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Author Notes:

Email: Guillermo A Escobar - guillermo.escobar@uchsc.edu; William A Robinson - william.robinson@uchsc.edu; Trevor L Nydam - Trevor.nydam@uchsc.edu; Drew C Heiple - drew.heiple@uchsc.edu; Glen J Weiss - glen.weiss@uchsc.edu; Linda Buckley - linda.buckley@uchsc.edu; Rene Gonzalez - rene.gonzalez@uchsc.edu; Martin D McCarter* - martin.mccarter@uchsc.edu

GAE conceived and drafted the manuscript, obtained references, participated in its design and coordination and edited the figures and legends.

WAR directed and obtained the GIST mutational analysis and participated in its design and editing.

TLN participated in its drafting, references, editing and design.

DCH contributed to the patient's care and contributed to the conception and drafting of the manuscript.

GJW Performed the GIST mutational analysis and participated in its design and coordination. LB contributed to the patient's care and coordinated communication with the University Medical Center Utrecht, Utrecht, The Netherlands for obtaining molecular analysis of IGF.

RG contributed to the patient's care and participated in its editing, design and coordination. MDM contributed to the patient's care, performed surgical resection, obtained operative photos and participated in its design, editing and coordination.

All authors read and approved the final manuscript.

We appreciate Dr. Jaap van Doorn and colleagues (University Medical Center Utrecht, Utrecht, The Netherlands) for analyzing and providing the images of the patient's tissue and serum analysis for IGF II and "big" IGF II.

Written consent was obtained from the patient for the publication of this report.

The author(s) declare that they have no competing interests.

Subjects:

Keywords:

  • Science & Technology
  • Life Sciences & Biomedicine
  • Oncology
  • ONCOLOGY
  • ISLET CELL TUMOR
  • GROWTH-FACTOR-II
  • GERMLINE MUTATION
  • KINASE
  • IMATINIB
  • BINDING

Severe paraneoplastic hypoglycemia in a patient with a gastrointestinal stromal tumor with an exon 9 mutation: a case report

Tools:

Journal Title:

BMC Cancer

Volume:

Volume 7

Publisher:

, Pages 13-13

Type of Work:

Article | Final Publisher PDF

Abstract:

Background: Non-islet cell tumor induced hypoglycemia (NICTH) is a very rare phenomenon, but even more so in gastrointestinal stromal tumors. It tends to present in large or metastatic tumors, and can appear at any time in the progression of the disease. We present herein a case of NICTH in a GIST tumor and report an exon 9 mutation associated to it. Case presentation: A thirty nine year-old man with a recurrent, metastatic gastrointestinal stromal tumor presented to the hospital with nausea, dizziness, loss of consciousness, and profound hypoglycemia (20 mg/dL). There was no evidence of factitious hypoglycemia. He was stabilized with a continuous glucose infusion and following selective vascular embolization, the patient underwent debulking of a multicentric 40 cm × 25 cm × 10 cm gastrointestinal stromal tumor. After resection, the patient became euglycemic and returned to his normal activities. Tumor analysis confirmed excessive production of insulin-like growth factor II m-RNA and the precursor protein, "big" insulin-like growth factor II. Mutational analysis also identified a rare, 6 bp tandem repeat insert (gcctat) at position 1530 in exon 9 of KIT. Conclusion: Optimal management of gastrointestinal stromal tumor-induced hypoglycemia requires a multidisciplinary approach, and surgical debulking is the treatment of choice to obtain immediate symptom relief. Imatinib or combinations of glucocorticoids and growth hormone are alternative palliative strategies for symptomatic hypoglycemia. In addition, mutations in exon 9 of the tyrosine kinase receptor KIT occur in 11-20% of GIST and are often associated with poor patient outcomes. The association of this KIT mutation with non-islet cell tumor induced hypoglycemia has yet to be established.

Copyright information:

© 2007 Escobar et al; licensee BioMed Central Ltd.

This is an Open Access work distributed under the terms of the Creative Commons Attribution 2.0 Generic License (http://creativecommons.org/licenses/by/2.0/).

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