Pancreatic islet cell carcinoma

Jump to: navigation, search
Pancreatic islet cell carcinoma

WikiDoc Resources for Pancreatic islet cell carcinoma

Articles

Most recent articles on Pancreatic islet cell carcinoma

Most cited articles on Pancreatic islet cell carcinoma

Review articles on Pancreatic islet cell carcinoma

Articles on Pancreatic islet cell carcinoma in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Pancreatic islet cell carcinoma

Images of Pancreatic islet cell carcinoma

Photos of Pancreatic islet cell carcinoma

Podcasts & MP3s on Pancreatic islet cell carcinoma

Videos on Pancreatic islet cell carcinoma

Evidence Based Medicine

Cochrane Collaboration on Pancreatic islet cell carcinoma

Bandolier on Pancreatic islet cell carcinoma

TRIP on Pancreatic islet cell carcinoma

Clinical Trials

Ongoing Trials on Pancreatic islet cell carcinoma at Clinical Trials.gov

Trial results on Pancreatic islet cell carcinoma

Clinical Trials on Pancreatic islet cell carcinoma at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Pancreatic islet cell carcinoma

NICE Guidance on Pancreatic islet cell carcinoma

NHS PRODIGY Guidance

FDA on Pancreatic islet cell carcinoma

CDC on Pancreatic islet cell carcinoma

Books

Books on Pancreatic islet cell carcinoma

News

Pancreatic islet cell carcinoma in the news

Be alerted to news on Pancreatic islet cell carcinoma

News trends on Pancreatic islet cell carcinoma

Commentary

Blogs on Pancreatic islet cell carcinoma

Definitions

Definitions of Pancreatic islet cell carcinoma

Patient Resources / Community

Patient resources on Pancreatic islet cell carcinoma

Discussion groups on Pancreatic islet cell carcinoma

Patient Handouts on Pancreatic islet cell carcinoma

Directions to Hospitals Treating Pancreatic islet cell carcinoma

Risk calculators and risk factors for Pancreatic islet cell carcinoma

Healthcare Provider Resources

Symptoms of Pancreatic islet cell carcinoma

Causes & Risk Factors for Pancreatic islet cell carcinoma

Diagnostic studies for Pancreatic islet cell carcinoma

Treatment of Pancreatic islet cell carcinoma

Continuing Medical Education (CME)

CME Programs on Pancreatic islet cell carcinoma

International

Pancreatic islet cell carcinoma en Espanol

Pancreatic islet cell carcinoma en Francais

Business

Pancreatic islet cell carcinoma in the Marketplace

Patents on Pancreatic islet cell carcinoma

Experimental / Informatics

List of terms related to Pancreatic islet cell carcinoma

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Jogeet Singh Sekhon, M.D. [2]

Overview

Pancreas has 2 types of cells, acinar and islet that produce exocrine and endocrine hormones respectively. The pancreatic islet cell carcinomas are also known as neuroendocrine tumors. They are of 6 types depending on the type of hormone the tumor produces. They can occur sporadically or in association with other disorders such as multiple endocrine neoplasia type 1, Von Hippel Lindau disease, neurofibromatosis type 1 and tuberous sclerosis which are inherited in an autosomal dominant pattern. The most common metastatic sites are the liver, the lymph nodes, and the bones. The cancer usually presents with jaundice, light-colored stools, dark urine, pain in the upper or middle abdomen and back, unexplained weight loss, anorexia, fatigue and symptoms according to the hormone produced. The treatment depends on the spread of the cancer and includes both medical and surgical treatment.

Historical perspective

In 1869, pancreatic islet cells were discovered by Paul Langerhans and the first adenoma of islets was discovered by Nicholls in 1902[1].

Classification

Pancreatic islet cell carcinoma is of 6 types[2]:

Pathophysiology

2010 WHO grading system for pNETs

Grade 1 (G1) Grade 2 (G2) Grade 3 (G3)
Ki-67 index <3% 3-20% >20%
Mitotic count <2/10 HPF 2-20/10 HPF >20/10 HPF
Differentiation Well differentiated Well differentiated Poorly differentiated
5-year survival rate 85% 78% 9%

Causes

There are no established causes of pancreatic islet cell carcinoma but there is an association with MEN 1 syndrome and rarely with Von Hippel-Lindau disease, Neurofibromatosis-1 and Tuberous sclerosis[9].

Epidemiology and Demographics

  • The incidence of pancreatic islet cell carcinoma is 1 in 100000 people[10].

Age

  • It mostly occurs in fourth to sixth decades of life.

Gender

  • Its incidence is equal in both males and females.

Race

  • Race has no effect on the incidence.

Staging

Pancreatic cancer is staged according to the TNM staging system based on the primary tumor, lymph nodes involved and distant metastasis[11][12].

TNM Classification for Pancreatic Cancer:
Primary tumor
TX Primary tumor cannot be assessed
T0 No evidence of primary tumor
Tis Carcinoma in situ
T1 Tumor limited to the pancreas, ≤2 cm in greatest dimension
T2 Tumor limited to the pancreas, >2 cm in greatest dimension
T3 Tumor extends beyond the pancreas but without involvement of the celiac axis or the superior mesenteric artery
T4 Tumor involves the celiac axis or the superior mesenteric artery (unresectable primary tumor)
Regional lymph nodes
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1 Regional lymph node metastasis
Distant metastases
MX Distant metastasis cannot be assessed
M0 No distant metastasis
M1 Distant metastasis

Screening

Screening for pancreatic islet cell carcinoma is not done.

Natural history, complications and prognosis

Natural history

Complications

Prognosis

Prognosis of pancreatic islet cell carcinoma depends on the following[16]:

  • Whether or not the tumor can be removed by surgery.
  • The stage of the tumor, the size of the tumor, whether cancer has spread outside the pancreas.
  • The patient’s general health.
  • Whether the tumor has just been diagnosed or has recurred.
  • The presence of metastasis is associated with a particularly poor prognosis. Grade 1 and 2 tumor have the most favorable prognosis.
  • The 10-year event free survival rate is less than 51.6% with metastasis and 64.3% without metastasis.

Diagnosis

Diagnostic findings

History and symptoms

History includes[15][4][19]:

Physical examination

Treatment

Medical Therapy

Surgery

References

  1. Stamatakos M, Safioleas C, Tsaknaki S, Safioleas P, Iannescu R, Safioleas M (2009). "Insulinoma: a rare neuroendocrine pancreatic tumor.". Chirurgia (Bucur). 104 (6): 669–73. PMID 20187464. 
  2. Klöppel G, Perren A, Heitz PU (2004). "The gastroenteropancreatic neuroendocrine cell system and its tumors: the WHO classification.". Ann N Y Acad Sci. 1014: 13–27. PMID 15153416. 
  3. Metz DC, Jensen RT (2008). "Gastrointestinal neuroendocrine tumors: pancreatic endocrine tumors.". Gastroenterology. 135 (5): 1469–92. PMC 2612755Freely accessible. PMID 18703061. doi:10.1053/j.gastro.2008.05.047. 
  4. 4.0 4.1 Falconi M, Plockinger U, Kwekkeboom DJ, Manfredi R, Korner M, Kvols L; et al. (2006). "Well-differentiated pancreatic nonfunctioning tumors/carcinoma.". Neuroendocrinology. 84 (3): 196–211. PMID 17312380. doi:10.1159/000098012. 
  5. Service FJ, McMahon MM, O'Brien PC, Ballard DJ (1991). "Functioning insulinoma--incidence, recurrence, and long-term survival of patients: a 60-year study.". Mayo Clin Proc. 66 (7): 711–9. PMID 1677058. 
  6. Frankton S, Bloom SR (1996). "Gastrointestinal endocrine tumours. Glucagonomas.". Baillieres Clin Gastroenterol. 10 (4): 697–705. PMID 9113318. 
  7. Madura JA, Cummings OW, Wiebke EA, Broadie TA, Goulet RL, Howard TJ (1997). "Nonfunctioning islet cell tumors of the pancreas: a difficult diagnosis but one worth the effort.". Am Surg. 63 (7): 573–7; discussion 577–8. PMID 9202529. 
  8. Bond-Smith G, Banga N, Hammond TM, Imber CJ (2012). "Pancreatic adenocarcinoma.". BMJ. 344: e2476. PMID 22592847. doi:10.1136/bmj.e2476. 
  9. Leoncini E, Carioli G, La Vecchia C, Boccia S, Rindi G (2016). "Risk factors for neuroendocrine neoplasms: a systematic review and meta-analysis.". Ann Oncol. 27 (1): 68–81. PMID 26487581. doi:10.1093/annonc/mdv505. 
  10. Hallet J, Law CH, Cukier M, Saskin R, Liu N, Singh S (2015). "Exploring the rising incidence of neuroendocrine tumors: a population-based analysis of epidemiology, metastatic presentation, and outcomes.". Cancer. 121 (4): 589–97. PMID 25312765. doi:10.1002/cncr.29099. 
  11. Ryan DP, Hong TS, Bardeesy N (2014). "Pancreatic adenocarcinoma.". N Engl J Med. 371 (11): 1039–49. PMID 25207767. doi:10.1056/NEJMra1404198. 
  12. Klimstra DS, Modlin IR, Coppola D, Lloyd RV, Suster S (2010). "The pathologic classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems.". Pancreas. 39 (6): 707–12. PMID 20664470. doi:10.1097/MPA.0b013e3181ec124e. 
  13. Graves TD, Gandhi S, Smith SJ, Sisodiya SM, Conway GS (2004). "Misdiagnosis of seizures: insulinoma presenting as adult-onset seizure disorder.". J Neurol Neurosurg Psychiatry. 75 (8): 1091–2. PMC 1739168Freely accessible. PMID 15258206. doi:10.1136/jnnp.2003.029249. 
  14. de Mestier L, Hentic O, Cros J, Walter T, Roquin G, Brixi H; et al. (2015). "Metachronous hormonal syndromes in patients with pancreatic neuroendocrine tumors: a case-series study.". Ann Intern Med. 162 (10): 682–9. PMID 25984844. doi:10.7326/M14-2132. 
  15. 15.0 15.1 Zerbi A, Falconi M, Rindi G, Delle Fave G, Tomassetti P, Pasquali C; et al. (2010). "Clinicopathological features of pancreatic endocrine tumors: a prospective multicenter study in Italy of 297 sporadic cases.". Am J Gastroenterol. 105 (6): 1421–9. PMID 20087335. doi:10.1038/ajg.2009.747. 
  16. Halfdanarson TR, Rabe KG, Rubin J, Petersen GM (2008). "Pancreatic neuroendocrine tumors (PNETs): incidence, prognosis and recent trend toward improved survival.". Ann Oncol. 19 (10): 1727–33. PMC 2735065Freely accessible. PMID 18515795. doi:10.1093/annonc/mdn351. 
  17. Vagefi PA, Razo O, Deshpande V, McGrath DJ, Lauwers GY, Thayer SP; et al. (2007). "Evolving patterns in the detection and outcomes of pancreatic neuroendocrine neoplasms: the Massachusetts General Hospital experience from 1977 to 2005.". Arch Surg. 142 (4): 347–54. PMC 3979851Freely accessible. PMID 17438169. doi:10.1001/archsurg.142.4.347. 
  18. Horwhat JD, Gerke H, Acosta RD, Pavey DA, Jowell PS (2009). "Focal or diffuse "fullness" of the pancreas on CT. Usually benign, but EUS plus/minus FNA is warranted to identify malignancy.". JOP. 10 (1): 37–42. PMID 19129613. 
  19. Khorana AA, Fine RL (2004). "Pancreatic cancer and thromboembolic disease.". Lancet Oncol. 5 (11): 655–63. PMID 15522652. doi:10.1016/S1470-2045(04)01606-7. 
  20. Tas F, Sen F, Odabas H, Kılıc L, Keskın S, Yıldız I (2013). "Performance status of patients is the major prognostic factor at all stages of pancreatic cancer.". Int J Clin Oncol. 18 (5): 839–46. PMID 22996141. doi:10.1007/s10147-012-0474-9. 
  21. Huguet F, Girard N, Guerche CS, Hennequin C, Mornex F, Azria D (2009). "Chemoradiotherapy in the management of locally advanced pancreatic carcinoma: a qualitative systematic review.". J Clin Oncol. 27 (13): 2269–77. PMID 19307501. doi:10.1200/JCO.2008.19.7921. 
  22. Brasiūniene B, Juozaityte E (2007). "The effect of combined treatment methods on survival and toxicity in patients with pancreatic cancer.". Medicina (Kaunas). 43 (9): 716–25. PMID 17986845. 
  23. Bancroft J (1992). "Sexual behaviour in Britain and France.". BMJ. 305 (6867): 1447–8. PMC 1884127Freely accessible. PMID 1493387  . 

Linked-in.jpg