Pineal germinoma: Difference between revisions

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==Overview==
==Overview==
Pineal germinoma is the most common tumor of the pineal gland accounting for 50% of all tumors and the majority (80%) of intracranial germ cell tumors.<ref name=overviewpg1>Pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref> Pineal germinoma is a type of germ cell tumor. It refers to a tumor in the pineal gland that has a histology identical to two other tumors: [[dysgerminoma]] in the ovary and [[seminoma]] in the testis.<ref name=overviewpg2>Classification of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref>
Pineal germinoma is the most common [[tumor]] of the pineal gland accounting for 50% of all tumors and the majority (80%) of intracranial [[germ cell tumors]].<ref name="overviewpg1">Pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref> Pineal germinoma is a type of [[germ cell tumor]]. It refers to a [[tumor]] in the [[pineal gland]] that has a [[histology]] identical to two other tumors, i.e. [[dysgerminoma]] in the [[ovary]] and [[seminoma]] in the [[testis]].<ref name="overviewpg2">Classification of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref>


==Pathophysiology==
==Pathophysiology==
===Pathogenesis===
===Pathogenesis===
*Pineal germinoma is a malignant neoplasm of the germinal tissue of the pineal region.<ref name=overviewpg2>Classification of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref>
*Pineal germinoma is a [[malignant]] [[neoplasm]] of the germinal tissue of the [[Pineal gland|pineal]] region.<ref name="overviewpg2">Classification of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref>
*Germinomas are thought to originate from an error of development, when certain primordial germ cells fail to migrate properly. Germinomas lack histologic differentiation, whereas nongerminomatous germ cell tumors display a variety of differentiation. Like other germ cell tumors, germinomas can undergo malignant transformation.<ref name=overviewpg3>Natural history of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref>
*Germinomas are thought to originate from an error of development, when certain [[primordial germ cells]] fail to migrate properly. Germinomas lack [[histologic]] [[differentiation]], whereas nongerminomatous [[germ cell tumors]] display a variety of [[differentiation]]. Like other [[germ cell tumors]], germinomas can undergo [[malignant transformation]].<ref name="overviewpg3">Natural history of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref>
 
===Associated Pathology===
*Pineal germinoma may be associated with [[Down syndrome]].<ref name="pmid15280413">{{cite journal| author=Tan HW, Ty A, Goh SG, Wong MC, Hong A, Chuah KL| title=Pineal yolk sac tumour with a solid pattern: a case report in a Chinese adult man with Down's syndrome. | journal=J Clin Pathol | year= 2004 | volume= 57 | issue= 8 | pages= 882-4 | pmid=15280413 | doi=10.1136/jcp.2004.016659 | pmc=PMC1770394 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15280413  }} </ref>


===Gross Pathology===
===Gross Pathology===
*On gross pathology, pineal germinoma is characterized by a mass whose external surface is smooth and bosselated (knobby) and the interior is soft, fleshy, and either cream-coloured, gray, pink, or tan.<ref name=grosspb1>Histology of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref>
*On [[gross pathology]], pineal germinoma is characterized by a mass whose external surface is smooth and bosselated (knobby) and the interior is soft, fleshy, and either cream-coloured, gray, pink, or tan.<ref name="grosspb1">Histology of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref>


===Microscopic Pathology===
===Microscopic Pathology===
*On histopathological analysis, pineal germinoma is characterized by uniform large, round cells with vesicular [[nuclei]] and clear or finely granular cytoplasm that is [[eosinophilic]]. Typically, the stroma contains [[lymphocytes]] and approximately 20% of patients have sarcoid-like granulomas.<ref name=grosspb1>Histology of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref>
*On [[histopathological]] analysis, pineal germinoma is characterized by uniform large, round cells with vesicular [[nuclei]] and clear or finely granular cytoplasm that is [[eosinophilic]]. Typically, the [[stroma]] contains [[lymphocytes]] and approximately 20% of patients have sarcoid-like [[granulomas]].<ref name="grosspb1">Histology of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015</ref>


===Immunohistochemistry===
===Immunohistochemistry===
Pineal germinoma is demonstrated by positivity to [[tumor markers]] such as:<ref name=ihcpg1>Histology of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma. Accessed on December 2, 2015</ref>
Pineal germinoma is demonstrated by positivity to [[tumor markers]] such as:<ref name="ihcpg1">Histology of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma. Accessed on December 2, 2015</ref>
*[[CD117]]
*[[CD117]]
*PLAP
*PLAP
*[[Human chorionic gonadotropin|β-HCG]]


==Differentiating Pineal Germinoma from other Diseases==
==Differentiating Pineal Germinoma from other Diseases==
Pineal germinoma 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>
Pineal germinoma 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]]
*[[Pineoblastoma]]
*[[Pineoblastoma]]
*Pineal embryonal carcinoma
*[[Pineal embryonal carcinoma]]
*Pineal choriocarcinoma
*[[Pineal choriocarcinoma]]
*Pineal yolk sac carcinoma (endodermal sinus tumor)
*[[Pineal yolk sac carcinoma]]
*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]]


==Epidemiology and Demographics==
==Epidemiology and Demographics==
===Prevalence===
===Prevalence===
Pineal germinoma is the most common tumor of the [[pineal gland]] but accounts for less than 1% of all the [[brain tumor|intracranial tumors]]. Pineal germinoma accounts for 50% of all the pineal gland tumors and the majority (80%) of the intracranial germ cell tumors.<ref name=overviewpg1>Pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref><ref name=epipg2>Epidemiology of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma. Accessed on Dcember 2, 2015</ref>
Pineal germinoma is the most common tumor of the [[pineal gland]], but accounts for less than 1% of all the [[brain tumor|intracranial tumors]]. Pineal germinoma accounts for 50% of all the [[pineal gland tumors]] and the majority (80%) of the intracranial [[germ cell tumors]].<ref name="overviewpg1">Pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref><ref name="epipg2">Epidemiology of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma. Accessed on Dcember 2, 2015</ref>


===Age===
===Age===
Pineal germinoma is a disease that tends to affect the children and young adult population.<ref name=epiagepb1>Epidemiology of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref> Most patients are 20 years or younger at the time of diagnosis.
Pineal germinoma is a disease that tends to affect the children and young adult population.<ref name="epiagepb1">Epidemiology of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref> Most patients are 20 years or younger at the time of [[diagnosis]].


===Gender===
===Gender===
Males are more commonly affected with pineal germinoma than females. The male to female ratio is approximately 13 to 1.<ref name=epiagepb1>Epidemiology of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref>
Males are more commonly affected with pineal germinoma than females. The male to female ratio is approximately 13 to 1.<ref name="epiagepb1">Epidemiology of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref>


==Natural History, Complications and Prognosis==
==Natural History, Complications and Prognosis==
===Natural History===
===Natural History===
If left untreated, patients with pineal germinoma may progress to develop [[seizures]], [[obstructive hydrocephalus]], 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>
If left untreated, patients with pineal germinoma may progress to develop [[seizures]], [[obstructive hydrocephalus]], and CSF metastasis.<ref name="nathispg1">Clinical presentation of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 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 pineal germinoma include:<ref name="nathispg1">Clinical presentation of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 2015</ref><ref name="complicapg1">Treatment and prognosis of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref>
*[[Obstructive hydrocephalus]]
*[[Obstructive hydrocephalus]]
*Local recurrence
*[[Leptomeningeal]] spread
*[[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 excellent, and the [[Five year survival rate|5-year survival rate]] of patients with pineal germinoma is approximately 90%.<ref name="prognosispg1">Prognosis of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma#fn__22. Accessed on December 2, 2015</ref>  
*Multifocal or disseminated lesions are associated with poorer [[prognosis]].<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>
*Pineal germinoma usually presents with negative biological markers. The prognosis of patients with [[Human chorionic gonadotropin|beta-human chorionic gonadotropin]] ([[Human chorionic gonadotropin|β-HCG]] )secreting germinoma has been suggested to be worse than that of patients with pure germinoma.


==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.
*When evaluating a patient for pineal germinoma, you should take a detailed history of the presenting [[symptom]] (onset, duration, and progression), other associated [[symptoms]], and a thorough [[Family history|family]] and past medical history review.


===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 pineal germinoma is mainly from the [[obstructive hydrocephalus]] secondary to compression of the [[tectum]] of the midbrain and obstruction of the [[Cerebral aqueduct|aqueduct]].<ref name="symppg1">Clinical presentation of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 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 pineal germinoma include:<ref name="pmid25552796">{{cite journal| author=Reddy MP, Saad AF, Doughty KE, Armstrong D, Melguizo-Gavilanes I, Cheek BS et al.| title=Intracranial germinoma. | journal=Proc (Bayl Univ Med Cent) | year= 2015 | volume= 28 | issue= 1 | pages= 43-5 | pmid=25552796 | doi= | pmc=PMC4264708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25552796 }} </ref>
:*[[Headache]]s
:*[[Headache]]s
:*[[Nausea]]
:*[[Nausea]]
:*[[Vomiting]]
:*[[Vomiting]]
:*[[Seizures]]
:*[[Seizures]]
:*[[Tinnitus]]
:*[[Hearing loss]]
:*[[Hearing loss]]
:*[[Somnolence|Sleepiness]]
:*[[Somnolence|Sleepiness]]
:*[[Irritability]]
:*[[Irritability]]
:*Slowed speech
:*[[Fatigue]]
:*[[anorexia|Loss of appetite]]
:*[[Hair loss]]
:*Presyncopal episodes
:*[[Personality pathology|Personality changes]]
:*[[Personality pathology|Personality changes]]
:*[[Diplopia|Double vision]]
:*[[Diplopia|Double vision]]
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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 [[Colliculus|colliculi]] can lead to a characteristic [[gaze palsy]], known as [[Parinaud syndrome]].<ref name="symppg1">Clinical presentation of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015</ref>
*Common [[physical examination]] findings of pineal germinoma include:<ref name="pmid25552796">{{cite journal| author=Reddy MP, Saad AF, Doughty KE, Armstrong D, Melguizo-Gavilanes I, Cheek BS et al.| title=Intracranial germinoma. | journal=Proc (Bayl Univ Med Cent) | year= 2015 | volume= 28 | issue= 1 | pages= 43-5 | pmid=25552796 | doi= | pmc=PMC4264708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25552796 }} </ref>


===HEENT===
===HEENT===
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*Eyes that are constantly looking down ([[sunsetting sign]])
*Eyes that are constantly looking down ([[sunsetting sign]])
*Deficiency in upward-gaze
*Deficiency in upward-gaze
*Pupillary light-near dissociation (pupils respond to near stimuli but not light)
*[[Pupillary]] [[light-near dissociation]] (pupils respond to near stimuli but not light)
*Convergence-retraction [[nystagmus]]
*Convergence-retraction [[nystagmus]]
*[[Papilledema]]
*[[Papilledema]]
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*[[Mental retardation]]
*[[Mental retardation]]
*[[Muscle spasms]]
*[[Muscle spasms]]
*[[Hemiparesis]]
*[[Loss of bladder control]]
*[[Loss of bladder control]]
*[[Ataxia]]
*[[Ataxia]]
*[[Nerve palsy|Cranioneuropathies]]
*[[Tremor]]
:*[[abducens nerve palsy|VI<sup>th</sup> nerve palsy]]
*[[Dysmetria]]
*Pronator drift


==CT==
==CT==
*Head CT scan may be diagnostic of pineoblastoma.
*Head [[Computed tomography|CT scan]] may be diagnostic of pineal germinoma.
*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 pineal germinoma include:<ref name="pmid25552796">{{cite journal| author=Reddy MP, Saad AF, Doughty KE, Armstrong D, Melguizo-Gavilanes I, Cheek BS et al.| title=Intracranial germinoma. | journal=Proc (Bayl Univ Med Cent) | year= 2015 | volume= 28 | issue= 1 | pages= 43-5 | pmid=25552796 | doi= | pmc=PMC4264708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25552796  }} </ref><ref name="ctpg1">CT radiographic features of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 2015</ref>
:*[[Obstructive hydrocephalus]]
 
:*Large midline mass in the [[Pineal gland|pineal]] region
::*Hyperdense compared to normal brain
::*Vivid contrast enhancement
::*[[Calcification]]: usually representing "engulfed" pineal calcification


===Gallery===
===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>
Image:Pineoblastoma ct image 4.jpg|<sub>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.<ref name=ctimage4pb>Image courtesy of Dr. Bita Abbasi. Radiopaedia (original file [http://radiopaedia.org/cases/pineoblastoma-1 here]). Creative Commons BY-SA-NC</ref></sub>
</gallery>
==MRI==
==MRI==
*Brain MRI may be diagnostic of pineoblastoma.
*[[Brain]] [[MRI]] may be diagnostic of pineal germinoma.
*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 pineal germinoma include:<ref name="pmid25552796">{{cite journal| author=Reddy MP, Saad AF, Doughty KE, Armstrong D, Melguizo-Gavilanes I, Cheek BS et al.| title=Intracranial germinoma. | journal=Proc (Bayl Univ Med Cent) | year= 2015 | volume= 28 | issue= 1 | pages= 43-5 | pmid=25552796 | doi= | pmc=PMC4264708 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25552796  }} </ref><ref name="mripg1">MRI radiographic features of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 2015</ref>


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T1
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*Isointense to hypointense to adjacent brain
*Isointense to adjacent brain
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T2
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*Isointense to adjacent brain
*Isointense to adjacent brain
*Areas of [[cyst]] formation or [[necrosis]] may be present
|-
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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 homogenous 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 [DWI/ADC]
Diffuse weighted imaging [DWI]
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
*Restricted diffusion due to dense cellular packing
*Restricted diffusion due to high cellularity
*ADC values are typically 400-800 mm2/s
|}
|}


===Gallery===
===Gallery===
<gallery>
<gallery>
Image:MRI image of pineoblastoma 1.jpg|<sub>MRI image of pineoblastoma demonstrating restricted diffusion on DWI.<ref name=mriimage1pb>Image courtesy of Dr. Michael Sargent. Radiopaedia (original file [http://radiopaedia.org/cases/pineoblastoma-2 here]). Creative Commons BY-SA-NC</ref></sub>
 
Image:MRI image of pineoblastoma 2.jpg|<sub>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.<ref name=mriimage1pb>Image courtesy of Dr. Michael Sargent. Radiopaedia (original file [http://radiopaedia.org/cases/pineoblastoma-2 here]). Creative Commons BY-SA-NC</ref></sub>
Image:MRI of pineal germinoma 1.jpg|<sub>Sagittal MRI with contrast demonstrating a large enhancing mass centered on the pineal region. It is heterogeneous with areas of cystic change. There is marked compression of the tectum with resulting obstructive hydrocephalus. A little surrounding edema is also present.<ref name=mriimage1pg>Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file [http://radiopaedia.org/cases/pineal-germinoma here]). Creative Commons BY-SA-NC</ref></sub>
Image:MRI image of pineoblastoma 3.jpg|<sub>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.<ref name=mriimage3pb>Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file [http://radiopaedia.org/cases/pineoblastoma-3 here]). Creative Commons BY-SA-NC</ref></sub>
Image:MRI image of pineoblastoma 4.jpg|<sub>Axial T2-weighted MRI image demonstrating a lesion that is hyperintense.<ref name=mriimage4pb>Image courtesy of Dr. Mohammad A. ElBeialy. Radiopaedia (original file [http://radiopaedia.org/cases/pineoblastoma-6 here]). Creative Commons BY-SA-NC</ref></sub>


</gallery>
</gallery>


==Other Imaging Findings==
==Treatment==
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>
*The mainstay of therapy for pineal germinoma is [[radiotherapy]], since it is highly radiosensitive.<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>
*Elevation of the [[choline]] and lipid [[lactate]] peaks
*In children, there is an attempt to reduce the toxicity of radiation therapy by the use of [[chemotherapy]] in combination with reduced dose radiation to decrease the volume of normal tissue irradiated by stereotactic radiotherapy.
*Depression of the neural markers ([[N-Acetylaspartate|N-acetyl aspartate (NAA)]] and [[creatine|creatine (Cr)]]
*The various chemotherapeutic agents that may be used for the treatment of pineal germinoma include:<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="rxpg1">Treatment and prognosis of pineal germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 3, 2015</ref>
*Prominent [[glutamate]] and [[taurine]] peaks at 3.4 ppm with shot TE signal voxel MR spectroscopy
**[[Cisplatin]]
**[[Etoposide]]
**[[Carboplatin]]
**[[Bleomycin]]


===Gallery===
{| class="wikitable"
<gallery>
|+
Image:MR spectroscopy.jpg|<sub>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).<ref name=mrspectroscopy111>Image courtesy of Dr. Mohammad A. ElBeialy. Radiopaedia (original file [http://radiopaedia.org/cases/pineoblastoma-6 here]). Creative Commons BY-SA-NC</ref></sub>
! colspan="2" align="center" style="background:#4479BA; color: #FFFFFF;" + |Management Options for Pineal Gland Tumors
|-
|'''Radiation'''
|
* Postoperative [[Adjuvant therapy|adjuvant RT]] is frequently (but not universally) recommended, and local control is dose-dependent.  
* The [[incidence]] of [[leptomeningeal]] recurrence was significantly lower among patients receiving CSI compared with those who did not.
* The [[Five-year survival rate|five-year survival rates]] are 86 and 49 percent for [[pineocytomas]] and non-[[pineocytoma]] PPTs, respectively.
* [[Adjuvant therapy|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 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.


</gallery>
* 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, IRS may develop into an attractive alternative to [[microsurgery]] in de novo diagnosed [[pineocytomas]]. In malignant PPTs, IRS may be routinely applied in a multimodality treatment schedule supplementary to conventional irradiation.
==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>
|'''Chemotherapy as part of multimodality therapy'''
*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>
* 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.
*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.
* [[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.
*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.
* 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
*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 [[Midbrain tectum|tectal region]]. Neuroendoscopic biopsy procedures have been proven safe with low 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>
|}
*Patients with pineal [[germinoma]] may develop [[hydrocephalus]] in majority of the cases and they will require [[CSF]] diversion. [[Ventriculoperitoneal shunt|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 [[Midbrain tectum|tectal region]]. Neuroendoscopic [[biopsy]] procedures have been proven safe with low 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 01:28, 3 June 2019


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

Synonyms and keywords: Pineal gland germinoma; Pineal germinomas; Germinoma of the pineal gland; Pineal dysgerminoma; Pineal dysgerminomas; Pineal gland tumor; Brain tumor

Overview

Pineal germinoma is the most common tumor of the pineal gland accounting for 50% of all tumors and the majority (80%) of intracranial germ cell tumors.[1] Pineal germinoma is a type of germ cell tumor. It refers to a tumor in the pineal gland that has a histology identical to two other tumors, i.e. dysgerminoma in the ovary and seminoma in the testis.[2]

Pathophysiology

Pathogenesis

Associated Pathology

Gross Pathology

  • On gross pathology, pineal germinoma is characterized by a mass whose external surface is smooth and bosselated (knobby) and the interior is soft, fleshy, and either cream-coloured, gray, pink, or tan.[5]

Microscopic Pathology

Immunohistochemistry

Pineal germinoma is demonstrated by positivity to tumor markers such as:[6]

Differentiating Pineal Germinoma from other Diseases

Pineal germinoma must be differentiated from:[7][8]

Epidemiology and Demographics

Prevalence

Pineal germinoma is the most common tumor of the pineal gland, but accounts for less than 1% of all the intracranial tumors. Pineal germinoma accounts for 50% of all the pineal gland tumors and the majority (80%) of the intracranial germ cell tumors.[1][9]

Age

Pineal germinoma is a disease that tends to affect the children and young adult population.[10] Most patients are 20 years or younger at the time of diagnosis.

Gender

Males are more commonly affected with pineal germinoma than females. The male to female ratio is approximately 13 to 1.[10]

Natural History, Complications and Prognosis

Natural History

If left untreated, patients with pineal germinoma may progress to develop seizures, obstructive hydrocephalus, and CSF metastasis.[11]

Complications

Common complications of pineal germinoma include:[11][12]

Prognosis

  • Prognosis is generally excellent, and the 5-year survival rate of patients with pineal germinoma is approximately 90%.[13]
  • Multifocal or disseminated lesions are associated with poorer prognosis.[14]
  • Pineal germinoma usually presents with negative biological markers. The prognosis of patients with beta-human chorionic gonadotropin (β-HCG )secreting germinoma has been suggested to be worse than that of patients with pure germinoma.

History and Symptoms

History

  • When evaluating a patient for pineal germinoma, 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

HEENT

Neurological

CT

  • Head CT scan may be diagnostic of pineal germinoma.
  • Findings on CT scan suggestive of pineal germinoma include:[16][17]
  • Large midline mass in the pineal region
  • Hyperdense compared to normal brain
  • Vivid contrast enhancement
  • Calcification: usually representing "engulfed" pineal calcification

Gallery

MRI

  • Brain MRI may be diagnostic of pineal germinoma.
  • Features on MRI suggestive of pineal germinoma include:[16][18]
MRI component Findings

T1

  • Isointense to adjacent brain

T2

  • Isointense to adjacent brain

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

  • Vivid homogenous enhancement

Diffuse weighted imaging [DWI]

  • Restricted diffusion due to high cellularity

Gallery

  • Sagittal MRI with contrast demonstrating a large enhancing mass centered on the pineal region. It is heterogeneous with areas of cystic change. There is marked compression of the tectum with resulting obstructive hydrocephalus. A little surrounding edema is also present.[19]

    Sagittal MRI with contrast demonstrating a large enhancing mass centered on the pineal region. It is heterogeneous with areas of cystic change. There is marked compression of the tectum with resulting obstructive hydrocephalus. A little surrounding edema is also present.[19]

Treatment

  • The mainstay of therapy for pineal germinoma is radiotherapy, since it is highly radiosensitive.[14]
  • In children, there is an attempt to reduce the toxicity of radiation therapy by the use of chemotherapy in combination with reduced dose radiation to decrease the volume of normal tissue irradiated by stereotactic radiotherapy.
  • The various chemotherapeutic agents that may be used for the treatment of pineal germinoma include:[14][20]
Management Options for Pineal Gland Tumors
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, IRS may develop into an attractive alternative to microsurgery in de novo 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

References

  1. ↑ 1.0 1.1 Pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015
  2. ↑ 2.0 2.1 Classification of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015
  3. ↑ Natural history of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015
  4. ↑ Tan HW, Ty A, Goh SG, Wong MC, Hong A, Chuah KL (2004). "Pineal yolk sac tumour with a solid pattern: a case report in a Chinese adult man with Down's syndrome". J Clin Pathol. 57 (8): 882–4. doi:10.1136/jcp.2004.016659. PMC 1770394. PMID 15280413.
  5. ↑ 5.0 5.1 Histology of germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 2, 2015
  6. ↑ Histology of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma. Accessed on December 2, 2015
  7. ↑ 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
  8. ↑ DDx of pineoblastoma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Pineal_gland#Pineoblastoma. Accessed on December 1, 2015
  9. ↑ Epidemiology of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma. Accessed on Dcember 2, 2015
  10. ↑ 10.0 10.1 Epidemiology of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015
  11. ↑ 11.0 11.1 Clinical presentation of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 2015
  12. ↑ Treatment and prognosis of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015
  13. ↑ Prognosis of pineal germinoma. Operative Neurosurgery 2015. http://operativeneurosurgery.com/doku.php?id=pineal_germinoma#fn__22. Accessed on December 2, 2015
  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. ↑ 15.0 15.1 Clinical presentation of pineal germinoma. Dr Henry Knipe et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-germinoma. Accessed on December 2, 2015
  16. ↑ 16.0 16.1 16.2 16.3 Reddy MP, Saad AF, Doughty KE, Armstrong D, Melguizo-Gavilanes I, Cheek BS; et al. (2015). "Intracranial germinoma". Proc (Bayl Univ Med Cent). 28 (1): 43–5. PMC 4264708. PMID 25552796.
  17. ↑ CT radiographic features of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 2015
  18. ↑ MRI radiographic features of intracranial germ cell tumors. Dr Ayush Goel and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/intracranial-germ-cell-tumours. Accessed on December 2, 2015
  19. ↑ Image courtesy of Dr. Frank Gaillard. Radiopaedia (original file here). Creative Commons BY-SA-NC
  20. ↑ Treatment and prognosis of pineal germinoma. Wikipedia 2015. https://en.wikipedia.org/wiki/Germinoma. Accessed on December 3, 2015


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