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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

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
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

Recent clinical studies

Etiology

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
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
Pancreatic Neoplasms With Acinar Differentiation: A Review of Pathologic and Molecular Features.
Thompson ED, Wood LD
Arch Pathol Lab Med 2020 Jul 1;144(7):808-815. doi: 10.5858/arpa.2019-0472-RA. PMID: 31869246
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

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
Pancreatoblastoma.
Saif MW
JOP 2007 Jan 9;8(1):55-63. PMID: 17228135
Pancreatoblastoma.
Chun Y, Kim W, Park K, Lee S, Jung S
J Pediatr Surg 1997 Nov;32(11):1612-5. doi: 10.1016/s0022-3468(97)90465-6. PMID: 9396538

Therapy

Association between Pancreatoblastoma and Familial Adenomatous Polyposis: Review of the Literature with an Additional Case.
Remo A, Negro S, Bao RQ, d'Angelo E, Alaggio R, Crivellari G, Mammi I, Intini R, Bergamo F, Fassan M, Agostini M, Vitellaro M, Pucciarelli S, Urso EDL
Genes (Basel) 2023 Dec 27;15(1) doi: 10.3390/genes15010044. PMID: 38254934Free 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
Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors.
Al-Hader A, Al-Rohil RN, Han H, Von Hoff D
World J Gastroenterol 2017 Dec 7;23(45):7945-7951. doi: 10.3748/wjg.v23.i45.7945. PMID: 29259370Free PMC Article
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
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

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
Pancreatic Neoplasms With Acinar Differentiation: A Review of Pathologic and Molecular Features.
Thompson ED, Wood LD
Arch Pathol Lab Med 2020 Jul 1;144(7):808-815. doi: 10.5858/arpa.2019-0472-RA. PMID: 31869246
Pancreatoblastoma.
Saif MW
JOP 2007 Jan 9;8(1):55-63. PMID: 17228135
Pancreatoblastoma.
Chun Y, Kim W, Park K, Lee S, Jung S
J Pediatr Surg 1997 Nov;32(11):1612-5. doi: 10.1016/s0022-3468(97)90465-6. PMID: 9396538

Clinical prediction guides

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
Pancreatic Neoplasms With Acinar Differentiation: A Review of Pathologic and Molecular Features.
Thompson ED, Wood LD
Arch Pathol Lab Med 2020 Jul 1;144(7):808-815. doi: 10.5858/arpa.2019-0472-RA. PMID: 31869246
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.
Chun Y, Kim W, Park K, Lee S, Jung S
J Pediatr Surg 1997 Nov;32(11):1612-5. doi: 10.1016/s0022-3468(97)90465-6. PMID: 9396538

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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