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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
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
Phase II treatment of medulloblastoma and pineoblastoma with melphalan: clinical therapy based on experimental models of human medulloblastoma.
Friedman HS, Schold SC Jr, Mahaley MS Jr, Colvin OM, Oakes WJ, Vick NA, Burger PC, Bigner SH, Borowitz M, Halperin EC
J Clin Oncol 1989 Jul;7(7):904-11. doi: 10.1200/JCO.1989.7.7.904. PMID: 2738624

Recent clinical studies

Etiology

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 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
Retinoblastoma and Neuroblastoma Predisposition and Surveillance.
Kamihara J, Bourdeaut F, Foulkes WD, Molenaar JJ, Mossé YP, Nakagawara A, Parareda A, Scollon SR, Schneider KW, Skalet AH, States LJ, Walsh MF, Diller LR, Brodeur GM
Clin Cancer Res 2017 Jul 1;23(13):e98-e106. doi: 10.1158/1078-0432.CCR-17-0652. PMID: 28674118Free 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

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
Retinoblastoma and Neuroblastoma Predisposition and Surveillance.
Kamihara J, Bourdeaut F, Foulkes WD, Molenaar JJ, Mossé YP, Nakagawara A, Parareda A, Scollon SR, Schneider KW, Skalet AH, States LJ, Walsh MF, Diller LR, Brodeur GM
Clin Cancer Res 2017 Jul 1;23(13):e98-e106. doi: 10.1158/1078-0432.CCR-17-0652. PMID: 28674118Free 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
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

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
Clinical and molecular heterogeneity of pineal parenchymal tumors: a consensus study.
Liu APY, Li BK, Pfaff E, Gudenas B, Vasiljevic A, Orr BA, Dufour C, Snuderl M, Karajannis MA, Rosenblum MK, Hwang EI, Ng HK, Hansford JR, Szathmari A, Faure-Conter C, Merchant TE, Levine M, Bouvier N, von Hoff K, Mynarek M, Rutkowski S, Sahm F, Kool M, Hawkins C, Onar-Thomas A, Robinson GW, Gajjar A, Pfister SM, Bouffet E, Northcott PA, Jones DTW, Huang A
Acta Neuropathol 2021 May;141(5):771-785. Epub 2021 Feb 22 doi: 10.1007/s00401-021-02284-5. PMID: 33619588Free 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

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

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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