Refractory hypoglycemia in a pediatric patient with desmoplastic small round cell tumor
-
Abdullah Almaghraby
,
Wendy J. Brickman
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.
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.
Research funding: None declared.
Employment or leadership: None declared.
Honorarium: None declared.
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.
References
1. Zapf J, Futo E, Peter M, Froesch ER. Can “big” insulin-like growth factor II in serum of tumor patients account for the development of extrapancreatic tumor hypoglycemia? J Clin Invest 1992;90:2574–84.10.1172/JCI116152Search in Google Scholar PubMed PubMed Central
2. Dufresne A, Cassier P, Couraud L, Marec-Bérard P, Meeus P, et al. Desmoplastic small round cell tumor: current management and recent findings. Sarcoma 2012;2012:5.10.1155/2012/714986Search in Google Scholar PubMed PubMed Central
3. Marks V, Teale JD. Tumours producing hypoglycaemia. Endocr Relat Cancer 1998;5:111–29.10.1677/erc.0.0050111Search in Google Scholar
4. Livingstone C. IGF2 and cancer. J Clin Invest 2013;20:R321–39.10.1530/ERC-13-0231Search in Google Scholar PubMed
5. Hizuka N, Fukuda I, Takano K, Okubo Y, Asakawa-Yasumoto K, et al. Serum insulin-like growth factor II in 44 patients with non-islet cell tumor hypoglycemia. Endocr J 1998;45 Suppl:S61–5.10.1507/endocrj.45.Suppl_S61Search in Google Scholar
6. Barra WF, Castro G, Hoff AO, Siqueira SA, Hoff PM. Symptomatic hypoglycemia related to inappropriately high IGF-II serum levels in a patient with desmoplastic small round cell tumor. Case Rep Med 2010;2010:684045.10.1155/2010/684045Search in Google Scholar PubMed PubMed Central
7. Daughaday WH, Emanuele MA, Brooks MH, Barbato AL, Kapadia M, et al. Synthesis and secretion of insulin-like growth factor II by a leiomyosarcoma with associated hypoglycemia. N Engl J Med 1988;319:1434–40.10.1056/NEJM198812013192202Search in Google Scholar PubMed
8. Rother KI, Accili D. Role of insulin receptors and IGF receptors in growth and development. Pediatr Nephrol 2000;14:558–61.10.1007/s004670000351Search in Google Scholar PubMed
9. Dynkevich Y, Rother KI, Whitford I, Qureshi I, Galiveeti S, et al. Tumors, IGF-2, and hypoglycemia: insights from the clinic, the laboratory, and the historical archive. Endocr Rev 2013;34:798–826.10.1210/er.2012-1033Search in Google Scholar PubMed
10. Drummond IA, Madden SL, Rohwer-Nutter P, Bell GI, Sukhatme VP, et al. Repression of the insulin-like growth factor II gene by the Wilms tumor suppressor WT1. Science 1992;257:674–8.10.1126/science.1323141Search in Google Scholar PubMed
11. Dutta P, Aggarwal A, Gogate Y, Nahar U, Shah VN, et al. Non-islet cell tumor-induced hypoglycemia: a report of five cases and brief review of the literature. Endocrinol Diabetes Metab Case Rep 2013;2013:130046.10.1530/EDM-13-0046Search in Google Scholar PubMed PubMed Central
12. Daughaday WH, Trivedi B. Heterogeneity of serum peptides with immunoactivity detected by a radioimmunoassay for proinsulin-like growth factor-II E domain: description of a free E domain peptide in serum. J Clin Endocrinol Metab 1992;75:641–5.10.1210/jcem.75.2.1379260Search in Google Scholar
©2018 Walter de Gruyter GmbH, Berlin/Boston
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Articles in the same Issue
- Frontmatter
- Original Articles
- Urinary bisphenol-A levels in children with type 1 diabetes mellitus
- The relationship between metabolic syndrome, cytokines and physical activity in obese youth with and without Prader-Willi syndrome
- Association of anthropometric measures and cardio-metabolic risk factors in normal-weight children and adolescents: the CASPIAN-V study
- The effect of obesity and insulin resistance on macular choroidal thickness in a pediatric population as assessed by enhanced depth imaging optical coherence tomography
- Hereditary 1,25-dihydroxyvitamin D-resistant rickets (HVDRR): clinical heterogeneity and long-term efficacious management of eight patients from four unrelated Arab families with a loss of function VDR mutation
- Long-term thyroid disorders in pediatric survivors of hematopoietic stem cell transplantation after chemotherapy-only conditioning
- Screening for autoimmune thyroiditis and celiac disease in minority children with type 1 diabetes
- Resistance exercise alone improves muscle strength in growth hormone deficient males in the transition phase
- Sequential measurements of IGF-I serum concentrations in adolescents with Laron syndrome treated with recombinant human IGF-I (rhIGF-I)
- Symptomatic Rathke cleft cyst in paediatric patients – clinical presentations, surgical treatment and postoperative outcomes – an analysis of 38 cases
- Molecular genetics of tetrahydrobiopterin deficiency in Chinese patients
- Single center experience of biotinidase deficiency: 259 patients and six novel mutations
- Next-generation sequencing as a second-tier diagnostic test for newborn screening
- Case Reports
- Thyroid storm after choking
- Three new Brazilian cases of 17α-hydroxylase deficiency: clinical, molecular, hormonal, and treatment features
- Diazoxide toxicity in a child with persistent hyperinsulinemic hypoglycemia of infancy: mixed hyperglycemic hyperosmolar coma and ketoacidosis
- Refractory hypoglycemia in a pediatric patient with desmoplastic small round cell tumor
