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Neoplasm of the pancreas

MedGen UID:
18279
Concept ID:
C0030297
Neoplastic Process
Synonym: Pancreatic neoplasm
SNOMED CT: Neoplasm of pancreas (126859007); Tumor of pancreas (126859007)
 
HPO: HP:0002894
Monarch Initiative: MONDO:0021040

Definition

A tumor (abnormal growth of tissue) of the pancreas. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
Follow this link to review classifications for Neoplasm of the pancreas in Orphanet.

Conditions with this feature

Von Hippel-Lindau syndrome
MedGen UID:
42458
Concept ID:
C0019562
Disease or Syndrome
Von Hippel-Lindau (VHL) syndrome is characterized by hemangioblastomas of the brain, spinal cord, and retina; renal cysts and clear cell renal cell carcinoma; pheochromocytoma, pancreatic cysts, and neuroendocrine tumors; endolymphatic sac tumors; and epididymal and broad ligament cysts. Cerebellar hemangioblastomas may be associated with headache, vomiting, gait disturbances, or ataxia. Spinal hemangioblastomas and related syrinx usually present with pain. Sensory and motor loss may develop with cord compression. Retinal hemangioblastomas may be the initial manifestation of VHL syndrome and can cause vision loss. Renal cell carcinoma occurs in about 70% of individuals with VHL and is the leading cause of mortality. Pheochromocytomas can be asymptomatic but may cause sustained or episodic hypertension. Pancreatic lesions often remain asymptomatic and rarely cause endocrine or exocrine insufficiency. Endolymphatic sac tumors can cause hearing loss of varying severity, which can be a presenting symptom. Cystadenomas of the epididymis are relatively common. They rarely cause problems, unless bilateral, in which case they may result in infertility.
Peutz-Jeghers syndrome
MedGen UID:
18404
Concept ID:
C0031269
Disease or Syndrome
Peutz-Jeghers syndrome (PJS) is characterized by the association of gastrointestinal (GI) polyposis, mucocutaneous pigmentation, and cancer predisposition. PJS-type hamartomatous polyps are most common in the small intestine (in order of prevalence: jejunum, ileum, and duodenum) but can also occur in the stomach, large bowel, and extraintestinal sites including the renal pelvis, bronchus, gall bladder, nasal passages, urinary bladder, and ureters. GI polyps can result in chronic bleeding, anemia, and recurrent obstruction and intussusception requiring repeated laparotomy and bowel resection. Mucocutaneous hyperpigmentation presents in childhood as dark blue to dark brown macules around the mouth, eyes, and nostrils, in the perianal area, and on the buccal mucosa. Hyperpigmented macules on the fingers are common. The macules may fade in puberty and adulthood. Recognition of the distinctive skin manifestations is important especially in individuals who have PJS as the result of a de novo pathogenic variant as these skin findings often predate GI signs and symptoms. Individuals with PJS are at increased risk for a wide variety of epithelial malignancies (colorectal, gastric, pancreatic, breast, and ovarian cancers). Females are at risk for sex cord tumors with annular tubules (SCTAT), a benign neoplasm of the ovaries, and adenoma malignum of the cervix, a rare aggressive cancer. Males occasionally develop large calcifying Sertoli cell tumors of the testes, which secrete estrogen and can lead to gynecomastia, advanced skeletal age, and ultimately short stature, if untreated.
Lynch syndrome 5
MedGen UID:
318886
Concept ID:
C1833477
Disease or Syndrome
Lynch syndrome-5 (LYNCH5), or hereditary nonpolyposis colorectal cancer type 5 (HNPCC5), is a cancer predisposition syndrome characterized by onset of colorectal cancer and/or extracolonic cancers, particularly endometrial cancer, usually in mid-adulthood. The disorder shows autosomal dominant inheritance with incomplete penetrance (summary by Castellsague et al., 2015). For a general phenotypic description and a discussion of genetic heterogeneity of Lynch syndrome, see 120435.
Li-Fraumeni syndrome 1
MedGen UID:
322656
Concept ID:
C1835398
Disease or Syndrome
Li-Fraumeni syndrome (LFS) is a cancer predisposition syndrome associated with high risks for a diverse spectrum of childhood- and adult-onset malignancies. The lifetime risk of cancer in individuals with LFS is =70% for men and =90% for women. Five cancer types account for the majority of LFS tumors: adrenocortical carcinomas, breast cancer, central nervous system tumors, osteosarcomas, and soft-tissue sarcomas. LFS is associated with an increased risk of several additional cancers including leukemia, lymphoma, gastrointestinal cancers, cancers of head and neck, kidney, larynx, lung, skin (e.g., melanoma), ovary, pancreas, prostate, testis, and thyroid. Individuals with LFS are at increased risk for cancer in childhood and young adulthood; survivors are at increased risk for multiple primary cancers.
Tropical pancreatitis
MedGen UID:
334069
Concept ID:
C1842402
Disease or Syndrome
Tropical calcific pancreatitis (TCP) is a type of chronic pancreatitis unique to tropical regions. Large intraductal calculi are present, and evidence of pancreatic calcification is seen on imaging studies (summary by Mahurkar et al., 2006).
Familial pancreatic carcinoma
MedGen UID:
419700
Concept ID:
C2931038
Neoplastic Process
Pancreatic cancer shows among the highest mortality rates of any cancer, with a 5-year relative survival rate of less than 5%. By the time of initial diagnosis, metastatic disease is commonly present. Established risk factors include a family history of pancreatic cancer, a medical history of diabetes type 2, and cigarette smoking (summary by Amundadottir et al., 2009). Genetic Heterogeneity of Pancreatic Cancer Somatic mutations in pancreatic cancer occur in the KRAS (190070), CDKN2A (600160), MADH4 (600993), TP53 (191170), ARMET (601916), STK11 (602216), ACVR1B (601300), and RBBP8 (604124) genes. Susceptibility loci for pancreatic cancer include PNCA1 (606856), related to mutation in the PALLD gene on chromosome 4q32 (608092); PNCA2 (613347), related to mutation in the BRCA2 gene on chromosome 13q12 (600185); PNCA3 (613348), related to mutation in the PALB2 gene on chromosome 16p12 (610355); PNCA4 (614320), related to mutation in the BRCA1 gene on chromosome 17q21 (113705); and PNCA5 (618680), related to mutation in the RABL3 gene on chromosome 3q13 (618542). Occurrence of Pancreatic Cancer in Other Disorders Several familial cancer syndromes increase the risk of pancreatic cancer. The best characterized include hereditary nonpolyposis colon cancer syndrome (HNPCC; see 120435); hereditary breast-ovarian cancer syndrome due to mutations in BRCA2; Peutz-Jeghers syndrome (175200); the melanoma-pancreatic cancer syndrome (606719), caused by mutations in CDKN2A (600160); von Hippel-Lindau syndrome (193300), ataxia-telangiectasia (208900) (Swift et al., 1976), and juvenile polyposis syndrome (174900). Patients with hereditary pancreatitis (167800) resulting from gain-of-function mutations in the protease serine-1 gene (PRSS1; 276000) have a lifetime pancreatic cancer risk ratio of 57 and a cumulative incidence, to age 70 years, of 40% (Lowenfels et al., 1997).
Pancreatic cancer, susceptibility to, 2
MedGen UID:
461896
Concept ID:
C3150546
Finding
BRCA1- and BRCA2-associated hereditary breast and ovarian cancer (HBOC) is characterized by an increased risk for female and male breast cancer, ovarian cancer (including fallopian tube and primary peritoneal cancers), and to a lesser extent other cancers such as prostate cancer, pancreatic cancer, and melanoma primarily in individuals with a BRCA2 pathogenic variant. The risk of developing an associated cancer varies depending on whether HBOC is caused by a BRCA1 or BRCA2 pathogenic variant.

Professional guidelines

PubMed

Ohtsuka T, Fernandez-Del Castillo C, Furukawa T, Hijioka S, Jang JY, Lennon AM, Miyasaka Y, Ohno E, Salvia R, Wolfgang CL, Wood LD
Pancreatology 2024 Mar;24(2):255-270. Epub 2023 Dec 28 doi: 10.1016/j.pan.2023.12.009. PMID: 38182527
Dal Borgo C, Perri G, Borin A, Marchegiani G, Salvia R, Bassi C
Dig Surg 2019;36(2):104-110. Epub 2018 Feb 8 doi: 10.1159/000486869. PMID: 29421807
Tanaka M, Fernández-del Castillo C, Adsay V, Chari S, Falconi M, Jang JY, Kimura W, Levy P, Pitman MB, Schmidt CM, Shimizu M, Wolfgang CL, Yamaguchi K, Yamao K; International Association of Pancreatology
Pancreatology 2012 May-Jun;12(3):183-97. Epub 2012 Apr 16 doi: 10.1016/j.pan.2012.04.004. PMID: 22687371

Recent clinical studies

Etiology

Han Y, Kwon W, Lee M, Jung HS, Yun WG, Cho YJ, Chae YS, Fernández-Del Castillo C, Marchegiani G, Salvia R, Goh BKP, Lee WJ, Jang JY
JAMA Surg 2024 Apr 1;159(4):389-396. doi: 10.1001/jamasurg.2023.7010. PMID: 38231494Free PMC Article
Ohtsuka T, Fernandez-Del Castillo C, Furukawa T, Hijioka S, Jang JY, Lennon AM, Miyasaka Y, Ohno E, Salvia R, Wolfgang CL, Wood LD
Pancreatology 2024 Mar;24(2):255-270. Epub 2023 Dec 28 doi: 10.1016/j.pan.2023.12.009. PMID: 38182527
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
Salinas W, Marani M, Reimondez S, Alcaraz Á, Signorini F, Maraschio M, Giordano E, Obeide L
Cir Cir 2021;89(2):263-268. doi: 10.24875/CIRU.19001163. PMID: 33784288
Tanaka M, Fernández-del Castillo C, Adsay V, Chari S, Falconi M, Jang JY, Kimura W, Levy P, Pitman MB, Schmidt CM, Shimizu M, Wolfgang CL, Yamaguchi K, Yamao K; International Association of Pancreatology
Pancreatology 2012 May-Jun;12(3):183-97. Epub 2012 Apr 16 doi: 10.1016/j.pan.2012.04.004. PMID: 22687371

Diagnosis

Ohtsuka T, Fernandez-Del Castillo C, Furukawa T, Hijioka S, Jang JY, Lennon AM, Miyasaka Y, Ohno E, Salvia R, Wolfgang CL, Wood LD
Pancreatology 2024 Mar;24(2):255-270. Epub 2023 Dec 28 doi: 10.1016/j.pan.2023.12.009. PMID: 38182527
Salinas W, Marani M, Reimondez S, Alcaraz Á, Signorini F, Maraschio M, Giordano E, Obeide L
Cir Cir 2021;89(2):263-268. doi: 10.24875/CIRU.19001163. PMID: 33784288
Pérez Fernández A, Alcaraz Mateos E, López Martín A
Rev Esp Enferm Dig 2021 Sep;113(9):682. doi: 10.17235/reed.2020.7671/2020. PMID: 33393336
Dhillon J, Betancourt M
Monogr Clin Cytol 2020;26:74-91. Epub 2020 Sep 28 doi: 10.1159/000455736. PMID: 32987385
Kuan LL, Dennison AR, Garcea G
Pancreas 2020 Apr;49(4):498-502. doi: 10.1097/MPA.0000000000001518. PMID: 32282762

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
Sugawara T, Franco SR, Ishida J, Kalra A, Saben JL, Gálvez KN, Kirsch MJ, Al-Musawi MH, Kaplan B, Pomfret EA, Schulick RD, Del Chiaro M
Am J Transplant 2023 Mar;23(3):429-436. Epub 2023 Jan 12 doi: 10.1016/j.ajt.2022.11.024. PMID: 36695699
Zhao W, Liu S, Cong L, Zhao Y
Ann Surg Oncol 2022 Feb;29(2):1297-1312. Epub 2021 Sep 23 doi: 10.1245/s10434-021-10662-2. PMID: 34554343
Testini M, Gurrado A, Lissidini G, Venezia P, Greco L, Piccinni G
World J Gastroenterol 2010 Dec 7;16(45):5682-92. doi: 10.3748/wjg.v16.i45.5682. PMID: 21128317Free PMC Article
Hamidieh AA, Jalili M, Khojasteh O, Ghavamzadeh A
Pediatr Blood Cancer 2010 Sep;55(3):573-6. doi: 10.1002/pbc.22536. PMID: 20658635

Prognosis

Sun G, Fang K, Fu X, Peng L, Shu J, Tu Y, Li Y, Xiao W
Pancreas 2023 Feb 1;52(2):e121-e126. doi: 10.1097/MPA.0000000000002219. PMID: 37523603
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
Kuan LL, Dennison AR, Garcea G
Pancreas 2020 Apr;49(4):498-502. doi: 10.1097/MPA.0000000000001518. PMID: 32282762
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
Kim H, Ro JY
Arch Pathol Lab Med 2018 Mar;142(3):420-423. doi: 10.5858/arpa.2016-0405-RSR2. PMID: 29494224

Clinical prediction guides

Ohtsuka T, Fernandez-Del Castillo C, Furukawa T, Hijioka S, Jang JY, Lennon AM, Miyasaka Y, Ohno E, Salvia R, Wolfgang CL, Wood LD
Pancreatology 2024 Mar;24(2):255-270. Epub 2023 Dec 28 doi: 10.1016/j.pan.2023.12.009. PMID: 38182527
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
Geramizadeh B, Marzban M, Shojazadeh A, Kadivar A, Maleki Z
Cytopathology 2021 Jul;32(4):397-406. Epub 2021 Apr 1 doi: 10.1111/cyt.12973. PMID: 33792980
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

Paolino G, Basturk O, Esposito I, Hong SM, Brosens LA, Tarcan Z, Wood LD, Gkountakos A, Omori Y, Mattiolo P, Ciulla C, Marchegiani G, Pea A, Bevere M, De Robertis R, D'Onofrio M, Salvia R, Cheng L, Furukawa T, Scarpa A, Adsay V, Luchini C
Mod Pathol 2024 Sep;37(9):100554. Epub 2024 Jun 29 doi: 10.1016/j.modpat.2024.100554. PMID: 38950698
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
Seki T, Watanabe A, Harimoto N, Shirabe K
Surg Today 2022 Apr;52(4):532-541. Epub 2021 Jul 1 doi: 10.1007/s00595-021-02324-3. PMID: 34196805
Wang YZ, Lu J, Jiang BL, Guo JC
Pancreatology 2019 Sep;19(6):858-865. Epub 2019 Jul 27 doi: 10.1016/j.pan.2019.07.040. PMID: 31375434
Manuel-Vazquez A, Ramia JM, Latorre-Fragua R, Valle-Rubio A, Arteaga-Peralta V, Ramiro-Pérez C, de la Plaza-Llamas R
Pancreas 2018 May/Jun;47(5):551-555. doi: 10.1097/MPA.0000000000001048. PMID: 29683974

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