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Refractory hypoglycemia in a pediatric patient with desmoplastic small round cell tumor

  • Abdullah Almaghraby EMAIL logo , Wendy J. Brickman , Jeffery A. Goldstein and Reema L. Habiby
Published/Copyright: July 25, 2018

Abstract

Background

Tumor-induced hypoglycemia is a rare and serious complication that is usually a consequence of either excessive insulin secretion (insulinoma) or because of non-islet cell tumor hypoglycemia (NICTH). NICTH is a rare phenomenon seen most often in adult patients. It is associated with different tumor types. Here, we report the first case to the best of our knowledge in the literature of a pediatric patient with NICTH associated with desmoplastic small round cell tumor (DSRT).

Case presentation

This is a 15-year-old girl who presented with symptomatic hypoglycemia and abdominal mass. She required an intravenous glucose infusion rate as high as 9 mg/kg/min in addition to glucose containing oral supplements in order to maintain her blood glucose above 60 mg/dL. Computed tomography (CT) scan of the chest, abdomen and pelvis showed multiple hepatic lesions with an intraperitoneal soft tissue mass which subsequently was diagnosed as DSRT. When the blood glucose was 45 mg/dL, the insulin, growth hormone (GH) and insulin-like growth factor-1 (IGF-1) levels were suppressed with an appropriate elevation of cortisol. Subsequently, an insulin-like growth factor-2 (IGF-2) level was sent and the IGF-2:IGF-1 ratio was found to be elevated >10 consistent with NICTH. After the first dose of chemotherapy, hypoglycemia improved, and she was weaned off glucose containing fluids.

Conclusions

NICTH should be considered in all cancer patients regardless of their age with refractory hypoglycemia.


Corresponding author: Abdullah Almaghraby, MD, Department of Pediatric Endocrinology, Division of Endocrinology, Ann and Robert H. Lurie Children’s Hospital of Chicago, Northwestern University Feinberg School of Medicine, 225 E Chicago Avenue, Box 54, Chicago, IL 60611-2605, USA, Phone: +312-227-6090

  1. Author contributions: Abdullah Almaghraby: Dr. Almaghraby took direct care of the patient, conducted the literature review, drafted the initial manuscript and edited the manuscript according to feedback of the other authors. Wendy J. Brickman: Dr. Brickman took direct care of the patient, provided feedback and reviewed and revised the manuscript. Jeffery A. Goldstein: Dr. Goldstein reviewed the histopathology to assist with confirmation of the diagnosis and created the histopathology figures and legends. Reema L. Habiby: Dr. Habiby took direct care of the patient, provided feedback and critically reviewed the manuscript. All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.

  2. Research funding: None declared.

  3. Employment or leadership: None declared.

  4. Honorarium: None declared.

  5. Competing interests: The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.

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Received: 2018-03-01
Accepted: 2018-06-11
Published Online: 2018-07-25
Published in Print: 2018-08-28

©2018 Walter de Gruyter GmbH, Berlin/Boston

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