Difference between revisions of "Pineal gland"

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Pineal gland (view source)

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==Overview==
==Overview==
Non-neoplastic:
* Cysts
Tumours:<ref name=pmid20971711>{{cite journal |author=Gaillard F, Jones J |title=Masses of the pineal region: clinical presentation and radiographic features |journal=Postgrad Med J |volume=86 |issue=1020 |pages=597–607 |year=2010 |month=October |pmid=20971711 |doi=10.1136/pgmj.2009.087460 |url=}}</ref>
Tumours:<ref name=pmid20971711>{{cite journal |author=Gaillard F, Jones J |title=Masses of the pineal region: clinical presentation and radiographic features |journal=Postgrad Med J |volume=86 |issue=1020 |pages=597–607 |year=2010 |month=October |pmid=20971711 |doi=10.1136/pgmj.2009.087460 |url=}}</ref>
*Primary pineal tumours ~15% of (pineal) tumours - benign to malignant:<ref name=pmid21057132>{{cite journal |author=Smith AB, Rushing EJ, Smirniotopoulos JG |title=From the archives of the AFIP: lesions of the pineal region: radiologic-pathologic correlation |journal=Radiographics |volume=30 |issue=7 |pages=2001–20 |year=2010 |month=November |pmid=21057132 |doi=10.1148/rg.307105131 |url=}}</ref>
*Primary pineal tumours ~15% of (pineal) tumours - benign to malignant:<ref name=pmid21057132>{{cite journal |author=Smith AB, Rushing EJ, Smirniotopoulos JG |title=From the archives of the AFIP: lesions of the pineal region: radiologic-pathologic correlation |journal=Radiographics |volume=30 |issue=7 |pages=2001–20 |year=2010 |month=November |pmid=21057132 |doi=10.1148/rg.307105131 |url=}}</ref>
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*Others:
*Others:
**Direct invasion/extension from surrounding structures (astrocytomas).
**Direct invasion/extension from surrounding structures (astrocytomas).
**Metastases.
**[[Metastases]].
**[[Lipoma]]s.
**[[Lipoma]]s.
**[[Meningioma]]s.
**[[Meningioma]]s.


==Primary pineal tumours==
==Pineal gland cyst==
Range from benign to malignant.
*Quite common. <ref name=pmid17885233> {{Cite journal  | last1 = Pu | first1 = Y. | last2 = Mahankali | first2 = S. | last3 = Hou | first3 = J. | last4 = Li | first4 = J. | last5 = Lancaster | first5 = JL. | last6 = Gao | first6 = JH. | last7 = Appelbaum | first7 = DE. | last8 = Fox | first8 = PT. | title = High prevalence of pineal cysts in healthy adults demonstrated by high-resolution, noncontrast brain MR imaging. | journal = AJNR Am J Neuroradiol | volume = 28 | issue = 9 | pages = 1706-9 | month = Oct | year = 2007 | doi = 10.3174/ajnr.A0656 | PMID = 17885233 }}</ref>
*large cysts can cause CSF obstruction. <ref>{{Cite journal  | last1 = Mena | first1 = H. | last2 = Armonda | first2 = RA. | last3 = Ribas | first3 = JL. | last4 = Ondra | first4 = SL. | last5 = Rushing | first5 = EJ. | title = Nonneoplastic pineal cysts: a clinicopathologic study of twenty-one cases. | journal = Ann Diagn Pathol | volume = 1 | issue = 1 | pages = 11-8 | month = Oct | year = 1997 | doi =  | PMID = 9869821 }}</ref>
 
<gallery>
File:Pinealiszyste.jpg | Pineal gland cyst found at autopsy. (WC/Marvin101)
File:Pineal_gland_cyst_low_mag_HE.jpg | Pineal gland cyst. HE, low mag. (WC/jensflorian)
File:Pineal_gland_cyst_highmagnification_HE.jpg | Pineal gland cyst. HE, higher mag. (WC/jensflorian)
</gallery>
 
 


==Pineocytoma==
==Pineocytoma==
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*Rosette = circular/flower-like arrangement of cells.
*Rosette = circular/flower-like arrangement of cells.


Images:
====Images====
*[http://commons.wikimedia.org/wiki/File:Pineocytoma_-_intermed_mag.jpg Pineocytoma - intermed. mag. (WC)].
<gallery>
*[http://commons.wikimedia.org/wiki/File:Pineocytoma_-_very_high_mag.jpg Pineocytoma - very high mag. (WC)].
Image:Pineocytoma_-_intermed_mag.jpg | Pineocytoma - intermed. mag. (WC)
Image:Pineocytoma_-_high_mag.jpg | Pineocytoma - high mag. (WC)
Image:Pineocytoma_-_very_high_mag.jpg | Pineocytoma - very high mag. (WC)
</gallery>
*www:
**[http://path.upmc.edu/cases/case157.html Pineocytoma with neuronal differentiation (upmc.edu)].


===IHC===
===IHC===
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*GFAP -ve.  
*GFAP -ve.  
**+ve in gliomas.
**+ve in gliomas.
*PLAP -ve.
*[[PLAP]] -ve.
**Usu. +ve in germ cell tumours.
**Usu. +ve in germ cell tumours.
*Ki-67.
*Ki-67.


Another ref.:<ref>URL: [http://www.springerlink.com/content/k4v88n6h6jknhp2t/fulltext.pdf http://www.springerlink.com/content/k4v88n6h6jknhp2t/fulltext.pdf]. Accessed on: 9 December 2010.</ref>
Another ref.:<ref>URL: [http://www.springerlink.com/content/k4v88n6h6jknhp2t/fulltext.pdf http://www.springerlink.com/content/k4v88n6h6jknhp2t/fulltext.pdf]. Accessed on: 9 December 2010.</ref>
==Pineal parenchymal tumor of intermediate differentiation==
===General===
*20% of all pineal tumors.
*Affects all ages.
*ICD-O: 9362/3
*No WHO grade yet, clinical behaviour corresponds to grade II/III<ref>{{Cite journal  | last1 = Jouvet | first1 = A. | last2 = Saint-Pierre | first2 = G. | last3 = Fauchon | first3 = F. | last4 = Privat | first4 = K. | last5 = Bouffet | first5 = E. | last6 = Ruchoux | first6 = MM. | last7 = Chauveinc | first7 = L. | last8 = Fèvre-Montange | first8 = M. | title = Pineal parenchymal tumors: a correlation of histological features with prognosis in 66 cases. | journal = Brain Pathol | volume = 10 | issue = 1 | pages = 49-60 | month = Jan | year = 2000 | doi =  | PMID = 10668895 }}</ref>
===Microscopic===
Features:<ref>{{Cite journal  | last1 = Fèvre-Montange | first1 = M. | last2 = Szathmari | first2 = A. | last3 = Champier | first3 = J. | last4 = Mokhtari | first4 = K. | last5 = Chrétien | first5 = F. | last6 = Coulon | first6 = A. | last7 = Figarella-Branger | first7 = D. | last8 = Polivka | first8 = M. | last9 = Varlet | first9 = P. | title = Pineocytoma and pineal parenchymal tumors of intermediate differentiation presenting cytologic pleomorphism: a multicenter study. | journal = Brain Pathol | volume = 18 | issue = 3 | pages = 354-9 | month = Jul | year = 2008 | doi = 10.1111/j.1750-3639.2008.00128.x | PMID = 18371183 }}</ref>
*High cellularity.
*Mild to moderate atypia.
*Mitoses.
*Usually no pinecytomatous rosettes.
*High pleomorphism possible.
===Molecular===
* Insertions in KBTBD4.<ref name="pmid31768671">{{cite journal |authors=Pfaff E, Aichmüller C, Sill M, Stichel D, Snuderl M, Karajannis MA, Schuhmann MU, Schittenhelm J, Hasselblatt M, Thomas C, Korshunov A, Rhizova M, Wittmann A, Kaufhold A, Iskar M, Ketteler P, Lohmann D, Orr BA, Ellison DW, von Hoff K, Mynarek M, Rutkowski S, Sahm F, von Deimling A, Lichter P, Kool M, Zapatka M, Pfister SM, Jones DTW |title=Molecular subgrouping of primary pineal parenchymal tumors reveals distinct subtypes correlated with clinical parameters and genetic alterations |journal=Acta Neuropathol |volume=139 |issue=2 |pages=243–257 |date=February 2020 |pmid=31768671 |doi=10.1007/s00401-019-02101-0 |url=}}</ref>
Images:
<gallery>
File:Pinealis parechymal tumor.jpg | PPID, Smear with plemorphic cells. (WC/jensflorian)
File:PPID intermed mag HE.jpg | PPID, HE intermed. mag. (WC/jensflorian)
File:PPID high mag mitoses.jpg | PPID, HE higher mag. (WC/jensflorian)
File:PPID high mag rosettes.jpg | PPID, HE, rosette-like growth pattern. (WC/jensflorian)
</gallery>
==Papillary tumour of pineal region (PTPR)==
===General===
*Very rare neuoepithelial tumor of adults.
*Proposed ICD-O: 9395/3
*No WHO grade yet, clinical behaviour corresponds to grade II/III<ref>{{Cite journal  | last1 = Fèvre-Montange | first1 = M. | last2 = Hasselblatt | first2 = M. | last3 = Figarella-Branger | first3 = D. | last4 = Chauveinc | first4 = L. | last5 = Champier | first5 = J. | last6 = Saint-Pierre | first6 = G. | last7 = Taillandier | first7 = L. | last8 = Coulon | first8 = A. | last9 = Paulus | first9 = W. | title = Prognosis and histopathologic features in papillary tumors of the pineal region: a retrospective multicenter study of 31 cases. | journal = J Neuropathol Exp Neurol | volume = 65 | issue = 10 | pages = 1004-11 | month = Oct | year = 2006 | doi = 10.1097/01.jnen.0000240462.80263.13 | PMID = 17021405 }}</ref>
Gross:
*Well circumscribed.
*Can be quite large (2-4cm).
*Macroscopy mimics pineocytoma.
<gallery>
File:PTPR MRI.jpg | PTPR MRI scan (WC/marvin101)
</gallery>
===Microscopic===
Features:<ref>{{Cite journal  | last1 = Heim | first1 = S. | last2 = Beschorner | first2 = R. | last3 = Mittelbronn | first3 = M. | last4 = Keyvani | first4 = K. | last5 = Riemenschneider | first5 = MJ. | last6 = Vajtai | first6 = I. | last7 = Hartmann | first7 = C. | last8 = Acker | first8 = T. | last9 = Blümcke | first9 = I. | title = Increased mitotic and proliferative activity are associated with worse prognosis in papillary tumors of the pineal region. | journal = Am J Surg Pathol | volume = 38 | issue = 1 | pages = 106-10 | month = Jan | year = 2014 | doi = 10.1097/PAS.0b013e31829e492d | PMID = 24121176 }}</ref>
*Papillary growth pattern.
*Dense areas exhibit ependymal features.
**Clear, vacuolated cytoplasm.
**Rosettes.
*Round to oval nuclei.
*Mitoses (0-10/HPF).
*Necroses.
*Hyalinized vessels.
===IHC===
* +ve for keratins (KL1, AE1/AE3, Cam5.2, CK18).
* GFAP and Synaptophysin focally +ve.
* S100+ve.
* EMA mostly -ve.
* NF and Kir 7.1-ve.
===Molecular===
* PTEN alterations. <ref name="pmid26113311">{{cite journal |authors=Heim S, Sill M, Jones DT, Vasiljevic A, Jouvet A, Fèvre-Montange M, Wesseling P, Beschorner R, Mittelbronn M, Kohlhof P, Hovestadt V, Johann P, Kool M, Pajtler KW, Korshunov A, Ruland V, Sperveslage J, Thomas C, Witt H, von Deimling A, Paulus W, Pfister SM, Capper D, Hasselblatt M |title=Papillary Tumor of the Pineal Region: A Distinct Molecular Entity |journal=Brain Pathol |volume=26 |issue=2 |pages=199–205 |date=March 2016 |pmid=26113311 |doi=10.1111/bpa.12282 |url=}}</ref>
*Chromosome 10 loss.
DDx:
[[Ependymoma]].
Images:
<gallery>
File:PTPR papillary tumor pinealis HE.jpg | PTPR H&E stain (WC/jensflorian)
File:PTPR KL1.jpg | PTPR CK KL1 (WC/marvin101)
File:PTPR papillary tumor pinealis CK18.jpg | PTPR CK18 IHC (WC/jensflorian)
File:PTPR EMA.jpg | PTPR EMA IHC (WC/marvin101)
File:PTPR Kir71.jpg | PTPR Kir 7.1 IHC (WC/marvin101)
</gallery>
==Pineoblastoma==
===General===
*Rare.
*Malignant.
*Males > females.
*Children & young adults.
*Corresponds to WHO IV (ICD-O: 9362/3).
*May be a manifestation of the [[DICER1 syndrome]].<ref name=pmid28960912>{{Cite journal  | last1 = van Engelen | first1 = K. | last2 = Villani | first2 = A. | last3 = Wasserman | first3 = JD. | last4 = Aronoff | first4 = L. | last5 = Greer | first5 = MC. | last6 = Tijerin Bueno | first6 = M. | last7 = Gallinger | first7 = B. | last8 = Kim | first8 = RH. | last9 = Grant | first9 = R. | title = DICER1 syndrome: Approach to testing and management at a large pediatric tertiary care center. | journal = Pediatr Blood Cancer | volume = 65 | issue = 1 | pages =  | month = Jan | year = 2018 | doi = 10.1002/pbc.26720 | PMID = 28960912 }}</ref>
===Microscopic===
Features:
*Hypercellular.
*Mitoses.
*Nuclear atypia.
*Homer-wright & Flexner-Winterstein rosettes
*Fleurettes.
===IHC===
*GFAP -ve/+ve.
*NF+ve.
*Synaptophysin+ve
*MIB-1: high.
===Molecular===
* DICER1 mutations.<ref name="pmid25022261">{{cite journal |authors=de Kock L, Sabbaghian N, Druker H, Weber E, Hamel N, Miller S, Choong CS, Gottardo NG, Kees UR, Rednam SP, van Hest LP, Jongmans MC, Jhangiani S, Lupski JR, Zacharin M, Bouron-Dal Soglio D, Huang A, Priest JR, Perry A, Mueller S, Albrecht S, Malkin D, Grundy RG, Foulkes WD |title=Germ-line and somatic DICER1 mutations in pineoblastoma |journal=Acta Neuropathol |volume=128 |issue=4 |pages=583–95 |date=October 2014 |pmid=25022261 |pmc=4381868 |doi=10.1007/s00401-014-1318-7 |url=}}</ref>
*DROSHA deletions. <ref name="pmid30877433">{{cite journal |authors=Lee JC, Mazor T, Lao R, Wan E, Diallo AB, Hill NS, Thangaraj N, Wendelsdorf K, Samuel D, Kline CN, Banerjee A, Auguste K, Raffel C, Gupta N, Berger M, Raleigh DR, Shai A, Phillips JJ, Bollen AW, Tihan T, Perry A, Costello J, Solomon DA |title=Recurrent KBTBD4 small in-frame insertions and absence of DROSHA deletion or DICER1 mutation differentiate pineal parenchymal tumor of intermediate differentiation (PPTID) from pineoblastoma |journal=Acta Neuropathol |volume=137 |issue=5 |pages=851–854 |date=May 2019 |pmid=30877433 |pmc=6483828 |doi=10.1007/s00401-019-01990-5 |url=}}</ref>
DDx:
*[[Glioblastoma]], small cell variant.
*[[Small round cell tumors]].
Images:
*[http://path.upmc.edu/cases/case212.html Pineoblastoma - several images (upmc.edu)].
<gallery>
File:Pineoblastoma HE x200.jpg | Pineoblastoma H&E (WC/jensflorian)
File:Pineoblastoma neurofilament.jpg | Neurofilament IHC in Pineoblastoma (WC/jensflorian)
File:Pineoblastoma gfap.jpg | GFAP IHC in Pineoblastoma (WC/jensflorian)
</gallery>


==See also==
==See also==
Michael
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