Pancreatic islet cell carcinoma: Difference between revisions

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== 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|Neurofibromatosis-1]] and [[Tuberous sclerosis]].


== Epidemiology and Demographics ==
== Epidemiology and Demographics ==
* The incidence of pancreatic islet cell carcinoma is 1 in 100000 people.
=== Age ===
* It mostly occurs in fourth to sixth decades of life.
=== Gender ===
* Its incidence is equal in both males and females.


They are uncommon cancers with 200 to 1,000 new cases per year and occur in only 1.5% of detailed autopsy series.  
=== Race ===
* Race has no effect on the incidence.  


==Staging==
==Staging==

Revision as of 18:05, 2 November 2018

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List of terms related to Pancreatic islet cell carcinoma

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

Overview

Cancer of the endocrine pancreas includes a highly treatable and often curable collection of tumors.

Classification

Pancreatic islet cell carcinoma is of 6 types:

Pathophysiology

  • Acinar cells and Islet cells are the 2 types of cells in pancreas.
  • Acinar cells are responsible for secretion of enzymes and bicarbonate involved in the digestion process.
  • Islet cells of pancreas play the endocrine organ role, by producing hormones such as insulin, gastrin, glucagon, somatostatin and vasocative intestinal polypeptide.
  • The pancreatic islet cell carcinomas are also known as neuroendocrine tumors.
  • 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.
  • Islet cell carcinoma is divided into 6 types depending on the type of hormone produced.
  • These tumors produce excessive amounts of hormones and cause symptoms similar to the hormone"s action.
    • Insulinoma - hypoglycemia due to decreased glucose synthesis in liver.
    • Gastrinoma - Zollinger-Ellison syndrome, in which hypersecretion of gastrin causes hydrochloric acid release which causes peptic ulcers.
    • Glucagonoma - hyperglucagonemia, zinc deficiency, fatty acid deficiency, hypoaminoacidemia that may cause necrolytic migratory erythema.
    • VIPoma - Elevated serum VIP levels leads to increased intracellular cAMP which causes increased intestinal secretion of water along with Na+, K+, HCO3 -, and Cl- in the intestinal lumen, causing diarrhea and hypokalemia.
    • Somatostatinoma - increased release of somatostatin causes gallstones, diarrhea and fat malabsorption resulting in steatorrhea.
    • Non functional islet cell tumor- These do not produce any hormones, symptoms depend on the size and metastasis.
  • The tumor cells are usually round with regular bland nuclei which produce large number of secretory granules with diffuse immunoexpression of neuroendocrinemarkers.
  • The poorly differentiated neuroendocrine tumor (NET) shows a atypical, sheet-like, diffuse and irregular nuclei, less cytoplasmic secretory granules, and limited biomarker immunoexpression.
  • Head of the pancreas is most commonly involved (75% of cases) followed by the tail.
  • The most common metastatic sites are the liver, the lymph nodes, and the bones.
  • Pancreatic islet cell carcinoma is divided into 3 grades by WHO.

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.

Epidemiology and Demographics

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

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.

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

Treatment

Medical Therapy

Combination chemotherapy may provide effective palliation as well as increased survival in selected patients. In patients with indolent, slow-growing metastatic islet cell tumors, the best therapy may be careful observation and no treatment until palliation is required.

Surgery

Surgery is the only curative modality.[4,5] Even in those cases not resectable for cure, effective palliation may be achieved because of the slow-growing nature of the majority of these tumors and the potential use of antihormonal pharmacologic therapy (for example, cimetidine in the ulcer-producing Zollinger-Ellison syndrome).

Patients with multiple endocrine neoplasia syndrome type 1, an autosomal dominant condition in which 85% have pancreatic islet cell tumors, 90% have hyperparathyroidism, and 65% have pituitary tumors, are less likely to be cured by pancreatic resection than are patients with sporadic islet cell tumors. With the exception of pain relief from bone metastases, radiation therapy has a limited role in this disease[1].

References

  1. Modlin IM, Lewis JJ, Ahlman H, et al.: Management of unresectable malignant endocrine tumors of the pancreas. Surg Gynecol Obstet 176 (5): 507-18, 1993


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