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Papillary tumor of the pineal region

MedGen UID:
457445
•Concept ID:
C2985219
•
Neoplastic Process
Synonyms: Papillary tumor of pineal region; Papillary Tumor of the Pineal Region; papillary tumor of the pineal region; PTPR
SNOMED CT: Papillary tumor of the pineal region (450899004); Papillary tumor of pineal region (1156472008)
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)
 
Monarch Initiative: MONDO:0016724
Orphanet: ORPHA251915

Definition

Papillary tumor of the pineal region (PTPR) is a very rare neoplasm of the pineal region that is thought to arise from the specialized ependymocytes of the subcommissural organ and that manifests with visual disturbances, headaches, loss of coordination and balance, nausea and vomiting due to obstructive hydrocephalus. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Papillary tumor of the pineal region
Follow this link to review classifications for Papillary tumor of the pineal region in Orphanet.

Professional guidelines

PubMed

Consensus guidelines for the management of pineal region tumours for low- and middle-income countries.
Bakhshi SK, Mushtaq N, Tariq R, Tariq M, Hafiz A, Gilani A, Aziz HF, Shamim MS, Laghari AA, Siddiqui K, Enam SA; Pakistan Brain Tumour Consortium
J Pak Med Assoc 2024 Mar;74(3 (Supple-3)):S135-S144. doi: 10.47391/JPMA.S3.GNO-16. PMID: 39262074
Papillary Tumors of Pineal Region: A Single-Center Experience in Management of 11 Cases.
Bora S, Santhoor HA, Kumar A, Das S, Sharma MC, Mishra S, Singh PK, Laythalling RK, Kale SS
World Neurosurg 2024 Apr;184:e486-e493. Epub 2024 Feb 1 doi: 10.1016/j.wneu.2024.01.149. PMID: 38307196
Results of the treatment of pineal tumors in children: the Lyon experience.
Szathmari A, Beuriat PA, Vasiljevic A, Leblond P, Faure-Conter C, Claude L, Di Rocco F, Mottolese C
Childs Nerv Syst 2023 Sep;39(9):2317-2327. Epub 2022 Oct 15 doi: 10.1007/s00381-022-05649-7. PMID: 36242638

Recent clinical studies

Etiology

Papillary tumor of the pineal region: analysis of DNA methylation profiles and clinical outcomes in 76 cases.
Wu Z, Dazelle K, Abdullaev Z, Chung HJ, Dahiya S, Wood M, Lee H, Lucas CG, Mao Q, Robinson L, Fernandes I, McCord M, Pytel P, Conway KS, Yoda R, Eschbacher JM, Maher OM, Hasselblatt M, Mobley BC, Raisanen JM, Hatanpaa KJ, Byers J, Lehman NL, Cimino PJ, Pratt D, Quezado M, Aldape K
Acta Neuropathol Commun 2024 Jul 16;12(1):117. doi: 10.1186/s40478-024-01781-4. PMID: 39014393Free PMC Article
Pineal Parenchymal Tumor of Intermediate Differentiation (PPTID) and Papillary Tumor of Pineal Region (PTPR): A Review.
Sadashiva N, Deora H, Arumalla K, Rao S, Saini J, Shukla D, Santosh V
Neurol India 2021 Sep-Oct;69(5):1153-1164. doi: 10.4103/0028-3886.329550. PMID: 34747779
Papillary Tumor of the Pineal Region: A Distinct Molecular Entity.
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
Brain Pathol 2016 Mar;26(2):199-205. Epub 2015 Jul 30 doi: 10.1111/bpa.12282. PMID: 26113311Free PMC Article
Outcomes After Surgery and Radiotherapy for Papillary Tumor of the Pineal Region.
Edson MA, Fuller GN, Allen PK, Levine NB, Ghia AJ, Mahajan A, Brown PD, DeMonte F, Li J
World Neurosurg 2015 Jul;84(1):76-81. Epub 2015 Mar 5 doi: 10.1016/j.wneu.2015.02.031. PMID: 25749579
Pineal tumors.
Dahiya S, Perry A
Adv Anat Pathol 2010 Nov;17(6):419-27. doi: 10.1097/PAP.0b013e3181f895a4. PMID: 20966647

Diagnosis

Papillary tumor of the pineal region in pediatric populations: An additional case and systematic review of a rare tumor entity.
Mathkour M, Hanna J, Ibrahim N, Scullen T, Kilgore MD, Werner C, Cormier I, Spencer P, Keen JR, Bui CJ
Clin Neurol Neurosurg 2021 Feb;201:106404. Epub 2020 Dec 8 doi: 10.1016/j.clineuro.2020.106404. PMID: 33360024
Papillary tumor of the pineal region: Case report and review of the literature.
Cañizares Méndez MLÁ, Amosa Delgado M, Álvarez Salgado JA, Villaseñor Ledezma JJ, Capilla Cabezuelo E, Díaz Crespo F
Neurocirugia (Astur : Engl Ed) 2019 Jan-Feb;30(1):38-43. Epub 2018 Apr 22 doi: 10.1016/j.neucir.2018.03.003. PMID: 29691144
Outcomes After Surgery and Radiotherapy for Papillary Tumor of the Pineal Region.
Edson MA, Fuller GN, Allen PK, Levine NB, Ghia AJ, Mahajan A, Brown PD, DeMonte F, Li J
World Neurosurg 2015 Jul;84(1):76-81. Epub 2015 Mar 5 doi: 10.1016/j.wneu.2015.02.031. PMID: 25749579
Papillary tumor of the pineal region: Histopathological characterization and review of the literature.
Fèvre Montange M, Vasiljevic A, Champier J, Jouvet A
Neurochirurgie 2015 Apr-Jun;61(2-3):138-42. Epub 2014 Feb 18 doi: 10.1016/j.neuchi.2013.04.011. PMID: 24556386
Pineal tumors.
Dahiya S, Perry A
Adv Anat Pathol 2010 Nov;17(6):419-27. doi: 10.1097/PAP.0b013e3181f895a4. PMID: 20966647

Therapy

PTEN R130Q Papillary Tumor of the Pineal Region (PTPR) with Chromosome 10 Loss Successfully Treated with Everolimus: A Case Report.
Assi HI, Kakati RT, Berro J, Saikali I, Youssef B, Hourany R, Alameh I, Tabbarah A, Khoury J, Darwish H, Alame S
Curr Oncol 2021 Mar 20;28(2):1274-1279. doi: 10.3390/curroncol28020121. PMID: 33804593Free PMC Article
Adjuvant temozolomide chemotherapy for treatment of papillary tumor of the pineal region.
Lorenzetti M, Motta F, Campanella R, Bauer D, Assi A, Arienta C, Gaini SM, Caroli M
World Neurosurg 2011 Jul-Aug;76(1-2):160-3. doi: 10.1016/j.wneu.2010.10.039. PMID: 21839968
Papillary tumor of the pineal region in a 15-month-old boy.
Li J, Recinos PF, Orr BA, Burger PC, Jallo GI, Recinos VR
J Neurosurg Pediatr 2011 May;7(5):534-8. doi: 10.3171/2011.2.PEDS10434. PMID: 21529195Free PMC Article
Successful treatment of neoadjuvant therapy for papillary tumor of the pineal region.
Nakamura H, Makino K, Kochi M, Nakazato Y, Kuratsu J
Brain Tumor Pathol 2009;26(2):73-7. Epub 2009 Oct 27 doi: 10.1007/s10014-009-0250-3. PMID: 19856218
Papillary tumor of the spinal cord: report of 2 cases.
Mobley B, Kalani MY, Harsh GR 4th, Edwards MS, Vogel H
Am J Surg Pathol 2009 Aug;33(8):1191-7. doi: 10.1097/PAS.0b013e31819c45ca. PMID: 19417584

Prognosis

Pineal Parenchymal Tumor of Intermediate Differentiation (PPTID) and Papillary Tumor of Pineal Region (PTPR): A Review.
Sadashiva N, Deora H, Arumalla K, Rao S, Saini J, Shukla D, Santosh V
Neurol India 2021 Sep-Oct;69(5):1153-1164. doi: 10.4103/0028-3886.329550. PMID: 34747779
Papillary Tumor of the Pineal Region: Systematic Review and Analysis of Prognostic Factors.
Yamaki VN, Solla DJF, Ribeiro RR, da Silva SA, Teixeira MJ, Figueiredo EG
Neurosurgery 2019 Sep 1;85(3):E420-E429. doi: 10.1093/neuros/nyz062. PMID: 30989225
Papillary tumor of the pineal region: Case report and review of the literature.
Cañizares Méndez MLÁ, Amosa Delgado M, Álvarez Salgado JA, Villaseñor Ledezma JJ, Capilla Cabezuelo E, Díaz Crespo F
Neurocirugia (Astur : Engl Ed) 2019 Jan-Feb;30(1):38-43. Epub 2018 Apr 22 doi: 10.1016/j.neucir.2018.03.003. PMID: 29691144
Papillary Tumor of the Pineal Region: A Distinct Molecular Entity.
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
Brain Pathol 2016 Mar;26(2):199-205. Epub 2015 Jul 30 doi: 10.1111/bpa.12282. PMID: 26113311Free PMC Article
Outcomes After Surgery and Radiotherapy for Papillary Tumor of the Pineal Region.
Edson MA, Fuller GN, Allen PK, Levine NB, Ghia AJ, Mahajan A, Brown PD, DeMonte F, Li J
World Neurosurg 2015 Jul;84(1):76-81. Epub 2015 Mar 5 doi: 10.1016/j.wneu.2015.02.031. PMID: 25749579

Clinical prediction guides

PTEN R130Q Papillary Tumor of the Pineal Region (PTPR) with Chromosome 10 Loss Successfully Treated with Everolimus: A Case Report.
Assi HI, Kakati RT, Berro J, Saikali I, Youssef B, Hourany R, Alameh I, Tabbarah A, Khoury J, Darwish H, Alame S
Curr Oncol 2021 Mar 20;28(2):1274-1279. doi: 10.3390/curroncol28020121. PMID: 33804593Free PMC Article
Papillary Tumor of the Pineal Region with Parinaud Syndrome: A Case Report.
Damgacı L, Hayat B, Güreşçi S
Curr Med Imaging 2020;16(8):1044-1047. doi: 10.2174/1573405615666191111143716. PMID: 33081665
Papillary Tumor of the Pineal Region: Systematic Review and Analysis of Prognostic Factors.
Yamaki VN, Solla DJF, Ribeiro RR, da Silva SA, Teixeira MJ, Figueiredo EG
Neurosurgery 2019 Sep 1;85(3):E420-E429. doi: 10.1093/neuros/nyz062. PMID: 30989225
Papillary tumor of the pineal region: a case involving isocitrate dehydrogenase (IDH) genotyping.
Ishida A, Shibuya M, Komori T, Nobusawa S, Niimura K, Matsuo S, Hori T
Brain Tumor Pathol 2013 Jan;30(1):45-9. Epub 2012 Mar 31 doi: 10.1007/s10014-012-0098-9. PMID: 22466620
Papillary tumor of the pineal region.
Boco T, Aalaei S, Musacchio M, Byrne R, Cochran E
Neuropathology 2008 Feb;28(1):87-92. Epub 2007 Dec 5 doi: 10.1111/j.1440-1789.2007.00832.x. PMID: 18069972

Recent systematic reviews

Papillary tumor of the pineal region in pediatric populations: An additional case and systematic review of a rare tumor entity.
Mathkour M, Hanna J, Ibrahim N, Scullen T, Kilgore MD, Werner C, Cormier I, Spencer P, Keen JR, Bui CJ
Clin Neurol Neurosurg 2021 Feb;201:106404. Epub 2020 Dec 8 doi: 10.1016/j.clineuro.2020.106404. PMID: 33360024
Radiotherapy for papillary tumor of the pineal region: A systematic review of the literature.
Lancia A, Becherini C, Detti B, Bottero M, Baki M, Cancelli A, Ferlosio A, Scoccianti S, Sun R, Livi L, Ingrosso G
Clin Neurol Neurosurg 2020 Mar;190:105646. Epub 2019 Dec 21 doi: 10.1016/j.clineuro.2019.105646. PMID: 31896492
Papillary Tumor of the Pineal Region: Systematic Review and Analysis of Prognostic Factors.
Yamaki VN, Solla DJF, Ribeiro RR, da Silva SA, Teixeira MJ, Figueiredo EG
Neurosurgery 2019 Sep 1;85(3):E420-E429. doi: 10.1093/neuros/nyz062. PMID: 30989225

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