyst formation or necrosis may be present

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

Gallery

  • MRI image of pineoblastoma demonstrating restricted diffusion on DWI.[25]

    MRI image of pineoblastoma demonstrating restricted diffusion on DWI.[25]

  • Sagittal T1 with contrast CT image of pineoblastoma demonstrating an enhancing mass in the region of the pineal gland is present. A tongue of tissue is observed extending inferiorly through the aqueduct, obstructing it, and resulting in hydrocephalus with transependymal edema.[25]

    Sagittal T1 with contrast CT image of pineoblastoma demonstrating an enhancing mass in the region of the pineal gland is present. A tongue of tissue is observed extending inferiorly through the aqueduct, obstructing it, and resulting in hydrocephalus with transependymal edema.[25]

  • Axial T1 with contrast demonstrating an irregular heterogenous enhancing pineal mass with several tiny cystic foci and eccentric coarse calcifications. There is moderate mass effect on the adjacent tectum and vermis, with loss of definition and possible parenchymal invasion on the left. There is associated aqueduct compression with moderate hydrocephalus.[26]

    Axial T1 with contrast demonstrating an irregular heterogenous enhancing pineal mass with several tiny cystic foci and eccentric coarse calcifications. There is moderate mass effect on the adjacent tectum and vermis, with loss of definition and possible parenchymal invasion on the left. There is associated aqueduct compression with moderate hydrocephalus.[26]

  • Axial T2-weighted MRI image demonstrating a lesion that is hyperintense.[27]

    Axial T2-weighted MRI image demonstrating a lesion that is hyperintense.[27]

Other Imaging Findings

Other imaging studies for pineoblastoma include magnetic resonance spectroscopy (MR spectroscopy), which demonstrates:[28]

Gallery

  • MR spectroscopy of the lesion demonstrating elevation of the choline and lipid lactate peaks and depression of the neural markers; N-acetyl aspartate (NAA) and creatine (Cr).[29]

    MR spectroscopy of the lesion demonstrating elevation of the choline and lipid lactate peaks and depression of the neural markers; N-acetyl aspartate (NAA) and creatine (Cr).[29]

Treatment

  • The predominant therapy for pineoblastoma is surgical resection. Adjunctive chemotherapy and radiation may be required.[14][18]
  • 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.
  • Gross total resection has been associated with improved survival, similar to treatment with craniospinal irradiation and multi-agent chemotherapy.[14]
  • 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.
  • Tate et al. summarized the existing literature on patients with pineoblastoma and found that children under 5 years of age and subtotal tumor resection 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 containing adjuvant chemotherapy, an 84% 2-years progression free survival was reported in pineoblastomas without evidence of dissemination at presentation.
  • Patients with pineoblastoma will develop hydrocephalus in majority of the cases and they will require CSF diversion. Ventriculo-peritoneal (V-P) shunt placement is a viable option 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.[14]

References

  1. ↑ 1.0 1.1 General features of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  2. ↑ 2.0 2.1 Pathology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
  3. ↑ Pinealoblastoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Pinealoblastoma. Accessed on December 1, 2015
  4. ↑ 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
  5. ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  6. ↑ Microscopic features of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  7. ↑ 7.0 7.1 Histology of pineoblastoma. Dr Frank Gaillard. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-3. Accessed on December 1, 2015
  8. ↑ 8.0 8.1 8.2 Microscopic images of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  9. ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  10. ↑ IHC of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  11. ↑ 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
  12. ↑ DDx of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  13. ↑ 13.0 13.1 13.2 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.
  14. ↑ 14.0 14.1 14.2 14.3 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.
  15. ↑ Epidemiology of pineoblastoma. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineoblastoma. Accessed on December 1, 2015
  16. ↑ 16.0 16.1 16.2 16.3 16.4 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
  17. ↑ 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.
  18. ↑ 18.0 18.1 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
  19. ↑ 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
  20. ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  21. ↑ Image courtesy of Dr. Michael Sargent. Radiopaedia (original file here). Creative Commons BY-SA-NC
  22. ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  23. ↑ Image courtesy of Dr. Bita Abbasi. Radiopaedia (original file here). Creative Commons BY-SA-NC
  24. ↑ 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
  25. ↑ 25.0 25.1 Image courtesy of Dr. Michael Sargent. Radiopaedia (original file here). Creative Commons BY-SA-NC
  26. ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  27. ↑ Image courtesy of Dr. Mohammad A. ElBeialy. Radiopaedia (original file here). Creative Commons BY-SA-NC
  28. ↑ MR spectroscopy of pineoblastoma. Dr Mohammad A. ElBeialy. Radiopaedia 2015. http://radiopaedia.org/cases/pineoblastoma-6. Accessed on December 1, 2015
  29. ↑ Image courtesy of Dr. Mohammad A. ElBeialy. Radiopaedia (original file here). Creative Commons BY-SA-NC


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