Pineal yolk sac tumor: Difference between revisions

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{{SK}} Pineal yolk sac tumors; Pineal yolk sac tumour; Pineal yolk sac tumours; Pineal endodermal sinus tumor; Pineal endodermal sinus tumors; Pineal endodermal sinus tumour; Pineal endodermal sinus tumours; Pineal yolk sac carcinoma; Pineal yolk sac carcinomas; Pineal gland tumor; Germ cell tumor; Brain tumor
{{SK}} [[Pineal choriocarcinoma|Pineal]] [[Yolk sac tumor|yolk sac tumors]]; [[Pineal body|Pineal yolk sac tumour; Pineal yolk sac tumours; Pineal endodermal sinus tumo]]<nowiki/>r; [[Pineal choriocarcinoma|Pineal endodermal sinus tumors]]; [[Pineal|Pineal endodermal sinus tumour; Pineal endodermal sinus tumours; Pineal yolk sac carcinoma; Pineal yolk sac carcinomas; Pineal gland tumor; Germ cell tumor; Brain tumor]]


==Overview==
==Overview==
*Pineal yolk sac tumor is a rare type of extra gonadal yolk sac tumor. They make up a small fraction of all intracranial [[germ cell tumor]]s and an even small fraction of pineal masses overall.<ref name=Pinealyolksactumouroverview1>Pineal yolk sac tumour. Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-yolk-sac-tumour. Accessed on December 8, 2015</ref>
[[Pineal]] [[yolk sac]] [[tumor]] is a rare type of [[Extra-axial hematoma|extra gonadal yolk sac tumor]]. They make up a small fraction of all [[Intracranial abscess / granuloma|intracranial]] [[germ cell tumor]]s and an even small fraction of [[pineal]] masses overall.Pure [[Pineal body|pineal]] [[Endodermal sinus tumor|yolk sac tumors]] secrete [[AFP]].On microscopic [[histopathological]] analysis, [[Pineal yolk sac tumors|pineal yolk sac tumor]] is characterized by poorly differentiated [[Endothelial|endothelial-]][[Likelihood|like]], [[cuboidal]], or columnar cells with prominent nucleoli and significant mitotic activity.[[Pineal yolk sac tumors|Pineal yolk sac tumor]] is demonstrated by positivity to [[tumor markers]] such as [[AFP]], [[cytokeratin]], and [[Alpha 1-antitrypsin|AAT]].In upto 50% of cases, these tumors co-exist with other [[Germ cell tumor classification|germ cell tumors]]
*Pure pineal yolk sac tumors secrete [[AFP]].
[[Pineal yolk sac tumors|Pineal yolk sac tumor]] may be associated with [[Down syndrome]].Common [[Complication (medicine)|complication]] of [[Pineal yolk sac tumors|pineal yolk sac tumor]] includes [[obstructive hydrocephalus]].[[Prognosis]] of [[Pineal yolk sac tumors|pineal yolk sac tumor]] is generally poor.[[Symptoms]] of [[Pineal yolk sac tumors|pineal yolk sac tumor]] include [[headache]], [[nausea]], [[vomiting]], [[weakness]], [[confusion]], and [[somnolence]].Head [[CT scan]] and brain [[MRI]] may be helpful in the diagnosis of [[Pineal yolk sac tumors|pineal yolk sac tumor]].On head CT scan, [[Pineal yolk sac tumors|pineal yolk sac tumor]] is characterized by a [[Hypo-functioning thyroid|hypodense]], [[Hetero-oligomer|heterogenous]] mass in the [[Pineal gland|pineal]] region with signs of [[obstructive hydrocephalus]].On brain MRI, [[Pineal gland|pineal]] yolk sac tumor is characterized by [[Hypo-functioning thyroid|hypointensity]] on [[T1|T1-weighted images]] and [[Hyper IgM syndrome|hyperintensity on T2-weighted images]].There may be enhancement after contrast administration.[[Biopsy]] is generally done to confirm the [[diagnosis]] of [[pineal]] yolk sac tumor.
*On microscopic histopathological analysis, pineal yolk sac tumor is characterized by poorly differentiated endothelium-like, cuboidal, or columnar cells.
 
*Pineal yolk sac tumor may be associated with [[Down syndrome]].<ref name=associationspy1>Associations of pineal yolk sac tumor. Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/pineal-yolk-sac-tumour. Accessed on December 8, 2015</ref>
==Historical Perspective==
 
*Tumors of [[Brain-computer interface|brain]]<nowiki/>[[Brain-computer interface|staim]] as well as [[Pineal gland|pineal]] gland,were [[Unoprostone Ophthalmic (patient information)|unoperable]] until late of 20th century.<ref name="pmid24436899">{{cite journal| author=Shahinian H, Ra Y| title=Fully endoscopic resection of pineal region tumors. | journal=J Neurol Surg B Skull Base | year= 2013 | volume= 74 | issue= 3 | pages= 114-7 | pmid=24436899 | doi=10.1055/s-0033-1338165 | pmc=3712663 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24436899  }} </ref>
*Dr Cushing reported one of the first case in 1904.<ref name="pmid24436899">{{cite journal| author=Shahinian H, Ra Y| title=Fully endoscopic resection of pineal region tumors. | journal=J Neurol Surg B Skull Base | year= 2013 | volume= 74 | issue= 3 | pages= 114-7 | pmid=24436899 | doi=10.1055/s-0033-1338165 | pmc=3712663 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24436899  }} </ref>
*Dr Horsley in 1905 was the first who did direct surgical intervention,and in 1913 Dr Oppenhein and Krause resected pineal tumor sucssesfully.<ref name="pmid24436899">{{cite journal| author=Shahinian H, Ra Y| title=Fully endoscopic resection of pineal region tumors. | journal=J Neurol Surg B Skull Base | year= 2013 | volume= 74 | issue= 3 | pages= 114-7 | pmid=24436899 | doi=10.1055/s-0033-1338165 | pmc=3712663 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24436899  }} </ref>
 
*There are only two case Reports of [[Pineal body|pineal]] yolk sac tumor that are associated with [[Down syndrome|Down's syndrome]] in English literature.<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=1770394 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15280413  }} </ref>
 
==Classification==
*
 
*There is so many [[classification]] system for pure [[Pineal gland|pineal]] tumors.<ref name="pmid31441839">{{cite journal| author=Ji J, Gu C, Zhang M, Zhang H, Wang H, Qu Y et al.| title=Pineal region metastasis with intraventricular seeding: A case report and literature review. | journal=Medicine (Baltimore) | year= 2019 | volume= 98 | issue= 34 | pages= e16652 | pmid=31441839 | doi=10.1097/MD.0000000000016652 | pmc=6716749 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31441839  }} </ref>
*The most current system is for [[World Health Organization]](WHO).<ref name="pmid31441839">{{cite journal| author=Ji J, Gu C, Zhang M, Zhang H, Wang H, Qu Y et al.| title=Pineal region metastasis with intraventricular seeding: A case report and literature review. | journal=Medicine (Baltimore) | year= 2019 | volume= 98 | issue= 34 | pages= e16652 | pmid=31441839 | doi=10.1097/MD.0000000000016652 | pmc=6716749 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31441839  }} </ref>
*Louis and associates edited classification of [[Central nervous system|Central Nervous System tumors]]  and published in 2007.<ref name="pmid31441839">{{cite journal| author=Ji J, Gu C, Zhang M, Zhang H, Wang H, Qu Y et al.| title=Pineal region metastasis with intraventricular seeding: A case report and literature review. | journal=Medicine (Baltimore) | year= 2019 | volume= 98 | issue= 34 | pages= e16652 | pmid=31441839 | doi=10.1097/MD.0000000000016652 | pmc=6716749 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31441839  }} </ref>
 
Adapted from WHO:
 
{| class="wikitable sortable mw-collapsible"
!TUMOR
!FREQUENCY
! colspan="2" |ORIGIN
|-
!GERMCELL TOMURS
!60%
! colspan="2" |Rest of germ cells
|-
|[[Germinoma]] [[MAATS1|MATURE]] [[Teratoma|TERATOMAMATURE]] [[TERF2IP|TERATOMATERATOMA]] with Malignant [[Transformation (genetics)|Transformstion]] [[Yolk|Yolk sac tomur]]
([[Endodermal sinus tumor(EST)|endodermal sinus tumor]]) [[Embryonal carcinoma]] [[Choriocarcinoma]]
!
| colspan="2" |
|-
|[[PIN4|PINEAL PARANCHIMAL TUMORS]]
!30%
| colspan="2" |pineal glandular tissue
|-
|[[pineocytoma]] (WHO grade ɪ ) [[Pineal body|pineal paranchymal tomur]] of intermediate diffrentiation(WHO grade ɪɪ or ɪɪɪ)
[[pineoblastoma]](WHO grade ɪv) [[Papilla|papillary tumor]] of pineal region
|
|
|
|}
 
{| class="wikitable"
|+
!TOMURS OF SUPPORTIVE AND ADJUCENT STRUCTURES               
!10%                 
!
|-
|[[Astrocytoma|ASTROCYTOMAGlioma]] ([[glioblastoma]] or [[oligodendroglioma]])[[Medulloepithelioma]]
|
|[[Glial cell|Glial cells]]
|-
|[[Ependymoma|Ependymomachoroid plexus]] [[papilloma]]
|
|[[Ependymoma|Ependymal lining]]
|-
|[[Meningioma|MENINGIOMA]]
|
|[[Arachnoid mater|Arachnoid]] cells
|-
| rowspan="2" |[[Hemangioma (patient information)|HemangiomaHemangiopericytoma]]            or
[[Blastoma|blastomaChemodectomaCraniopharyngioma]]
|
| rowspan="2" |vascular cells
|-
|
|-
|NON-NEOPLASTIC TUMOR LIKE CONDITIONS
|
|< 1%
|-
|[[Arachnoid cysts]]
|
|[[Arachnoid|Arachnoid cells]]
|-
|[[Degenerative]] [[Cyst|cysts]]([[Pineal|pineal cysts]])
|
|[[Glial cells]]
|-
|[[Cysticercosis]]
|
|[[parasites]]
|-
|[[Arteriovenous malformations]]
|
|[[Vascularity|vascularization]]
|-
|[[Cavernous angioma|Cavernomas Aneurysms of the vein Galen]]
|
|
|-
|[[Metastasis|METASTASES]]
|<.,1%
|Absence of [[Blood-brain barrier|blood -]]
[[Blood-brain barrier|brain barrier]]
|-
|Lung (most common),breast,stomach,kidney,[[melanoma]]
| colspan="2" |
|}
 
==Pathophysiology==
*The exact [[pathogenesis]] of [[Extragonadal Germ Cell Tumors|extragonadal]] [[Germ cell tumor|germ cell tumors]] is not fully understood.<ref name="pmid31568647">{{cite journal| author=Ronchi A, Cozzolino I, Montella M, Panarese I, Zito Marino F, Rossetti S et al.| title=Extragonadal germ cell tumors: Not just a matter of location. A review about clinical, molecular and pathological features. | journal=Cancer Med | year= 2019 | volume=  | issue=  | pages=  | pmid=31568647 | doi=10.1002/cam4.2195 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31568647  }} </ref>
*It is thought that [[extragonadal germ cell tumors]]  is the result of arresting erroneously of precursos germ cells in midline migration during [[Embryonal carcinoma|emberiogenesis]].<ref name="pmid31568647">{{cite journal| author=Ronchi A, Cozzolino I, Montella M, Panarese I, Zito Marino F, Rossetti S et al.| title=Extragonadal germ cell tumors: Not just a matter of location. A review about clinical, molecular and pathological features. | journal=Cancer Med | year= 2019 | volume=  | issue=  | pages=  | pmid=31568647 | doi=10.1002/cam4.2195 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31568647  }} </ref>
*The other theory is that [[Epigallocatechin gallate|EGGCTs]] are metastases of undiagnosed primary [[GCTs]]. <ref name="pmid31568647">{{cite journal| author=Ronchi A, Cozzolino I, Montella M, Panarese I, Zito Marino F, Rossetti S et al.| title=Extragonadal germ cell tumors: Not just a matter of location. A review about clinical, molecular and pathological features. | journal=Cancer Med | year= 2019 | volume=  | issue=  | pages=  | pmid=31568647 | doi=10.1002/cam4.2195 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31568647  }} </ref>
 
==Causes==
*The [[Intracranial Bleeding|intracranial GCTs]] are resulting of many mutations of [[MAPK/ERK pathway|MAPK]]/[[PI3K]]/[[mTOR]] pathway.<ref name="pmid31420671">{{cite journal| author=Takami H, Fukuoka K, Fukushima S, Nakamura T, Mukasa A, Saito N et al.| title=Integrated Clinical, Histopathological, and Molecular Data Analysis of 190 Central Nervous System Germ Cell Tumors from the iGCT Consortium. | journal=Neuro Oncol | year= 2019 | volume=  | issue=  | pages=  | pmid=31420671 | doi=10.1093/neuonc/noz139 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31420671  }} </ref>
 
==Differentiating Intracranial Germ cell Tumors from Other Diseases==
*[[Intra-abdominal abscess|Intracranial germcell tumors]] must be differentiated from other diseases that cause [[Compressive myelopathies|compressive]] [[syndrome]] ,rise of [[intracranial pressure]] , such as other brain tumors and every disease who can rise [[ICP]].<ref name="pmid23640020">{{cite journal| author=Fang AS, Meyers SP| title=Magnetic resonance imaging of pineal region tumours. | journal=Insights Imaging | year= 2013 | volume= 4 | issue= 3 | pages= 369-82 | pmid=23640020 | doi=10.1007/s13244-013-0248-6 | pmc=3675249 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23640020  }} </ref>
*[[Primary peritoneal cancer|Extra gonadal Germ cell tumors]] should be in differential diagnosis of [[Pineal body|pineal]] [[Germ cell neoplasm|germ cell]] tumors.<ref name="pmid23559987">{{cite journal| author=Mufti ST, Jamal A| title=Primary intracranial germ cell tumors. | journal=Asian J Neurosurg | year= 2012 | volume= 7 | issue= 4 | pages= 197-202 | pmid=23559987 | doi=10.4103/1793-5482.106652 | pmc=3613642 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23559987  }} </ref>
 
==Epidemiology and Demographics==
*The incidence of [[Pineal body|pineal]] tumor is approximately [1%] of all [[Intraclated cell|intracranial tumors]].<ref name="pmid31441839">{{cite journal| author=Ji J, Gu C, Zhang M, Zhang H, Wang H, Qu Y et al.| title=Pineal region metastasis with intraventricular seeding: A case report and literature review. | journal=Medicine (Baltimore) | year= 2019 | volume= 98 | issue= 34 | pages= e16652 | pmid=31441839 | doi=10.1097/MD.0000000000016652 | pmc=6716749 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31441839  }} </ref>
*The incidence of [[Pineal gland|pineal]] tumor is 0,06 -0,07 per 100,000 persons per year.<ref name="pmid31441839">{{cite journal| author=Ji J, Gu C, Zhang M, Zhang H, Wang H, Qu Y et al.| title=Pineal region metastasis with intraventricular seeding: A case report and literature review. | journal=Medicine (Baltimore) | year= 2019 | volume= 98 | issue= 34 | pages= e16652 | pmid=31441839 | doi=10.1097/MD.0000000000016652 | pmc=6716749 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31441839  }} </ref>
*Patients of all age groups may develop [[Pineal gland|pineal]] yolk sac tumor,But is more common in children(3-8%)and also in  Japenes population.<ref name="pmid31441839">{{cite journal| author=Ji J, Gu C, Zhang M, Zhang H, Wang H, Qu Y et al.| title=Pineal region metastasis with intraventricular seeding: A case report and literature review. | journal=Medicine (Baltimore) | year= 2019 | volume= 98 | issue= 34 | pages= e16652 | pmid=31441839 | doi=10.1097/MD.0000000000016652 | pmc=6716749 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31441839  }} </ref>
*There is no racial predilection to [[Pineal gland|pineal]] tumors.<ref name="pmid31441839">{{cite journal| author=Ji J, Gu C, Zhang M, Zhang H, Wang H, Qu Y et al.| title=Pineal region metastasis with intraventricular seeding: A case report and literature review. | journal=Medicine (Baltimore) | year= 2019 | volume= 98 | issue= 34 | pages= e16652 | pmid=31441839 | doi=10.1097/MD.0000000000016652 | pmc=6716749 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31441839  }} </ref>
*[[Pineal gland|pineal]] tumors affect men more than women.<ref name="pmid19373436">{{cite journal| author=Al-Hussaini M, Sultan I, Abuirmileh N, Jaradat I, Qaddoumi I| title=Pineal gland tumors: experience from the SEER database. | journal=J Neurooncol | year= 2009 | volume= 94 | issue= 3 | pages= 351-8 | pmid=19373436 | doi=10.1007/s11060-009-9881-9 | pmc=2804886 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19373436  }} </ref>
*The majority of [[Pineal gland|pineal]] tumor cases are reported in Japenes population.<ref name="pmid31441839">{{cite journal| author=Ji J, Gu C, Zhang M, Zhang H, Wang H, Qu Y et al.| title=Pineal region metastasis with intraventricular seeding: A case report and literature review. | journal=Medicine (Baltimore) | year= 2019 | volume= 98 | issue= 34 | pages= e16652 | pmid=31441839 | doi=10.1097/MD.0000000000016652 | pmc=6716749 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31441839  }} </ref>
 
==Risk Factors==
*There are no established risk factors for [[Pineal gland|pineal]] yolk sac tumor.
 
==Screening==
*There is insufficient evidence to recommend routine screening for [[Pineal gland|pineal]] yolk sac tumors.
 
==Natural History, Complications, and Prognosis=
 
*The 5 year survival rate of patients with [[Intracranial germ cell tumor|intracranial germ cell tumors]] is approximately 80%.<ref name="pmid30271875">{{cite journal| author=Fetcko K, Dey M| title=Primary Central Nervous System Germ Cell Tumors: A Review and Update. | journal=Med Res Arch | year= 2018 | volume= 6 | issue= 3 | pages=  | pmid=30271875 | doi=10.18103/mra.v6i3.1719 | pmc=6157918 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30271875  }} </ref>
 
*We can classified [[Intercalary deletion|intracranial germ cell tumors]] to three main groups;  good,intermediate and bad.<ref name="pmid31470154">{{cite journal| author=Uda H, Uda T, Nakajo K, Tanoue Y, Okuno T, Koh S et al.| title=Adult-Onset Mixed Germ Cell Tumor Composed Mainly of Yolk Sac Tumor Around the Pineal Gland: A Case Report and Review of the Literature. | journal=World Neurosurg | year= 2019 | volume= 132 | issue=  | pages= 87-92 | pmid=31470154 | doi=10.1016/j.wneu.2019.08.079 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31470154  }} </ref>
*[[Pineal gland|pineal]] yolk sac tumor is in the poor prognosis classification.<ref name="pmid31470154">{{cite journal| author=Uda H, Uda T, Nakajo K, Tanoue Y, Okuno T, Koh S et al.| title=Adult-Onset Mixed Germ Cell Tumor Composed Mainly of Yolk Sac Tumor Around the Pineal Gland: A Case Report and Review of the Literature. | journal=World Neurosurg | year= 2019 | volume= 132 | issue=  | pages= 87-92 | pmid=31470154 | doi=10.1016/j.wneu.2019.08.079 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31470154  }} </ref>
*Factors that make poor prognosis is ;female ,age greater than 18,[[Non-AIDS associated Kaposi sarcoma|non germinoma GCTs]].<ref name="pmid30271875">{{cite journal| author=Fetcko K, Dey M| title=Primary Central Nervous System Germ Cell Tumors: A Review and Update. | journal=Med Res Arch | year= 2018 | volume= 6 | issue= 3 | pages=  | pmid=30271875 | doi=10.18103/mra.v6i3.1719 | pmc=6157918 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30271875  }} </ref>
== Diagnosis==
===Diagnostic Study of Choice===
*In 54% of cases diagnosis is delayed because of non-specific symptoms.<ref name="pmid30271875">{{cite journal| author=Fetcko K, Dey M| title=Primary Central Nervous System Germ Cell Tumors: A Review and Update. | journal=Med Res Arch | year= 2018 | volume= 6 | issue= 3 | pages=  | pmid=30271875 | doi=10.18103/mra.v6i3.1719 | pmc=6157918 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30271875  }} </ref>
 
*The diagnosis of [[Cranial Electrobiological Stimulation|cranial germ cell tumors]] is based on the CT,MRI with gadolinium fundings and level of [[biomarkers]]([[HCG]],[[AFP]])of serum and csf.also [[histology]] need for diffrentiate type of [[GCTs]].With normal [[Biomarkers of cardiac injury|biomarkers]] level ,for definitive diagnosis biopsy is requiered.<ref name="pmid30271875">{{cite journal| author=Fetcko K, Dey M| title=Primary Central Nervous System Germ Cell Tumors: A Review and Update. | journal=Med Res Arch | year= 2018 | volume= 6 | issue= 3 | pages=  | pmid=30271875 | doi=10.18103/mra.v6i3.1719 | pmc=6157918 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30271875  }} </ref>
*In addition we should determine [[Immunoblotting|Immuno]][[histo]]<nowiki/>chemichal markers for diagnosis([[c-kit]]/[[CD117]],oct3/4,PLAP).<ref name="pmid30271875">{{cite journal| author=Fetcko K, Dey M| title=Primary Central Nervous System Germ Cell Tumors: A Review and Update. | journal=Med Res Arch | year= 2018 | volume= 6 | issue= 3 | pages=  | pmid=30271875 | doi=10.18103/mra.v6i3.1719 | pmc=6157918 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30271875  }} </ref>
 
===History and Symptoms==
 
* The most common symptoms of [[Intracranial germ cell tumor|intracranial germ cell tumors]] include [[Parinaud syndrome|parinaud syn]] (because of hydrocephalus or commpresion of [[tectal]] plate ). Common symptoms of [[Intra-abdominal hypertension|intracranial]] GCTs include DI(due to [[hydrocephalus]]), [[ataxia]] and [[dysmetria]] if affecting superior [[cerebellar]] peduncles, precocious puberty,headache ,nausea,[[Occipital|ocolomotor]] or [[Facial|facia]]<nowiki/>l [[paresis]],[[seizure]].<ref name="pmid30271875">{{cite journal| author=Fetcko K, Dey M| title=Primary Central Nervous System Germ Cell Tumors: A Review and Update. | journal=Med Res Arch | year= 2018 | volume= 6 | issue= 3 | pages=  | pmid=30271875 | doi=10.18103/mra.v6i3.1719 | pmc=6157918 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=30271875  }} </ref><ref name="pmid23640020">{{cite journal| author=Fang AS, Meyers SP| title=Magnetic resonance imaging of pineal region tumours. | journal=Insights Imaging | year= 2013 | volume= 4 | issue= 3 | pages= 369-82 | pmid=23640020 | doi=10.1007/s13244-013-0248-6 | pmc=3675249 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23640020  }} </ref>
 
 
===Physical Examination ===
 
*Common physical examination findings of [[Intracranial Bleeding|intracranial GTCs]] include [[papillary]] [[edema]][[Gate control theory of pain|,gate disturbance]],
facial nerve parasia.<ref name="pmid23640020">{{cite journal| author=Fang AS, Meyers SP| title=Magnetic resonance imaging of pineal region tumours. | journal=Insights Imaging | year= 2013 | volume= 4 | issue= 3 | pages= 369-82 | pmid=23640020 | doi=10.1007/s13244-013-0248-6 | pmc=3675249 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23640020  }} </ref>
 
===Laboratory Findings===
*An elevated concentration of [[Serum-ascites albumin gradient|serum/CSF HCG,AFP]] is diagnostic of ''[[Germ cell tumor|Germ cell tumors]]''.<ref name="pmid23640020">{{cite journal| author=Fang AS, Meyers SP| title=Magnetic resonance imaging of pineal region tumours. | journal=Insights Imaging | year= 2013 | volume= 4 | issue= 3 | pages= 369-82 | pmid=23640020 | doi=10.1007/s13244-013-0248-6 | pmc=3675249 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23640020  }} </ref>
 
===Electrocardiogram===
*There are no [[ECG]] findings associated with [[Intercalary deletion|intracranial GCTs]].
 
===X-ray===
*There are no [[X-rays|x-ray]] findings associated with [[Pineal gland|pineal]] yolk sac tumors.
 
===Echocardiography or Ultrasound===
*There are no [[Echocardiography for diagnosis of atrial fibrillation|echocardiography]]/[[ultrasound]]  findings associated with [[Pineal body|Pineal]] yolk sac tumors.
 
===CT scan===
*Brain CT scan may be helpful in the diagnosis of [[Intracranial Bleeding|intracranial Germ cell tumors]]. Findings on CT scan suggestive of cranial yolk sac tumors include irregular mass,may have multi cysts, and solid component has calcifications,also in plain CT is [[Isoaminile|iso-low density]] mass with [[Heterogeneity|heterogenous enhancement]].<ref name="pmid7807172">{{cite journal| author=Fujimaki T, Matsutani M, Funada N, Kirino T, Takakura K, Nakamura O et al.| title=CT and MRI features of intracranial germ cell tumors. | journal=J Neurooncol | year= 1994 | volume= 19 | issue= 3 | pages= 217-26 | pmid=7807172 | doi=10.1007/bf01053275 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7807172  }} </ref>
.
 
===MRI==
 
*Brain [[MRI]][[T2 phage|T2]]-[[Based on Duration of Episode|based]] may be helpful in the diagnosis of cranial [[Germ|germ celltumors]]. Findings on [[MRI|MRI T2]] based diagnostic of [[Inter-ictal spiking|intracranial GCTs]] include [[Hyperinfection syndrome|hyperintense]] to normal paranchyma.<ref name="pmid29128947">{{cite journal| author=Morana G, Alves CA, Tortora D, Finlay JL, Severino M, Nozza P et al.| title=T2*-based MR imaging (gradient echo or susceptibility-weighted imaging) in midline and off-midline intracranial germ cell tumors: a pilot study. | journal=Neuroradiology | year= 2018 | volume= 60 | issue= 1 | pages= 89-99 | pmid=29128947 | doi=10.1007/s00234-017-1947-3 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29128947  }} </ref>
 
===Other Imaging Findings===
*There are no other imaging findings associated with [[Cranial|cranial GCTs.]]
 
===Other Diagnostic Studies==
 
*[[Immunohistochemical|Immunohistochemichal]] markers and [[Histology|histological]] features may be helpful in the diagnosis of [[Cranial|cranial GCTs]]. Findings suggestive of yolk sac tumor include reticular /[[microcytic]],psudopapillary/[[Endodermal sinus tumor|endodermal]] [[sinus]],[[glandular]] and [[solid]] patterns.<ref name="pmid31568647">{{cite journal| author=Ronchi A, Cozzolino I, Montella M, Panarese I, Zito Marino F, Rossetti S et al.| title=Extragonadal germ cell tumors: Not just a matter of location. A review about clinical, molecular and pathological features. | journal=Cancer Med | year= 2019 | volume=  | issue=  | pages=  | pmid=31568647 | doi=10.1002/cam4.2195 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31568647  }} </ref>
 
==Treatment==
*The mainstay of therapy for [[Pineal body|pineal]] yolk sac tumor is [[radiotherapy]] and/or [[chemotherapy]]. Sometimes, [[surgical resection]] may be done.<ref name="pmid17607429">{{cite journal| author=Davaus T, Gasparetto EL, Carvalho Neto Ad, Jung JE, Bleggi-Torres LF| title=Pineal yolk sac tumor: correlation between neuroimaging and pathological findings. | journal=Arq Neuropsiquiatr | year= 2007 | volume= 65 | issue= 2A | pages= 283-5 | pmid=17607429 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17607429  }} </ref>
{| 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|pineal]] tumors is uncertain.
 
*[[CSF|CSF diversion]] ([[Ventriculoperitoneal shunt|ventriculoperitoneal]] [VP] shunt or third [[ventriculostomy]] may be necessary in symptomatic patients, although [[Debulking|debulking surgery]] may obviate the need for this procedure
 
* When [[CSF|CSF diversion]] is necessary, endoscopic third [[ventriculostomy]] can be carried out at the same time as the biopsy and is preferred over [[VP-16-213|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 [[Pineoblastoma|pineoblastomas.]]
* Indeed, for [[pineoblastomas]], gross total surgical resection appears to correlate with improved survival.
* Patients with symptomatic recurrent [[Pineocytoma|pineocytomas]] should also be considered for [[surgical resection]] of the lesion
|-
|'''Radiation'''
|
*[[Postoperative Alkaline Gastritis|Postoperative 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 [[CSDA|CSI]] compared with those who did not.
* The five-year survival rates were 86 and 49 percent for [[Pineocytoma|pineocytomas]] and [[Non-AIDS associated Kaposi sarcoma|non-pineocytoma PPTs]], respectively.
*[[Adjuvant treatment|Adjuvant RT]] is not universally recommended after gross total resection of a [[pineocytoma]]
|-
|'''Stereotactic radiosurgery'''
|
*[[Stereotactic surgery|Stereotactic radiosurgery]] (SRS) is emerging as a useful treatment alternative for [[Pineocytoma|pineocytomas]], although experience is limited.
* The precise radiation fields that are defined by MRI or CT-computerized treatment planning minimize damage to the surrounding brain, and the risks of general anesthesia and [[craniotomy]] are avoided.
 
*[[SRSV|SRS]] is increasingly being used to treat pineal region tumors, either as an additional therapy after [[Conventional X-ray generator|conventional treatments]] or as a primary treatment.
* Due to the low rate of side effects, [[IRS1|IRS]] may develop into an attractive alternative to [[microsurgery]] in de novo diagnosed pineocytomas. In malignant PPTs, [[IRS1|IRS]] may be routinely applied in a [[Multimodal integration|multimodality treatment]] schedule supplementary to conventional irradiation.
|-
|'''Chemotherapy as part of multimodality therapy'''
|
* The similarity of [[Pineoblastoma|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 [[CranioMaxilloFacial Trauma|craniospinal]] irradiation (CSI) are a major concern.
* The importance of [[radiation therapy]] as a component of the initial treatment of [[Supratentorial|supratentorial primitive neuroectodermal tumors (PNETs)]] is also supported by the German [[HIT-III|HIT-SKK87]] and [[HITT|HIT-SKK92]] protocols, as well as the Canadian pediatric brain tumor protocol
|}


==References==
==References==
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Latest revision as of 19:38, 18 October 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Niloofarsadaat Eshaghhosseiny, MD[2]Sujit Routray, M.D. [3]

Synonyms and keywords: Pineal yolk sac tumors; Pineal yolk sac tumour; Pineal yolk sac tumours; Pineal endodermal sinus tumor; Pineal endodermal sinus tumors; Pineal endodermal sinus tumour; Pineal endodermal sinus tumours; Pineal yolk sac carcinoma; Pineal yolk sac carcinomas; Pineal gland tumor; Germ cell tumor; Brain tumor

Overview

Pineal yolk sac tumor is a rare type of extra gonadal yolk sac tumor. They make up a small fraction of all intracranial germ cell tumors and an even small fraction of pineal masses overall.Pure pineal yolk sac tumors secrete AFP.On microscopic histopathological analysis, pineal yolk sac tumor is characterized by poorly differentiated endothelial-like, cuboidal, or columnar cells with prominent nucleoli and significant mitotic activity.Pineal yolk sac tumor is demonstrated by positivity to tumor markers such as AFP, cytokeratin, and AAT.In upto 50% of cases, these tumors co-exist with other germ cell tumors Pineal yolk sac tumor may be associated with Down syndrome.Common complication of pineal yolk sac tumor includes obstructive hydrocephalus.Prognosis of pineal yolk sac tumor is generally poor.Symptoms of pineal yolk sac tumor include headache, nausea, vomiting, weakness, confusion, and somnolence.Head CT scan and brain MRI may be helpful in the diagnosis of pineal yolk sac tumor.On head CT scan, pineal yolk sac tumor is characterized by a hypodense, heterogenous mass in the pineal region with signs of obstructive hydrocephalus.On brain MRI, pineal yolk sac tumor is characterized by hypointensity on T1-weighted images and hyperintensity on T2-weighted images.There may be enhancement after contrast administration.Biopsy is generally done to confirm the diagnosis of pineal yolk sac tumor.

Historical Perspective

  • Tumors of brainstaim as well as pineal gland,were unoperable until late of 20th century.[1]
  • Dr Cushing reported one of the first case in 1904.[1]
  • Dr Horsley in 1905 was the first who did direct surgical intervention,and in 1913 Dr Oppenhein and Krause resected pineal tumor sucssesfully.[1]
  • There are only two case Reports of pineal yolk sac tumor that are associated with Down's syndrome in English literature.[2]

Classification

Adapted from WHO:

TUMOR FREQUENCY ORIGIN
GERMCELL TOMURS 60% Rest of germ cells
Germinoma MATURE TERATOMAMATURE TERATOMATERATOMA with Malignant Transformstion Yolk sac tomur

(endodermal sinus tumor) Embryonal carcinoma Choriocarcinoma

PINEAL PARANCHIMAL TUMORS 30% pineal glandular tissue
pineocytoma (WHO grade ɪ ) pineal paranchymal tomur of intermediate diffrentiation(WHO grade ɪɪ or ɪɪɪ)

pineoblastoma(WHO grade ɪv) papillary tumor of pineal region

TOMURS OF SUPPORTIVE AND ADJUCENT STRUCTURES 10%
ASTROCYTOMAGlioma (glioblastoma or oligodendroglioma)Medulloepithelioma Glial cells
Ependymomachoroid plexus papilloma Ependymal lining
MENINGIOMA Arachnoid cells
HemangiomaHemangiopericytoma or

blastomaChemodectomaCraniopharyngioma

vascular cells
NON-NEOPLASTIC TUMOR LIKE CONDITIONS < 1%
Arachnoid cysts Arachnoid cells
Degenerative cysts(pineal cysts) Glial cells
Cysticercosis parasites
Arteriovenous malformations vascularization
Cavernomas Aneurysms of the vein Galen
METASTASES <.,1% Absence of blood -

brain barrier

Lung (most common),breast,stomach,kidney,melanoma

Pathophysiology

Causes

Differentiating Intracranial Germ cell Tumors from Other Diseases

Epidemiology and Demographics

  • The incidence of pineal tumor is approximately [1%] of all intracranial tumors.[3]
  • The incidence of pineal tumor is 0,06 -0,07 per 100,000 persons per year.[3]
  • Patients of all age groups may develop pineal yolk sac tumor,But is more common in children(3-8%)and also in Japenes population.[3]
  • There is no racial predilection to pineal tumors.[3]
  • pineal tumors affect men more than women.[8]
  • The majority of pineal tumor cases are reported in Japenes population.[3]

Risk Factors

  • There are no established risk factors for pineal yolk sac tumor.

Screening

  • There is insufficient evidence to recommend routine screening for pineal yolk sac tumors.

=Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

  • In 54% of cases diagnosis is delayed because of non-specific symptoms.[9]

=History and Symptoms


Physical Examination

facial nerve parasia.[6]

Laboratory Findings

Electrocardiogram

X-ray

  • There are no x-ray findings associated with pineal yolk sac tumors.

Echocardiography or Ultrasound

CT scan

.

=MRI

Other Imaging Findings

  • There are no other imaging findings associated with cranial GCTs.

=Other Diagnostic Studies

Treatment

Management Options of Penial Gland tumors
CSF diversion
  • The optimal surgical strategy to treat acute hydrocephalus in patients with pineal tumors is uncertain.
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.
  • Patients with symptomatic recurrent pineocytomas should also be considered for surgical resection of the lesion
Radiation
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-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 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 1.2 Shahinian H, Ra Y (2013). "Fully endoscopic resection of pineal region tumors". J Neurol Surg B Skull Base. 74 (3): 114–7. doi:10.1055/s-0033-1338165. PMC 3712663. PMID 24436899.
  2. ↑ 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.
  3. ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Ji J, Gu C, Zhang M, Zhang H, Wang H, Qu Y; et al. (2019). "Pineal region metastasis with intraventricular seeding: A case report and literature review". Medicine (Baltimore). 98 (34): e16652. doi:10.1097/MD.0000000000016652. PMC 6716749 Check |pmc= value (help). PMID 31441839.
  4. ↑ 4.0 4.1 4.2 4.3 Ronchi A, Cozzolino I, Montella M, Panarese I, Zito Marino F, Rossetti S; et al. (2019). "Extragonadal germ cell tumors: Not just a matter of location. A review about clinical, molecular and pathological features". Cancer Med. doi:10.1002/cam4.2195. PMID 31568647.
  5. ↑ Takami H, Fukuoka K, Fukushima S, Nakamura T, Mukasa A, Saito N; et al. (2019). "Integrated Clinical, Histopathological, and Molecular Data Analysis of 190 Central Nervous System Germ Cell Tumors from the iGCT Consortium". Neuro Oncol. doi:10.1093/neuonc/noz139. PMID 31420671.
  6. ↑ 6.0 6.1 6.2 6.3 Fang AS, Meyers SP (2013). "Magnetic resonance imaging of pineal region tumours". Insights Imaging. 4 (3): 369–82. doi:10.1007/s13244-013-0248-6. PMC 3675249. PMID 23640020.
  7. ↑ Mufti ST, Jamal A (2012). "Primary intracranial germ cell tumors". Asian J Neurosurg. 7 (4): 197–202. doi:10.4103/1793-5482.106652. PMC 3613642. PMID 23559987.
  8. ↑ Al-Hussaini M, Sultan I, Abuirmileh N, Jaradat I, Qaddoumi I (2009). "Pineal gland tumors: experience from the SEER database". J Neurooncol. 94 (3): 351–8. doi:10.1007/s11060-009-9881-9. PMC 2804886. PMID 19373436.
  9. ↑ 9.0 9.1 9.2 9.3 9.4 9.5 Fetcko K, Dey M (2018). "Primary Central Nervous System Germ Cell Tumors: A Review and Update". Med Res Arch. 6 (3). doi:10.18103/mra.v6i3.1719. PMC 6157918. PMID 30271875.
  10. ↑ 10.0 10.1 Uda H, Uda T, Nakajo K, Tanoue Y, Okuno T, Koh S; et al. (2019). "Adult-Onset Mixed Germ Cell Tumor Composed Mainly of Yolk Sac Tumor Around the Pineal Gland: A Case Report and Review of the Literature". World Neurosurg. 132: 87–92. doi:10.1016/j.wneu.2019.08.079. PMID 31470154.
  11. ↑ Fujimaki T, Matsutani M, Funada N, Kirino T, Takakura K, Nakamura O; et al. (1994). "CT and MRI features of intracranial germ cell tumors". J Neurooncol. 19 (3): 217–26. doi:10.1007/bf01053275. PMID 7807172.
  12. ↑ Morana G, Alves CA, Tortora D, Finlay JL, Severino M, Nozza P; et al. (2018). "T2*-based MR imaging (gradient echo or susceptibility-weighted imaging) in midline and off-midline intracranial germ cell tumors: a pilot study". Neuroradiology. 60 (1): 89–99. doi:10.1007/s00234-017-1947-3. PMID 29128947.
  13. ↑ Davaus T, Gasparetto EL, Carvalho Neto Ad, Jung JE, Bleggi-Torres LF (2007). "Pineal yolk sac tumor: correlation between neuroimaging and pathological findings". Arq Neuropsiquiatr. 65 (2A): 283–5. PMID 17607429.


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