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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

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVPineoblastoma

Pathological Conditions, Signs and Symptoms
- Pathological process
  - Disease
    - Neoplasm
      - Embryonal neoplasm
        Central Nervous System Embryonal Tumor
        Pineoblastoma
        Adult pineoblastoma
        Childhood Pineoblastoma
        Recurrent Childhood Pineoblastoma

Follow this link to review classifications for Pineoblastoma in Orphanet.

Professional guidelines

PubMed

Evaluation of age-dependent treatment strategies for children and young adults with pineoblastoma: analysis of pooled European Society for Paediatric Oncology (SIOP-E) and US Head Start data.

Mynarek M, Pizer B, Dufour C, van Vuurden D, Garami M, Massimino M, Fangusaro J, Davidson T, Gil-da-Costa MJ, Sterba J, Benesch M, Gerber N, Juhnke BO, Kwiecien R, Pietsch T, Kool M, Clifford S, Ellison DW, Giangaspero F, Wesseling P, Gilles F, Gottardo N, Finlay JL, Rutkowski S, von Hoff K
Neuro Oncol 2017 Apr 1;19(4):576-585. doi: 10.1093/neuonc/now234. PMID: 28011926 Free PMC Article

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

See all (28)

Recent clinical studies

Etiology

Histopathology and molecular pathology of pediatric pineal parenchymal tumors.

Vasiljevic A
Childs Nerv Syst 2023 Sep;39(9):2273-2284. Epub 2022 Aug 16 doi: 10.1007/s00381-022-05637-x. PMID: 35972537

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: 33939299 Free 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: 29343557 Free 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: 28674118 Free PMC Article

Pineal tumors.

Linggood RM, Chapman PH
J Neurooncol 1992 Jan;12(1):85-91. doi: 10.1007/BF00172460. PMID: 1541982

See all (157)

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

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: 35148412 Free PMC Article

Asynchronous pineoblastoma is more likely after early diagnosis of retinoblastoma: a meta-analysis.

DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.

See all (215)

Therapy

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: 37081608 Free PMC Article

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: 33619588 Free 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

See all (85)

Prognosis

Histopathology and molecular pathology of pediatric pineal parenchymal tumors.

Vasiljevic A
Childs Nerv Syst 2023 Sep;39(9):2273-2284. Epub 2022 Aug 16 doi: 10.1007/s00381-022-05637-x. PMID: 35972537

Next-generation sequencing of cerebrospinal fluid for clinical molecular diagnostics in pediatric, adolescent and young adult brain tumor patients.

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: 34599283 Free PMC Article

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

Pineal tumors.

Linggood RM, Chapman PH
J Neurooncol 1992 Jan;12(1):85-91. doi: 10.1007/BF00172460. PMID: 1541982

See all (150)

Clinical prediction guides

Patterns of Care and Age-Specific Impact of Extent of Resection and Adjuvant Radiotherapy in Pediatric Pineoblastoma.

Jin MC, Prolo LM, Wu A, Azad TD, Shi S, Rodrigues AJ, Soltys SG, Pollom EL, Li G, Hiniker SM, Grant GA
Neurosurgery 2020 May 1;86(5):E426-E435. doi: 10.1093/neuros/nyaa023. PMID: 32110805

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: 29314142 Free PMC Article

Pediatric imaging in DICER1 syndrome.

Bueno MT, Martínez-Ríos C, la Puente Gregorio A, Ahyad RA, Villani A, Druker H, van Engelen K, Gallinger B, Aronoff L, Grant R, Malkin D, Greer MC
Pediatr Radiol 2017 Sep;47(10):1292-1301. Epub 2017 May 4 doi: 10.1007/s00247-017-3875-0. PMID: 28474256

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

See all (76)