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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

Pineal/germ cell tumors and pineal parenchymal tumors.
Ogiwara H, Liao YM, Wong TT
Childs Nerv Syst 2023 Oct;39(10):2649-2665. Epub 2023 Oct 13 doi: 10.1007/s00381-023-06081-1. PMID: 37831207
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 separation of pineocytoma from pineoblastoma.
Borit A, Blackwood W, Mair WG
Cancer 1980 Mar 15;45(6):1408-18. doi: 10.1002/1097-0142(19800315)45:6<1408::aid-cncr2820450619>3.0.co;2-0. PMID: 6986979

Diagnosis

Pineal/germ cell tumors and pineal parenchymal tumors.
Ogiwara H, Liao YM, Wong TT
Childs Nerv Syst 2023 Oct;39(10):2649-2665. Epub 2023 Oct 13 doi: 10.1007/s00381-023-06081-1. PMID: 37831207
The surgical intervention for pineal region tumors.
Kondo A, Suzuki M, Shimizu Y, Akiyama O
Childs Nerv Syst 2023 Sep;39(9):2341-2348. Epub 2023 Jul 12 doi: 10.1007/s00381-023-06071-3. PMID: 37436474
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 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 separation of pineocytoma from pineoblastoma.
Borit A, Blackwood W, Mair WG
Cancer 1980 Mar 15;45(6):1408-18. doi: 10.1002/1097-0142(19800315)45:6<1408::aid-cncr2820450619>3.0.co;2-0. PMID: 6986979

Therapy

The effectiveness and safety of proton beam radiation therapy in children and young adults with Central Nervous System (CNS) tumours: a systematic review.
Wilson JS, Main C, Thorp N, Taylor RE, Majothi S, Kearns PR, English M, Dandapani M, Phillips R, Wheatley K, Pizer B
J Neurooncol 2024 Mar;167(1):1-34. Epub 2024 Jan 31 doi: 10.1007/s11060-023-04510-4. PMID: 38294638Free PMC Article
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
Pediatric phase 2 trial of a WEE1 inhibitor, adavosertib (AZD1775), and irinotecan for relapsed neuroblastoma, medulloblastoma, and rhabdomyosarcoma.
Cole KA, Ijaz H, Surrey LF, Santi M, Liu X, Minard CG, Maris JM, Voss S, Reid JM, Fox E, Weigel BJ
Cancer 2023 Jul 15;129(14):2245-2255. Epub 2023 Apr 20 doi: 10.1002/cncr.34786. PMID: 37081608Free PMC Article
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
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

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
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
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
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
The separation of pineocytoma from pineoblastoma.
Borit A, Blackwood W, Mair WG
Cancer 1980 Mar 15;45(6):1408-18. doi: 10.1002/1097-0142(19800315)45:6<1408::aid-cncr2820450619>3.0.co;2-0. PMID: 6986979

Clinical prediction guides

Modeling germline mutations in pineoblastoma uncovers lysosome disruption-based therapy.
Chung PED, Gendoo DMA, Ghanbari-Azarnier R, Liu JC, Jiang Z, Tsui J, Wang DY, Xiao X, Li B, Dubuc A, Shih D, Remke M, Ho B, Garzia L, Ben-David Y, Kang SG, Croul S, Haibe-Kains B, Huang A, Taylor MD, Zacksenhaus E
Nat Commun 2020 Apr 14;11(1):1825. doi: 10.1038/s41467-020-15585-2. PMID: 32286280Free 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
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

Recent systematic reviews

The effectiveness and safety of proton beam radiation therapy in children and young adults with Central Nervous System (CNS) tumours: a systematic review.
Wilson JS, Main C, Thorp N, Taylor RE, Majothi S, Kearns PR, English M, Dandapani M, Phillips R, Wheatley K, Pizer B
J Neurooncol 2024 Mar;167(1):1-34. Epub 2024 Jan 31 doi: 10.1007/s11060-023-04510-4. PMID: 38294638Free PMC Article
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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