CD Markers
CD117

Author: Nat Pernick, M.D. (see Authors page)

Revised: 30 October 2015, last major update August 2013

Copyright: (c) 2003-2015, PathologyOutlines.com, Inc.

PubMed Search: CD117[title]
Cite this page: CD117. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cdmarkerscd117.html. Accessed December 7th, 2016.
Definition / General
  • Summary: proto-oncogene activated in GIST tumors
  • Aka c-kit, stem cell factor receptor
  • Gene at 4q11-21
  • Receptor for kit protein, a 145 kD tyrosine kinase growth factor receptor protein important for development and survival of mast cells, hematopoietic stem cells, melanocytes, germ cells, interstitial cells of Cajal
  • Has activating or gain of function mutations in most GIST tumors, often at exon 11, less often at exons 9 and 13 (Hum Pathol 2002;33:484)
  • Tyrosine kinase activity of c-kit in GIST and bcl-abl overexpression in CML are inhibited by imatinib mesylate (Gleevec, STI571), a tyrosine kinase inhibitor used to treat these diseases (Hum Pathol 2002;33:466)
Uses by Pathologists
  • Confirming diagnosis of GIST (but see other CD117+ tumors below)
  • Possibly confirm chronic intestinal pseudo-obstruction (Am J Surg Pathol 2003;27:228)
Interpretation
  • Should be strong and diffuse cytoplasmic staining, like the positive control
Micro Images

Images hosted on other servers:

GIST vs. solitary fibrous tumor

Malignant stromal tumor
of gallbladder (figure 4)



Images hosted on Nature.com:

GIST (figures G, H)

Interstitial cells of Cajal in patient
with multiple GIST tumors

Positive Staining - Normal
  • Interstitial cells of Cajal, hematopoietic progenitor cells, melanocytes, embryonic/fetal brain, endothelium, gonads, mast cells, breast epithelium, germ cells
Positive staining - Tumors
  • AML, angiomyolipoma (Am J Surg Pathol 2002;26:493), angiosarcomas (50%), clear cell sarcoma, CML, epithelioid sarcoma, Ewing sarcoma, gastrointestinal autonomic tumor (GANT), gastrointestinal stromal tumor (GIST), granulocytic sarcoma, Hodgkin lymphoma (some Reed-Sternberg cells), intraabdominal fibromatosis (depends on antibody used, Am J Surg Pathol 2001;25:549), mast cell disease (also positive for tryptase, CD43, CD68); melanoma, mesenteric fibromatosis (variable, Am J Surg Pathol 2002;26:1296), metanephric adenosarcoma (Am J Surg Pathol 2001;25:1451), omental mesenchymal tumor, osteosarcoma, rhabdomyosarcoma, sclerosing mesenteritis (variable), seminomas /dysgerminomas, small cell lung cancer (Hum Pathol 2002;33:1182), synovial sarcoma (~10%, usually cytoplasmic staining), adenoid cystic carcinoma of salivary gland (strong staining)
Negative Staining
  • Alveolar soft part sarcoma, desmoplastic small round cell tumor, glomus tumor, leiomyoma (retroperitoneal, colorectal), leiomyosarcoma, myxoma (cardiac), schwannoma (colorectal), smooth muscle tumor of uncertain malignant potential, solitary fibrous tumor