Gastrointestinal Stromal Tumors (GISTs)
Zollinger-Ellison Syndrome (Gastrinoma)
Gastrointestinal Stromal Tumors (GISTs)
Definition:
Gastrointestinal stromal tumor (GIST) is a potentially malignant tumor and the most frequent type of sarcoma in the gastrointestinal tract. They are usually found in stomach (60%), small intestine, jejunum and ileum (30%), duodenum (5%), rectum (2–3%) and colon (1–2%).
Panel test:
- CD117
- DOG1
- BRAF
- SDHB
- CD34
- Caldesmon
- Smooth Muscle Actin (SMA)
- Desmin
- S-100
- Glial Fibrillary Acidic protein (GFAP)
- Beta-catenin-1
References
Guidelines
- ESMO/European Sarcoma Network Working Group. Gastrointestinal stromal tumours: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up.Ann Oncol. 2014; 25 Suppl 3: iii21-6. PubMed
- NCCN Clinical Practice Guidelines in Oncology, Soft Tissue Sarcomas, Version 2.2018. National Comprehensive Cancer Network. Fort Washington, PA [Updated: Mar 2018; Accessed: Nov 2018]
- Hampel H, Bennett RL, Buchanan A, Pearlman R, Wiesner GL, Guideline Development Group, American College of Medical Genetics and Genomics Professional Practice and Guidelines Committee and National Society of Genetic Counselors Practice Guidelines Committee. A practice guideline from the American College of Medical Genetics and Genomics and the National Society of Genetic Counselors: referral indications for cancer predisposition assessment.Genet Med. 2015; 17(1): 70-87. PubMed
Zollinger-Ellison Syndrome (Gastrinoma)
Definition:
Zollinger-Ellison syndrome (ZES) is characterized by refractory peptic ulcer disease, diarrhea, and gastric acid hypersecretion as a result of a functional pancreatic or duodenal neuroendocrine tumor (NET). ZES is also referred to as gastrinoma.
Panel test:
- Gastrin
- Chromogranin
- Synaptophysin
- Ki-67
- Neuron Specific Enolase polyclonal (NSE P)
- Cytokeratin 7 (CK7)
- Pan Cytokeratin (AE1,3)
References
Guidelines
- Protocol for the Examination of Specimens from Patients with Neuroendocrine Tumors (Carcinoid Tumors) of the Small Intestine and Ampulla. Based on AJCC/UICC TNM, 7th ed. Protocol web posting date: Oct 2013. College of American Pathologists (CAP). Northfield, IL [Revised: Oct 2013; Accessed: Mar 2018]
- Protocol for the Examination of Specimens from Patients with Neuroendocrine Tumors (Carcinoid Tumors) of the Stomach. Based on AJCC/UICC TNM, 7th ed. Protocol web posting date: Oct 2013. College of American Pathologists (CAP). Northfield, IL [Revised: Jun 2014; Accessed: Mar 2018]
- Protocol for the Examination of Specimens from Patients with Tumors of the Endocrine Pancreas. Based on AJCC/UICC TNM, 7th ed. Protocol web posting date: Aug 2016. College of American Pathologists (CAP). Northfield, IL [Revised: Aug 2016; Accessed: July 2018]
- NCCN Clinical Practice Guidelines in Oncology, Neuroendocrine and Adrenal Tumors. Version 4.2018. National Comprehensive Cancer Network. Fort Washington, PA [Updated: May 2018; Accessed: Jul 2018]
- Vinik AI, Woltering EA, Warner RR, Caplin M, O’Dorisio TM, Wiseman GA, Coppola D, Go VL, North American Neuroendocrine Tumor Society (NANETS). NANETS consensus guidelines for the diagnosis of neuroendocrine tumor. 2010; 39(6): 713-34. PubMed
- Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ) – Health Professional Version. National Cancer Institute. [Updated: Feb 2018; Accessed: Aug 2018]
- Oberg K, Couvelard A, Fave GD, Gross D, Grossman A, Jensen RT, Pape U, Perren A, Rindi G, Ruszniewski P, Scoazec J, Welin S, Wiedenmann B, Ferone D, all other Antibes Consensus Conference participants. ENETS Consensus Guidelines for Standard of Care in Neuroendocrine Tumours: Biochemical Markers. 2017; PubMed
