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Solid pseudopapillary neoplasm of the pancreas

MedGen UID:
234800
Concept ID:
C1336030
Neoplastic Process
Synonyms: Frantz tumor; Frantz tumour; Solid Pseudopapillary Neoplasm of the Pancreas; solid pseudopapillary neoplasm of the pancreas; Solid Pseudopapillary Tumor of the Pancreas; solid pseudopapillary tumor of the pancreas; solid pseudopapillary tumour of the pancreas
 
Monarch Initiative: MONDO:0044786

Definition

A low-grade malignant neoplasm that arises from the exocrine pancreas. It is characterized by the presence of uniform cells that form solid and pseudopapillary patterns, cystic changes, and hemorrhage. Perineural invasion, vascular invasion, and invasion into surrounding tissues may be present. It usually presents as an encapsulated, solitary, and lobulated pancreatic mass. It is usually found incidentally during physical examination or it may present with abdominal discomfort and pain. It occurs predominantly in young women. Complete removal of the tumor is curative in the majority of cases. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSolid pseudopapillary neoplasm of the pancreas

Professional guidelines

PubMed

Hosokawa I, Shimizu H, Ohtsuka M, Kato A, Yoshitomi H, Furukawa K, Takayashiki T, Ishihara T, Yokosuka O, Miyazaki M
J Hepatobiliary Pancreat Sci 2014 Aug;21(8):573-8. Epub 2014 Feb 17 doi: 10.1002/jhbp.96. PMID: 24535774
Guo N, Zhou QB, Chen RF, Zou SQ, Li ZH, Lin Q, Wang J, Chen JS
Can J Surg 2011 Dec;54(6):368-74. doi: 10.1503/cjs.011810. PMID: 21939604Free PMC Article
Romics L Jr, Oláh A, Belágyi T, Hajdú N, Gyurus P, Ruszinkó V
Langenbecks Arch Surg 2010 Aug;395(6):747-55. Epub 2010 Feb 14 doi: 10.1007/s00423-010-0599-0. PMID: 20155425

Recent clinical studies

Therapy

Gao Y, Guo F, Lu Z, Xi C, Wei J, Jiang K, Miao Y, Wu J, Chen J
World J Surg Oncol 2023 Mar 31;21(1):119. doi: 10.1186/s12957-023-03003-y. PMID: 37004027Free PMC Article
Yamaguchi H, Morisaka H, Sano K, Nagata K, Ryozawa S, Okamoto K, Ichikawa T
Intern Med 2020 Mar 15;59(6):779-782. Epub 2019 Nov 29 doi: 10.2169/internalmedicine.3244-19. PMID: 31787691Free PMC Article
Magoga G, Felder L, Palazzo F, Berghella V
J Matern Fetal Neonatal Med 2020 Jan;33(2):344-348. Epub 2018 Jul 22 doi: 10.1080/14767058.2018.1488959. PMID: 29895203
Li G, Baek NH, Yoo K, Hwang JC, Kim JH, Yoo BM, Kim WH, Kim JH
Hepatogastroenterology 2014 Sep;61(134):1780-4. PMID: 25436379
Hofmann H, von Haken R, Werner J, Kortes N, Bergmann F, Schemmer P, Jäger D, Radeleff B, Schulze-Bergkamen H
Pancreatology 2014 Nov-Dec;14(6):546-9. Epub 2014 Sep 16 doi: 10.1016/j.pan.2014.08.006. PMID: 25280592

Prognosis

Fleming AM, Hendrick LE, Yakoub D, Abdelhafeez H, Deneve JL, Langham MR Jr, Glazer ES, Davidoff AM, Merchant NB, Dickson PV, Murphy AJ
Ann Surg Oncol 2024 Jan;31(1):475-487. Epub 2023 Sep 28 doi: 10.1245/s10434-023-14343-0. PMID: 37768414
Liu Q, Dai M, Guo J, Wu H, Wang W, Chen G, Hu Y, Han X, Xu Q, Zhang X, Yang S, Zhang Y, Kleeff J, Liao Q, Wu W, Liang Z, Zhang T, Zhao Y
Ann Surg 2023 Dec 1;278(6):1009-1017. Epub 2023 Apr 10 doi: 10.1097/SLA.0000000000005842. PMID: 37036095
Zalatnai A, Kis-Orha V
Pathol Oncol Res 2020 Apr;26(2):641-649. Epub 2019 Jun 17 doi: 10.1007/s12253-019-00671-8. PMID: 31209654Free PMC Article
Lubezky N, Papoulas M, Lessing Y, Gitstein G, Brazowski E, Nachmany I, Lahat G, Goykhman Y, Ben-Yehuda A, Nakache R, Klausner JM
Eur J Surg Oncol 2017 Jun;43(6):1056-1060. Epub 2017 Feb 10 doi: 10.1016/j.ejso.2017.02.001. PMID: 28238521
Bhatnagar R, Olson MT, Fishman EK, Hruban RH, Lennon AM, Ali SZ
Acta Cytol 2014;58(4):347-55. Epub 2014 Jun 21 doi: 10.1159/000363546. PMID: 24969629

Clinical prediction guides

Liu YA, Liu Y, Tu J, Shi Y, Pang J, Huang Q, Wang X, Lin Z, Zhao Y, Wang W, Peng J, Wu W
Am J Surg Pathol 2024 May 1;48(5):511-520. Epub 2024 Apr 3 doi: 10.1097/PAS.0000000000002205. PMID: 38567813Free PMC Article
Fleming AM, Hendrick LE, Yakoub D, Abdelhafeez H, Deneve JL, Langham MR Jr, Glazer ES, Davidoff AM, Merchant NB, Dickson PV, Murphy AJ
Ann Surg Oncol 2024 Jan;31(1):475-487. Epub 2023 Sep 28 doi: 10.1245/s10434-023-14343-0. PMID: 37768414
Liu Q, Dai M, Guo J, Wu H, Wang W, Chen G, Hu Y, Han X, Xu Q, Zhang X, Yang S, Zhang Y, Kleeff J, Liao Q, Wu W, Liang Z, Zhang T, Zhao Y
Ann Surg 2023 Dec 1;278(6):1009-1017. Epub 2023 Apr 10 doi: 10.1097/SLA.0000000000005842. PMID: 37036095
La Rosa S, Bongiovanni M
Arch Pathol Lab Med 2020 Jul 1;144(7):829-837. doi: 10.5858/arpa.2019-0473-RA. PMID: 31958381
Zalatnai A, Kis-Orha V
Pathol Oncol Res 2020 Apr;26(2):641-649. Epub 2019 Jun 17 doi: 10.1007/s12253-019-00671-8. PMID: 31209654Free PMC Article

Recent systematic reviews

Fleming AM, Hendrick LE, Yakoub D, Abdelhafeez H, Deneve JL, Langham MR Jr, Glazer ES, Davidoff AM, Merchant NB, Dickson PV, Murphy AJ
Ann Surg Oncol 2024 Jan;31(1):475-487. Epub 2023 Sep 28 doi: 10.1245/s10434-023-14343-0. PMID: 37768414
Magoga G, Felder L, Palazzo F, Berghella V
J Matern Fetal Neonatal Med 2020 Jan;33(2):344-348. Epub 2018 Jul 22 doi: 10.1080/14767058.2018.1488959. PMID: 29895203
Hao EIU, Hwang HK, Yoon DS, Lee WJ, Kang CM
Medicine (Baltimore) 2018 Dec;97(49):e13147. doi: 10.1097/MD.0000000000013147. PMID: 30544374Free PMC Article
Bender AM, Thompson ED, Hackam DJ, Cameron JL, Rhee DS
Pancreas 2018 Nov/Dec;47(10):1364-1368. doi: 10.1097/MPA.0000000000001183. PMID: 30325866

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