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Pancreatoblastoma

MedGen UID:
87250
•Concept ID:
C0334489
•
Neoplastic Process
Synonyms: pancreatoblastoma; pancreatoblastoma (morphologic abnormality); PB
SNOMED CT: Pancreatoblastoma (189814006); Pancreatoblastoma (53618008)
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:0100757
Monarch Initiative: MONDO:0019035
Orphanet: ORPHA677

Definition

A rare pediatric carcinoma of the pancreas. [from HPO]

Term Hierarchy

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

Professional guidelines

PubMed

Diagnosis, treatment, and prognosis of adult pancreatoblastoma.
Yuan J, Guo Y, Li Y
Cancer Med 2024 Aug;13(16):e70132. doi: 10.1002/cam4.70132. PMID: 39162366Free PMC Article
Treatment Strategies for Children With Relapsed Pancreatoblastoma: A Literature Review.
Reggiani G, Affinita MC, Dall'Igna P, Virgone C, Sorbara S, Bisogno G
J Pediatr Hematol Oncol 2021 Nov 1;43(8):288-293. doi: 10.1097/MPH.0000000000002033. PMID: 33323880
Differential diagnosis of solid pancreatic masses.
Iglesias-Garcia J, de la Iglesia-Garcia D, Olmos-Martinez JM, Lariño-Noia J, Dominguez-Muñoz JE
Minerva Gastroenterol Dietol 2020 Mar;66(1):70-81. Epub 2020 Jan 28 doi: 10.23736/S1121-421X.20.02646-X. PMID: 31994370

Recent clinical studies

Etiology

DNA Methylation Profiling Enables Accurate Classification of Nonductal Primary Pancreatic Neoplasms.
Verschuur AVD, Hackeng WM, Westerbeke F, Benhamida JK, Basturk O, Selenica P, Raicu GM, Molenaar IQ, van Santvoort HC, Daamen LA, Klimstra DS, Yachida S, Luchini C, Singhi AD, Geisenberger C, Brosens LAA
Clin Gastroenterol Hepatol 2024 Jun;22(6):1245-1254.e10. Epub 2024 Feb 20 doi: 10.1016/j.cgh.2024.02.007. PMID: 38382726
CT, MRI, and (18)F-FDG PET/CT imaging features of seven cases of adult pancreatoblastoma.
Wu M, Lin J, Liu Z, Huang Z, Wang R
BMC Med Imaging 2022 Dec 29;22(1):228. doi: 10.1186/s12880-022-00958-4. PMID: 36581821Free PMC Article
Non-Ductal Tumors of the Pancreas.
Dhillon J
Monogr Clin Cytol 2020;26:92-108. Epub 2020 Sep 28 doi: 10.1159/000455737. PMID: 32987393
Pancreatoblastoma in childhood: clinical course and therapeutic management of seven patients.
Défachelles AS, Martin De Lassalle E, Boutard P, Nelken B, Schneider P, Patte C
Med Pediatr Oncol 2001 Jul;37(1):47-52. doi: 10.1002/mpo.1162. PMID: 11466723
Pancreatoblastoma. A clinicopathologic study and review of the literature.
Klimstra DS, Wenig BM, Adair CF, Heffess CS
Am J Surg Pathol 1995 Dec;19(12):1371-89. doi: 10.1097/00000478-199512000-00005. PMID: 7503360

Diagnosis

Adult pancreatoblastoma: Current concepts in pathology.
Omiyale AO
World J Gastroenterol 2021 Jul 14;27(26):4172-4181. doi: 10.3748/wjg.v27.i26.4172. PMID: 34326617Free PMC Article
Malignant epithelial/exocrine tumors of the pancreas.
Luchini C, Grillo F, Fassan M, Vanoli A, Capelli P, Paolino G, Ingravallo G, Renzulli G, Doglioni C, D'Amuri A, Mattiolo P, Pecori S, Parente P, Florena AM, Zamboni G, Scarpa A
Pathologica 2020 Sep;112(3):210-226. doi: 10.32074/1591-951X-167. PMID: 33179623Free PMC Article
Non-Ductal Tumors of the Pancreas.
Dhillon J
Monogr Clin Cytol 2020;26:92-108. Epub 2020 Sep 28 doi: 10.1159/000455737. PMID: 32987393
Differential diagnosis of solid pancreatic masses.
Iglesias-Garcia J, de la Iglesia-Garcia D, Olmos-Martinez JM, Lariño-Noia J, Dominguez-Muñoz JE
Minerva Gastroenterol Dietol 2020 Mar;66(1):70-81. Epub 2020 Jan 28 doi: 10.23736/S1121-421X.20.02646-X. PMID: 31994370
Nonductal Pancreatic Cancers.
Jun SY, Hong SM
Surg Pathol Clin 2016 Dec;9(4):581-593. Epub 2016 Oct 12 doi: 10.1016/j.path.2016.05.005. PMID: 27926361

Therapy

DNA Methylation Profiling Enables Accurate Classification of Nonductal Primary Pancreatic Neoplasms.
Verschuur AVD, Hackeng WM, Westerbeke F, Benhamida JK, Basturk O, Selenica P, Raicu GM, Molenaar IQ, van Santvoort HC, Daamen LA, Klimstra DS, Yachida S, Luchini C, Singhi AD, Geisenberger C, Brosens LAA
Clin Gastroenterol Hepatol 2024 Jun;22(6):1245-1254.e10. Epub 2024 Feb 20 doi: 10.1016/j.cgh.2024.02.007. PMID: 38382726
Treatment Strategies for Children With Relapsed Pancreatoblastoma: A Literature Review.
Reggiani G, Affinita MC, Dall'Igna P, Virgone C, Sorbara S, Bisogno G
J Pediatr Hematol Oncol 2021 Nov 1;43(8):288-293. doi: 10.1097/MPH.0000000000002033. PMID: 33323880
Management of pancreatoblastoma in children and young adults.
Glick RD, Pashankar FD, Pappo A, Laquaglia MP
J Pediatr Hematol Oncol 2012 May;34 Suppl 2:S47-50. doi: 10.1097/MPH.0b013e31824e3839. PMID: 22525406
Diagnosis and treatment of pancreatoblastoma in China.
Cao L, Liu D
Pancreas 2007 Jan;34(1):92-5. doi: 10.1097/01.mpa.0000240610.33936.cb. PMID: 17198189
Pancreatoblastoma in childhood: clinical course and therapeutic management of seven patients.
Défachelles AS, Martin De Lassalle E, Boutard P, Nelken B, Schneider P, Patte C
Med Pediatr Oncol 2001 Jul;37(1):47-52. doi: 10.1002/mpo.1162. PMID: 11466723

Prognosis

Diagnosis, treatment, and prognosis of adult pancreatoblastoma.
Yuan J, Guo Y, Li Y
Cancer Med 2024 Aug;13(16):e70132. doi: 10.1002/cam4.70132. PMID: 39162366Free PMC Article
Could the burden of pancreatic cancer originate in childhood?
Diaconescu S, Gîlcă-Blanariu GE, Poamaneagra S, Marginean O, Paduraru G, Stefanescu G
World J Gastroenterol 2021 Aug 28;27(32):5322-5340. doi: 10.3748/wjg.v27.i32.5322. PMID: 34539135Free PMC Article
Treatment Strategies for Children With Relapsed Pancreatoblastoma: A Literature Review.
Reggiani G, Affinita MC, Dall'Igna P, Virgone C, Sorbara S, Bisogno G
J Pediatr Hematol Oncol 2021 Nov 1;43(8):288-293. doi: 10.1097/MPH.0000000000002033. PMID: 33323880
Pancreatoblastoma.
Saif MW
JOP 2007 Jan 9;8(1):55-63. PMID: 17228135
Pancreatoblastoma and solid and cystic papillary tumor: two tumors related to pancreatic ontogeny.
Kissane JM
Semin Diagn Pathol 1994 May;11(2):152-64. PMID: 7809509

Clinical prediction guides

DNA Methylation Profiling Enables Accurate Classification of Nonductal Primary Pancreatic Neoplasms.
Verschuur AVD, Hackeng WM, Westerbeke F, Benhamida JK, Basturk O, Selenica P, Raicu GM, Molenaar IQ, van Santvoort HC, Daamen LA, Klimstra DS, Yachida S, Luchini C, Singhi AD, Geisenberger C, Brosens LAA
Clin Gastroenterol Hepatol 2024 Jun;22(6):1245-1254.e10. Epub 2024 Feb 20 doi: 10.1016/j.cgh.2024.02.007. PMID: 38382726
CT, MRI, and (18)F-FDG PET/CT imaging features of seven cases of adult pancreatoblastoma.
Wu M, Lin J, Liu Z, Huang Z, Wang R
BMC Med Imaging 2022 Dec 29;22(1):228. doi: 10.1186/s12880-022-00958-4. PMID: 36581821Free PMC Article
Adult pancreatoblastoma: clinical features and Imaging findings.
Zhang X, Ni SJ, Wang XH, Huang D, Tang W
Sci Rep 2020 Jul 9;10(1):11285. doi: 10.1038/s41598-020-68083-2. PMID: 32647222Free PMC Article
Pancreatoblastoma: a rare tumor still evolving in clinical presentation and histology.
Balasundaram C, Luthra M, Chavalitdhamrong D, Chow J, Khan H, Endres PJ
JOP 2012 May 10;13(3):301-3. PMID: 22572137
Pancreatoblastoma in adults: a review of the literature.
Cavallini A, Falconi M, Bortesi L, Crippa S, Barugola G, Butturini G
Pancreatology 2009;9(1-2):73-80. Epub 2008 Dec 12 doi: 10.1159/000178877. PMID: 19077457

Recent systematic reviews

Solid pseudopapillary and malignant pancreatic tumors in childhood: A systematic review and evidence quality assessment.
Mylonas KS, Doulamis IP, Tsilimigras DI, Nasioudis D, Schizas D, Masiakos PT, Kelleher CM
Pediatr Blood Cancer 2018 Oct;65(10):e27114. Epub 2018 Apr 26 doi: 10.1002/pbc.27114. PMID: 29697193

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