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Pineoblastoma

MedGen UID:
104745
•Concept ID:
C0205898
•
Neoplastic Process
Synonym: Pineoblastomas
SNOMED CT: Pineoblastoma (767448007); Pineoblastoma (31671006)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
HPO: HP:0030408
Monarch Initiative: MONDO:0016722
Orphanet: ORPHA251909

Definition

Pineoblastoma is a rare primitive neuroectodermal tumor (PNET) arising in the pineal gland. Pineoblastomas are classified as a WHO grade IV tumor and comprise one-fourth to one-half of pineal parenchymal tumors. Pineoblastoma is a highly cellular tumor originating in the pineal gland and containing small, poorly differentiated cells. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPineoblastoma
Follow this link to review classifications for Pineoblastoma in Orphanet.

Professional guidelines

PubMed

Molecular Classification and Management of Rare Pediatric Embryonal Brain Tumors.
Sin-Chan P, Li BK, Ho B, Fonseca A, Huang A
Curr Oncol Rep 2018 Jul 11;20(9):69. doi: 10.1007/s11912-018-0717-7. PMID: 29995179
Role of radiosurgery in the management of pineal region tumours: indications, method, outcome.
Balossier A, Blond S, Touzet G, Sarrazin T, Lartigau E, Reyns N
Neurochirurgie 2015 Apr-Jun;61(2-3):216-22. Epub 2014 May 27 doi: 10.1016/j.neuchi.2013.11.007. PMID: 24874722
Contemporary management of pineoblastoma.
Tate MC, Rutkowski MJ, Parsa AT
Neurosurg Clin N Am 2011 Jul;22(3):409-12, ix. Epub 2011 Jun 16 doi: 10.1016/j.nec.2011.05.001. PMID: 21801990

Recent clinical studies

Etiology

Asynchronous pineoblastoma is more likely after early diagnosis of retinoblastoma: a meta-analysis.
de Jong MC, Shaikh F, Gallie B, Kors WA, Jansen RW, Dommering C, de Graaf P, Moll AC, Dimaras H, Shroff M, Kivelä TT, Soliman SE
Acta Ophthalmol 2022 Feb;100(1):e47-e52. Epub 2021 May 3 doi: 10.1111/aos.14855. PMID: 33939299Free PMC Article
DICER1 Syndrome.
Hořínová V, Drábová K, Nosková H, Bajčiová V, Šoukalová J, Černá L, Hůrková V, Slabý O, Štěrba J
Klin Onkol 2019 Summer;32(Supplementum2):123-127. doi: 10.14735/amko2019S123. PMID: 31409088
DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.
Schultz KAP, Williams GM, Kamihara J, Stewart DR, Harris AK, Bauer AJ, Turner J, Shah R, Schneider K, Schneider KW, Carr AG, Harney LA, Baldinger S, Frazier AL, Orbach D, Schneider DT, Malkin D, Dehner LP, Messinger YH, Hill DA
Clin Cancer Res 2018 May 15;24(10):2251-2261. Epub 2018 Jan 17 doi: 10.1158/1078-0432.CCR-17-3089. PMID: 29343557Free PMC Article
The long-term postsurgical prognosis of patients with pineoblastoma.
Tate M, Sughrue ME, Rutkowski MJ, Kane AJ, Aranda D, McClinton L, McClinton L, Barani IJ, Parsa AT
Cancer 2012 Jan 1;118(1):173-9. Epub 2011 Jun 29 doi: 10.1002/cncr.26300. PMID: 21717450
Pineal tumors.
Linggood RM, Chapman PH
J Neurooncol 1992 Jan;12(1):85-91. doi: 10.1007/BF00172460. PMID: 1541982

Diagnosis

Next-generation sequencing of cerebrospinal fluid for clinical molecular diagnostics in pediatric, adolescent and young adult brain tumor patients.
Miller AM, Szalontay L, Bouvier N, Hill K, Ahmad H, Rafailov J, Lee AJ, Rodriguez-Sanchez MI, Yildirim O, Patel A, Bale TA, Benhamida JK, Benayed R, Arcila ME, Donzelli M, Dunkel IJ, Gilheeney SW, Khakoo Y, Kramer K, Sait SF, Greenfield JP, Souweidane MM, Haque S, Mauguen A, Berger MF, Mellinghoff IK, Karajannis MA
Neuro Oncol 2022 Oct 3;24(10):1763-1772. doi: 10.1093/neuonc/noac035. PMID: 35148412Free PMC Article
Asynchronous pineoblastoma is more likely after early diagnosis of retinoblastoma: a meta-analysis.
de Jong MC, Shaikh F, Gallie B, Kors WA, Jansen RW, Dommering C, de Graaf P, Moll AC, Dimaras H, Shroff M, Kivelä TT, Soliman SE
Acta Ophthalmol 2022 Feb;100(1):e47-e52. Epub 2021 May 3 doi: 10.1111/aos.14855. PMID: 33939299Free PMC Article
Pediatric embryonal brain tumors in the molecular era.
Li BK, Al-Karmi S, Huang A, Bouffet E
Expert Rev Mol Diagn 2020 Mar;20(3):293-303. Epub 2020 Jan 15 doi: 10.1080/14737159.2020.1714439. PMID: 31917601
DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.
Schultz KAP, Williams GM, Kamihara J, Stewart DR, Harris AK, Bauer AJ, Turner J, Shah R, Schneider K, Schneider KW, Carr AG, Harney LA, Baldinger S, Frazier AL, Orbach D, Schneider DT, Malkin D, Dehner LP, Messinger YH, Hill DA
Clin Cancer Res 2018 May 15;24(10):2251-2261. Epub 2018 Jan 17 doi: 10.1158/1078-0432.CCR-17-3089. PMID: 29343557Free PMC Article
Pineal tumors.
Linggood RM, Chapman PH
J Neurooncol 1992 Jan;12(1):85-91. doi: 10.1007/BF00172460. PMID: 1541982

Therapy

Current Open Trials and Molecular Update for Pediatric Embryonal Tumors.
Rosenberg T, Cooney T
Pediatr Neurosurg 2023;58(5):299-306. Epub 2023 May 26 doi: 10.1159/000531256. PMID: 37245504
Prognostic factors and outcome of pineoblastoma: 10 years single-center experience.
Elhemaly A, Zaghloul MS, Ahmed S, Taha H, Refaat A, Maher E, El-Beltagy M, Zekry W
J Egypt Natl Canc Inst 2021 Sep 20;33(1):26. doi: 10.1186/s43046-021-00083-3. PMID: 34541625
Embryonal Tumors of the Central Nervous System: An Update.
Blessing MM, Alexandrescu S
Surg Pathol Clin 2020 Jun;13(2):235-247. Epub 2020 Apr 7 doi: 10.1016/j.path.2020.01.003. PMID: 32389264
Risk-adapted therapy and biological heterogeneity in pineoblastoma: integrated clinico-pathological analysis from the prospective, multi-center SJMB03 and SJYC07 trials.
Liu APY, Gudenas B, Lin T, Orr BA, Klimo P Jr, Kumar R, Bouffet E, Gururangan S, Crawford JR, Kellie SJ, Chintagumpala M, Fisher MJ, Bowers DC, Hassall T, Indelicato DJ, Onar-Thomas A, Ellison DW, Boop FA, Merchant TE, Robinson GW, Northcott PA, Gajjar A
Acta Neuropathol 2020 Feb;139(2):259-271. Epub 2019 Dec 4 doi: 10.1007/s00401-019-02106-9. PMID: 31802236Free PMC Article
Trilateral retinoblastoma.
Antoneli CB, Ribeiro Kde C, Sakamoto LH, Chojniak MM, Novaes PE, Arias VE
Pediatr Blood Cancer 2007 Mar;48(3):306-10. doi: 10.1002/pbc.20793. PMID: 16572402

Prognosis

Next-generation sequencing of cerebrospinal fluid for clinical molecular diagnostics in pediatric, adolescent and young adult brain tumor patients.
Miller AM, Szalontay L, Bouvier N, Hill K, Ahmad H, Rafailov J, Lee AJ, Rodriguez-Sanchez MI, Yildirim O, Patel A, Bale TA, Benhamida JK, Benayed R, Arcila ME, Donzelli M, Dunkel IJ, Gilheeney SW, Khakoo Y, Kramer K, Sait SF, Greenfield JP, Souweidane MM, Haque S, Mauguen A, Berger MF, Mellinghoff IK, Karajannis MA
Neuro Oncol 2022 Oct 3;24(10):1763-1772. doi: 10.1093/neuonc/noac035. PMID: 35148412Free PMC Article
DICER1 tumor predisposition syndrome: an evolving story initiated with the pleuropulmonary blastoma.
González IA, Stewart DR, Schultz KAP, Field AP, Hill DA, Dehner LP
Mod Pathol 2022 Jan;35(1):4-22. Epub 2021 Oct 1 doi: 10.1038/s41379-021-00905-8. PMID: 34599283Free PMC Article
SWI/SNF deficient central nervous system neoplasms.
Cai C
Semin Diagn Pathol 2021 May;38(3):167-174. Epub 2021 Mar 15 doi: 10.1053/j.semdp.2021.03.003. PMID: 33762087
The long-term postsurgical prognosis of patients with pineoblastoma.
Tate M, Sughrue ME, Rutkowski MJ, Kane AJ, Aranda D, McClinton L, McClinton L, Barani IJ, Parsa AT
Cancer 2012 Jan 1;118(1):173-9. Epub 2011 Jun 29 doi: 10.1002/cncr.26300. PMID: 21717450
Pineal region tumors in children.
Dhall G, Khatua S, Finlay JL
Curr Opin Neurol 2010 Dec;23(6):576-82. doi: 10.1097/WCO.0b013e3283404ef1. PMID: 21042218

Clinical prediction guides

DICER1 Syndrome.
Hořínová V, Drábová K, Nosková H, Bajčiová V, Šoukalová J, Černá L, Hůrková V, Slabý O, Štěrba J
Klin Onkol 2019 Summer;32(Supplementum2):123-127. doi: 10.14735/amko2019S123. PMID: 31409088
Retinoblastoma, the visible CNS tumor: A review.
Dimaras H, Corson TW
J Neurosci Res 2019 Jan;97(1):29-44. Epub 2018 Jan 3 doi: 10.1002/jnr.24213. PMID: 29314142Free PMC Article
The long-term postsurgical prognosis of patients with pineoblastoma.
Tate M, Sughrue ME, Rutkowski MJ, Kane AJ, Aranda D, McClinton L, McClinton L, Barani IJ, Parsa AT
Cancer 2012 Jan 1;118(1):173-9. Epub 2011 Jun 29 doi: 10.1002/cncr.26300. PMID: 21717450
Pathology of pineal parenchymal tumors.
Sato K, Kubota T
Prog Neurol Surg 2009;23:12-25. Epub 2009 Mar 23 doi: 10.1159/000210050. PMID: 19329858
Pathology of pineal region tumors.
Hirato J, Nakazato Y
J Neurooncol 2001 Sep;54(3):239-49. doi: 10.1023/a:1012721723387. PMID: 11767290

Recent systematic reviews

Primary Gamma Knife Radiosurgery for pineal region tumors: A systematic review and pooled analysis of available literature with histological stratification.
Gagliardi F, De Domenico P, Garbin E, Snider S, Mortini P
J Pineal Res 2023 Dec;75(4):e12910. Epub 2023 Sep 13 doi: 10.1111/jpi.12910. PMID: 37705383
Is there a role for high dose chemotherapy with hematopoietic stem cell rescue in patients with relapsed supratentorial PNET?
Raghuram CP, Moreno L, Zacharoulis S
J Neurooncol 2012 Feb;106(3):441-7. Epub 2011 Aug 18 doi: 10.1007/s11060-011-0690-6. PMID: 21850536

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