Successful Imatinib Therapy of Neuroendocrine Carcinoma with Activating KIT Mutation: A Case Study

Neuroendocrine tumors (NET) and gastrointestinal stromal tumors (GIST) are believed to originate from the cells of Cajal that are randomly dispersed along the aerodigestive tract. Despite their distinct morphologic appearance, NET and GIST may share oncogenic mechanisms. Often presenting in the metastatic setting, treatment options for patients with NET are limited. This case report presents a patient with refractory metastatic NET that did not respond conventional chemotherapy. The patient was treated with a KIF11 inhibitor in a phase I clinical trial and experienced a prolonged and clinically meaningful partial response. On progression at 20 months, the patient’s tumor was sequenced to reveal a KIT exon 11 mutation. Institution of imatinib therapy achieved a rapid and sustained antitumor effect with profound clinical benefit. Despite previously reported KIT expression in NET, this is the first documented case of an activating KIT mutation in NET and of successful treatment with both a KIF11 inhibitor and imatinib, each of which was elucidated through molecular profiling of the patient’s tumor. Imatinib may be a valuable therapy in NET harboring activating KIT mutations.

Target Audience

This activity has been designated to meet the educational needs of physicians and nurses involved in the management of patients with cancer.

Learning Objectives

Upon completion of this activity, participants will be able to:

  • Summarize the criteria on which the classification of neuroendocrine tumors (NET) is based and list some markers for NET
  • Describe the rationale for the use of imatinib in the treatment of patients with neuroendocrine tumors with activating KIT mutations
  • Evaluate the data supporting the hypothesis that NET and gastrointestinal stromal tumors (GIST) arise from a common precursor
Additional information
Supporters: 

No commercial support was received for this article.

Course summary
Available credit: 
  • 1.00 Participation
  • 1.00 Nurse
  • 1.00 Physician
Course opens: 
06/16/2014
Course expires: 
06/16/2015
Cost:
$0.00
James Perkins, MD
Department of Medical Oncology
Fox Chase Cancer Center
Philadelphia, Pennsylvania
Yan Zhou, PhD
Biostatistics and Bioinformatics Facility
Fox Chase Cancer Center
Philadelphia, Pennsylvania
Patrick Boland, MD
Department of Medicine, Division of Medical Oncology
Roswell Park Cancer Institute
Buffalo, New York
Paul Engstrom, MD
Department of Medical Oncology
Fox Chase Cancer Center
Philadelphia, Pennsylvania
Steven J. Cohen, MD
Department of Medical Oncology
Fox Chase Cancer Center
Philadelphia, Pennsylvania
Igor Astsaturov, MD, PhD
Department of Medical Oncology
Fox Chase Cancer Center
Philadelphia, Pennsylvania
Anthony J. Olszanski, RPh, MD
Department of Medical Oncology
Fox Chase Cancer Center
Philadelphia, Pennsylvania
 

Available Credit

  • 1.00 Participation
  • 1.00 Nurse
  • 1.00 Physician

Accreditation Period

Course opens: 
06/16/2014
Course expires: 
06/16/2015

Price

Cost:
$0.00
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Required Hardware/software

To access this activity, users will need:

  • A device with an Internet connection
  • Adobe Reader or other PDF reader software for article and certificate viewing/printing