Hypoglycemia mediated by paraneoplastic production of Insulin like growth factor–2 from a malignant renal solitary fibrous tumor – clinical case and literature review

BMC Endocrine Disorders

Amir Moheet¹  John Bantle¹  Brianna Johnson-Rabbett¹  Ameer Khowaja¹ 

[1] Department of Medicine, Division of Diabetes, Endocrinology and Metabolism, University of Minnesota, 516 Delaware St SE, Minneapolis, MN 55455, USA

关键词: Solitary fibrous tumor;    Insulinoma;    Paraneoplastic production;    Hypoglycemia;    Big IGF-2;    Insulin like Growth Factor–2 (IGF-2);    Non islet cell tumor hypoglycemia (NICTH);   

Others  :  864914
DOI  :  10.1186/1472-6823-14-49

 received in 2013-12-09, accepted in 2014-06-05,  发布年份 2014

【 摘 要 】

Background

Hypoglycemic episodes are infrequent in individuals without a history of diabetes mellitus or bariatric surgery. When hypoglycemia does occur in such individuals, an uncommon but important diagnosis to consider is non-islet cell tumor hypoglycemia (NICTH). We report a case of NICTH associated with paraneoplastic insulin-like growth factor-2 (IGF-2) production and review current relevant medical literature.

Case presentation

A 60 year old male with no relevant past medical history was referred to the endocrinology clinic with 18 month history of episodic hypoglycemic symptoms and, on one occasion was noted to have a fingerstick glucose of 36 mg/dL while having symptoms of hypoglycemia. Basic laboratory evaluation was unrevealing. Further evaluation however showed an elevated serum IGF-2 level at 2215 ng/mL (reference range 411–1248 ng/mL). Imaging demonstrated a large right suprarenal mass. A right nephrectomy with resection of the mass demonstrated a malignant solitary fibrous tumor. Post resection, the patient’s IGF-2 levels normalized and hypoglycemic symptoms resolved.

Conclusion

Due to the structural and biochemical homology between IGF-2 and insulin, elevated levels of IGF-2 can result in hypoglycemia. A posttranslational precursor to IGF-2 known as “big IGF” also possesses biologic activity. Review of recent reported cases of NICTH identified widespread anatomic locations and varied pathologic diagnoses of tumors associated with paraneoplastic production of IGF-2 causing hypoglycemia. Definitive management of hypoglycemia associated with paraneoplastic production of IGF-2 consists of resection of the tumor responsible for IGF-2 production. Accumulating literature provides a firm basis for routine IGF-2 laboratory evaluation in patients presenting with spontaneous hypoglycemia with no readily apparent cause.

【 授权许可】

   
2014 Khowaja et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 4.

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