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IGF-2 mediated hypoglycemia and the paradox of an apparently benign lesion: a case report & review of the literature
BACKGROUND: Non-islet cell tumour hypoglycemia (NICTH) is rarely encountered in clinical practice. Insulin-like growth factor 2 (IGF2) is the most common cause of NICTH observed in the setting of mesenchymal and epithelial neoplasia. This is a paraneoplastic syndrome caused by IGF2 activation of the...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9615362/ https://www.ncbi.nlm.nih.gov/pubmed/36303203 http://dx.doi.org/10.1186/s12902-022-01175-4 |
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author | Crowley, Mairead T. Lonergan, Eibhlin O’Callaghan, Peter Joyce, Caroline M. Morita, M. Conlon, Niamh O’Halloran, Domhnall J. |
author_facet | Crowley, Mairead T. Lonergan, Eibhlin O’Callaghan, Peter Joyce, Caroline M. Morita, M. Conlon, Niamh O’Halloran, Domhnall J. |
author_sort | Crowley, Mairead T. |
collection | PubMed |
description | BACKGROUND: Non-islet cell tumour hypoglycemia (NICTH) is rarely encountered in clinical practice. Insulin-like growth factor 2 (IGF2) is the most common cause of NICTH observed in the setting of mesenchymal and epithelial neoplasia. This is a paraneoplastic syndrome caused by IGF2 activation of the insulin receptor. CASE PRESENTATION: An 80 year old female presented with a short history of recurrent episodes of confusion with laboratory confirmed hypoglycemia with a plasma glucose of 2.7 mmol/L on fasting which fulfilled Whipple’s triad. Diagnostic clues to the aetiology at presentation include the fasting pattern of hypoglycemia, hypokalaemia and the absence of weight gain. A 72 hour fast with results showed early hypoglycemia and suppression of serum insulin, c-peptide, and proinsulin. Serum insulin antibody was not detected. Subsequent measurement of the serum IGF2:IGF1 ratio was elevated at 22.3 and consistent with IGF-2 mediated hypoglycemia and imaging studies demonstrated a pelvic mass. Dietary intervention and oral prednisolone abated hypoglycemia prior to surgery. Ultimately, hypoglycemia resolved following operative intervention and steroid therapy was successfully withdrawn. Histopathology was remarkable for dual neoplastic processes with uterine solitary fibrous tumour (SFT) confirmed as the source of IGF2 hypersecretion on IGF-2 immunohistochemistry and a coincidental invasive high grade serous carcinoma involving the fimbria of the right fallopian tube. CONCLUSION: The paradox in this case is that the benign solitary fibrous tumour accounted for patient morbidity through secretion of IGF2 and without treatment, posed a mortality risk. This is despite the synchronous presence of a highly malignant fallopian tube neoplasm. This case reinforces the need for thorough clinical evaluation of hypoglycemia to allow prompt and definitive management. |
format | Online Article Text |
id | pubmed-9615362 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-96153622022-10-29 IGF-2 mediated hypoglycemia and the paradox of an apparently benign lesion: a case report & review of the literature Crowley, Mairead T. Lonergan, Eibhlin O’Callaghan, Peter Joyce, Caroline M. Morita, M. Conlon, Niamh O’Halloran, Domhnall J. BMC Endocr Disord Case Report BACKGROUND: Non-islet cell tumour hypoglycemia (NICTH) is rarely encountered in clinical practice. Insulin-like growth factor 2 (IGF2) is the most common cause of NICTH observed in the setting of mesenchymal and epithelial neoplasia. This is a paraneoplastic syndrome caused by IGF2 activation of the insulin receptor. CASE PRESENTATION: An 80 year old female presented with a short history of recurrent episodes of confusion with laboratory confirmed hypoglycemia with a plasma glucose of 2.7 mmol/L on fasting which fulfilled Whipple’s triad. Diagnostic clues to the aetiology at presentation include the fasting pattern of hypoglycemia, hypokalaemia and the absence of weight gain. A 72 hour fast with results showed early hypoglycemia and suppression of serum insulin, c-peptide, and proinsulin. Serum insulin antibody was not detected. Subsequent measurement of the serum IGF2:IGF1 ratio was elevated at 22.3 and consistent with IGF-2 mediated hypoglycemia and imaging studies demonstrated a pelvic mass. Dietary intervention and oral prednisolone abated hypoglycemia prior to surgery. Ultimately, hypoglycemia resolved following operative intervention and steroid therapy was successfully withdrawn. Histopathology was remarkable for dual neoplastic processes with uterine solitary fibrous tumour (SFT) confirmed as the source of IGF2 hypersecretion on IGF-2 immunohistochemistry and a coincidental invasive high grade serous carcinoma involving the fimbria of the right fallopian tube. CONCLUSION: The paradox in this case is that the benign solitary fibrous tumour accounted for patient morbidity through secretion of IGF2 and without treatment, posed a mortality risk. This is despite the synchronous presence of a highly malignant fallopian tube neoplasm. This case reinforces the need for thorough clinical evaluation of hypoglycemia to allow prompt and definitive management. BioMed Central 2022-10-27 /pmc/articles/PMC9615362/ /pubmed/36303203 http://dx.doi.org/10.1186/s12902-022-01175-4 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Case Report Crowley, Mairead T. Lonergan, Eibhlin O’Callaghan, Peter Joyce, Caroline M. Morita, M. Conlon, Niamh O’Halloran, Domhnall J. IGF-2 mediated hypoglycemia and the paradox of an apparently benign lesion: a case report & review of the literature |
title | IGF-2 mediated hypoglycemia and the paradox of an apparently benign lesion: a case report & review of the literature |
title_full | IGF-2 mediated hypoglycemia and the paradox of an apparently benign lesion: a case report & review of the literature |
title_fullStr | IGF-2 mediated hypoglycemia and the paradox of an apparently benign lesion: a case report & review of the literature |
title_full_unstemmed | IGF-2 mediated hypoglycemia and the paradox of an apparently benign lesion: a case report & review of the literature |
title_short | IGF-2 mediated hypoglycemia and the paradox of an apparently benign lesion: a case report & review of the literature |
title_sort | igf-2 mediated hypoglycemia and the paradox of an apparently benign lesion: a case report & review of the literature |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9615362/ https://www.ncbi.nlm.nih.gov/pubmed/36303203 http://dx.doi.org/10.1186/s12902-022-01175-4 |
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