Zollinger-Ellison syndrome classification

Jump to navigation Jump to search

Zollinger-Ellison syndrome Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Zollinger-Ellison syndrome from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Zollinger-Ellison syndrome classification On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Zollinger-Ellison syndrome classification

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Zollinger-Ellison syndrome classification

CDC on Zollinger-Ellison syndrome classification

Zollinger-Ellison syndrome classification in the news

Blogs on Zollinger-Ellison syndrome classification

Directions to Hospitals Treating Zollinger-Ellison syndrome

Risk calculators and risk factors for Zollinger-Ellison syndrome classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aravind Reddy Kothagadi M.B.B.S[2] Shadan Mehraban, M.D.[3]

Overview

World Health Organization has classified neuroendocrine tumors (NETs) into two broad categories, namely well-differentiated and poorly-differentiated. On the basis of histopathological analysis, most gastrinomas are considered well-differentiated neuroendocrine tumors (NETs). There is also another classification for differentiating sporadic Zollinger-Ellison from MEN 1 Zollinger-Ellison syndrome which is based on familial history, associated endocrinopathies, gastrinoma size, number of tumors, tumor location, and lymph node involvement.

Classification

Grade Diffrentiation Mitotic Range Ki-67 index Behavior WHO Category
G1 Low grade well-differentiated < 2 < 3% (10% to 30%) Uncertain Neuroendocrine tumor
G2 Intermediate grade, well-differentiated 2 to 20 3% to 20% (50% to 80%) Low-grade malignant Neuroendocrine tumor
G3 High grade, poorly differentiated > 20 > 20% (1% to 3%) High-grade malignant Neuroendocrine carcinoma
  • The following table illustrates the factors associated and the differences between sporadic and MEN1 associated Zollinger-Ellison syndrome (ZES):[5][6][7][8][9][10]
Sporadic and MEN-1-associated ZES
Factors Sopradic ZES MEN-1 ZES
Prevalence 80% 20%
Familial history No Yes
Associated endocrinopathies No Yes
Gastrinoma size > 2 cm < 2 cm
Tumors number Single Multiple
Tumor location Pancreas Duodenum
Lymph node involvement 10% No
Surgical cure rate 60% Rare
Malignancy rate High Low

References

  1. Norton JA (1994). "Neuroendocrine tumors of the pancreas and duodenum". Curr Probl Surg. 31 (2): 77–156. PMID 7904550.
  2. O'Toole D, Delle Fave G, Jensen RT (2012). "Gastric and duodenal neuroendocrine tumours". Best Pract Res Clin Gastroenterol. 26 (6): 719–35. doi:10.1016/j.bpg.2013.01.002. PMID 23582915.
  3. "Gastrinoma - StatPearls - NCBI Bookshelf".
  4. Tang LH, Basturk O, Sue JJ, Klimstra DS (2016). "A Practical Approach to the Classification of WHO Grade 3 (G3) Well-differentiated Neuroendocrine Tumor (WD-NET) and Poorly Differentiated Neuroendocrine Carcinoma (PD-NEC) of the Pancreas". Am J Surg Pathol. 40 (9): 1192–202. doi:10.1097/PAS.0000000000000662. PMC 4988129. PMID 27259015.
  5. Ellison EC, Johnson JA (2009). "The Zollinger-Ellison syndrome: a comprehensive review of historical, scientific, and clinical considerations". Curr Probl Surg. 46 (1): 13–106. doi:10.1067/j.cpsurg.2008.09.001. PMID 19059523.
  6. Jensen RT, Niederle B, Mitry E, Ramage JK, Steinmuller T, Lewington V; et al. (2006). "Gastrinoma (duodenal and pancreatic)". Neuroendocrinology. 84 (3): 173–82. doi:10.1159/000098009. PMID 17312377.
  7. Gibril F, Jensen RT (2005). "Advances in evaluation and management of gastrinoma in patients with Zollinger-Ellison syndrome". Curr Gastroenterol Rep. 7 (2): 114–21. PMID 15802099.
  8. Norton JA, Jensen RT (2003). "Current surgical management of Zollinger-Ellison syndrome (ZES) in patients without multiple endocrine neoplasia-type 1 (MEN1)". Surg Oncol. 12 (2): 145–51. PMID 12946485.
  9. Norton JA, Alexander HR, Fraker DL, Venzon DJ, Gibril F, Jensen RT (2001). "Comparison of surgical results in patients with advanced and limited disease with multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome". Ann Surg. 234 (4): 495–505, discussion 505-6. PMC 1422073. PMID 11573043.
  10. Epelboym I, Mazeh H (2014). "Zollinger-Ellison syndrome: classical considerations and current controversies". Oncologist. 19 (1): 44–50. doi:10.1634/theoncologist.2013-0369. PMC 3903066. PMID 24319020.

Template:WH Template:WS