Pancreatic neuroendocrine tumor: Difference between revisions

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==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
==Treatment==
{{Main|Neuroendocrine tumor}}
In general, treatment for PanNET encompasses the same array of options as other [[neuroendocrine tumor]]s, as discussed in that main article.<!--duplication of that content here is probably unnecessary – see the main article link--> However, there are some specific differences, which are discussed here.
In functioning PanNETs, [[octreotide]] is usually recommended prior to biopsy or surgery but is generally avoided in [[insulinoma]]s to avoid profound [[hypoglycemia]].
PanNETs in [[MEN1]] are often multiple, and thus require different treatment and surveillance strategies.
Some PanNETs are more responsive to [[chemotherapy]] than are gastroenteric [[carcinoid]] tumors. Several agents have shown activity. In well differentiated PanNETs, chemotherapy is generally reserved for when there are no other treatment options. Combinations of several medicines have been used, such as [[doxorubicin]] with [[streptozocin]] and [[fluorouracil]] (5-FU) and capecitabine with temozolomide. Although marginally effective in well-differentiated PETs, [[cisplatin]] with [[etoposide]] has some activity in poorly differentiated neuroendocrine cancers (PDNECs), particularly if the PDNEC has an extremely high [[Ki-67 (protein)|Ki-67]] score of over 50%.
Several [[targeted therapy]] agents have been approved in PanNETs by the [[Food and Drug Administration|FDA]] based on improved [[progression-free survival]] (PFS):
* [[everolimus]] (Afinitor) is labeled for treatment of progressive neuroendocrine tumors of pancreatic origin in patients with unresectable, locally advanced or metastatic disease. The safety and effectiveness of everolimus in carcinoid tumors have not been established.
* [[sunitinib]] (Sutent) is labeled for treatment of progressive, well-differentiated pancreatic neuroendocrine tumors in patients with unresectable locally advanced or metastatic disease. Sutent also has approval from the European Commission for the treatment of 'unresectable or metastatic, well-differentiated pancreatic neuroendocrine tumors with disease progression in adults'. A phase III study of sunitinib treatment in well differentiated pNET that had worsened within the past 12 months (either advanced or metastatic disease) showed that sunitinib treatment improved [[progression-free survival]] (11.4 months vs. 5.5 months), [[overall survival]], and the [[objective response rate]] (9.3% vs. 0.0%) when compared with placebo.


==Genetics==
==Genetics==

Revision as of 19:19, 17 August 2015

Pancreatic neuroendocrine tumor
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Islet-cell carcinoma

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Pancreatic neuroendocrine tumor from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Staging | History and Symptoms | Physical Examination | Laboratory Findings | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Future or Investigational Therapies

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Pancreatic neuroendocrine tumor Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Pancreatic neuroendocrine tumor from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Staging

History and Symptoms

Physical Examination

Laboratory Findings

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Future or Investigational Therapies

Case Studies

Case #1

Pancreatic neuroendocrine tumor On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Pancreatic neuroendocrine tumor

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Pancreatic neuroendocrine tumor

CDC on Pancreatic neuroendocrine tumor

Pancreatic neuroendocrine tumor in the news

Blogs on Pancreatic neuroendocrine tumor

Directions to Hospitals Treating Pancreatic neuroendocrine tumor

Risk calculators and risk factors for Pancreatic neuroendocrine tumor

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References

Genetics

DNA mutation analysis in well-differentiated pancreatic neuroendocrine tumors identified four important findings:[1][2]

  • one in six well-differentiated pancreatic NETs have mutations in mTOR pathway genes, such as TSC2, PTEN and PIK3CA.[1] The sequencing discovery might allow selection of which NETs would benefit from mTOR inhibition such as with everolimus, but this awaits validation in a clinical trial.
  • mutations affecting a new cancer pathway involving ATRX and DAXX genes were found in about 40% of pancreatic NETs.[1] The proteins encoded by ATRX and DAXX participate in chromatin remodeling of telomeres; these mutations are associated with a telomerase-independent maintenance mechanism termed ALT (alternative lengthening of telomeres) that results in abnormally long telomeric ends of chromosomes.

References

  1. 1.0 1.1 1.2 1.3 1.4 Jiao, Y.; Shi, C.; Edil, B. H.; De Wilde, R. F.; Klimstra, D. S.; Maitra, A.; Schulick, R. D.; Tang, L. H.; Wolfgang, C. L.; Choti, M. A.; Velculescu, V. E.; Diaz Jr, L. A.; Vogelstein, B.; Kinzler, K. W.; Hruban, R. H.; Papadopoulos, N. (2011). "DAXX/ATRX, MEN1, and mTOR Pathway Genes Are Frequently Altered in Pancreatic Neuroendocrine Tumors". Science. 331 (6021): 1199–1203. doi:10.1126/science.1200609. PMID 21252315.
  2. McKenna, L. R.; Edil, B. H. (2014). "Update on pancreatic neuroendocrine tumors". Gland surgery. 3 (4): 258–275. doi:10.3978/j.issn.2227-684X.2014.06.03. PMID 25493258.
  3. PMID 18772397 (PMID 18772397)
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  4. PMID 19077451 (PMID 19077451)
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