IGF-2 mediated hypoglycemia and the paradox of an apparently benign lesion: a case report & review of the literature.
Hypoglycemia
Insulin-like growth factor 2
Non-islet cell tumour hypoglycemia
Journal
BMC endocrine disorders
ISSN: 1472-6823
Titre abrégé: BMC Endocr Disord
Pays: England
ID NLM: 101088676
Informations de publication
Date de publication:
27 Oct 2022
27 Oct 2022
Historique:
received:
22
07
2021
accepted:
04
10
2022
entrez:
28
10
2022
pubmed:
29
10
2022
medline:
1
11
2022
Statut:
epublish
Résumé
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. 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 Ultimately, hypoglycemia resolved following operative intervention and steroid therapy was successfully withdrawn.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. 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.
Sections du résumé
BACKGROUND
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
METHODS
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
CONCLUSIONS
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.
Identifiants
pubmed: 36303203
doi: 10.1186/s12902-022-01175-4
pii: 10.1186/s12902-022-01175-4
pmc: PMC9615362
doi:
Substances chimiques
Insulin-Like Growth Factor II
67763-97-7
Insulins
0
Types de publication
Review
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
262Informations de copyright
© 2022. The Author(s).
Références
Pediatrics. 1976 May;57(5):702-11
pubmed: 940710
J Clin Endocrinol Metab. 2009 Mar;94(3):709-28
pubmed: 19088155
Clin Endocrinol (Oxf). 1990 Jul;33(1):87-98
pubmed: 2205424
QJM. 2014 Apr;107(4):297-300
pubmed: 22171014
Eur J Endocrinol. 1998 Nov;139(5):532-6
pubmed: 9849819
J Clin Endocrinol Metab. 2007 May;92(5):1600-5
pubmed: 17299065
Growth Horm IGF Res. 2006 Aug;16(4):211-6
pubmed: 16860583
Endocr Rev. 2013 Dec;34(6):798-826
pubmed: 23671155
Am J Surg Pathol. 1989 Aug;13(8):640-58
pubmed: 2665534
Front Endocrinol (Lausanne). 2019 May 15;10:316
pubmed: 31156561
Clin Endocrinol (Oxf). 2004 Apr;60(4):457-60
pubmed: 15049960
Cancer. 1998 Apr 15;82(8):1585-92
pubmed: 9554538
Ann Endocrinol (Paris). 2020 Jun;81(2-3):110-117
pubmed: 32409005
J Clin Endocrinol Metab. 2009 Jul;94(7):2226-31
pubmed: 19383775
Endocr Relat Cancer. 2007 Dec;14(4):979-93
pubmed: 18045950
Endocr J. 2017 Jul 28;64(7):719-726
pubmed: 28529277
J Clin Endocrinol Metab. 1982 Nov;55(5):833-9
pubmed: 6749876
Am J Med. 1969 Aug;47(2):220-35
pubmed: 4309111
Clin Endocrinol (Oxf). 1995 Apr;42(4):433-5
pubmed: 7750199
Ann Intern Med. 1988 Feb;108(2):252-7
pubmed: 3277509
Eur J Endocrinol. 2014 Mar 14;170(4):R147-57
pubmed: 24459236
Int J Clin Oncol. 2006 Dec;11(6):478-81
pubmed: 17180519
Br J Cancer. 2006 Oct 9;95(7):853-61
pubmed: 16953241
N Engl J Med. 1988 Dec 1;319(22):1434-40
pubmed: 3185662
Ann Endocrinol (Paris). 2019 Feb;80(1):21-25
pubmed: 29555080