Nonislet Cell Tumor Hypoglycemia: A Rare Paraneoplastic Syndrome

Nonislet cell tumor hypoglycemia (NICTH) is a rare and underreported cause of hypoglycemia due to excessive production of insulin-like growth factor 2 (IGF-2) and its intermediate forms, which activate the insulin receptor. Typically, certain malignancies can cause NICTH, usually as a paraneoplastic syndrome. Diagnosis requires a raised IGF-2/IGF-1 ratio. Surgery forms the cornerstone of management, while glucocorticoids are an alternative when surgery is not possible. We present a unique case of a 27-year-old male, who was a follow-up case of chronic hepatitis B infection and presented with a gall bladder fossa mass and recurrent, severe episodes of hypoglycemia. Workup revealed low insulin and C-peptide and suppressed IGF-1 with normal pituitary function. Clinicians should consider the possible diagnosis of NICTH when laboratory and immunohistochemical (IHC) data do not support more common causes, such as insulinoma.

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Journal
Journal of the Association of Physicians of India
Published
2025-02-01
DOI
https://doi.org/10.59556/japi.73.0837
Primary Topic
Neuroendocrine Tumor Research Advances
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article
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article

Nonislet Cell Tumor Hypoglycemia: A Rare Paraneoplastic Syndrome

Anupama Gaur, Prabhat Narain Sharma, Shyam Sunder
Journal of the Association of Physicians of India
Neuroendocrine Tumor Research Advances
article

Nonislet Cell Tumor Hypoglycemia: A Rare Paraneoplastic Syndrome

Anupama Gaur, Prabhat Narain Sharma, Shyam Sunder
article en

Abstract

Nonislet cell tumor hypoglycemia (NICTH) is a rare and underreported cause of hypoglycemia due to excessive production of insulin-like growth factor 2 (IGF-2) and its intermediate forms, which activate the insulin receptor. Typically, certain malignancies can cause NICTH, usually as a paraneoplastic syndrome. Diagnosis requires a raised IGF-2/IGF-1 ratio. Surgery forms the cornerstone of management, while glucocorticoids are an alternative when surgery is not possible. We present a unique case of a 27-year-old male, who was a follow-up case of chronic hepatitis B infection and presented with a gall bladder fossa mass and recurrent, severe episodes of hypoglycemia. Workup revealed low insulin and C-peptide and suppressed IGF-1 with normal pituitary function. Clinicians should consider the possible diagnosis of NICTH when laboratory and immunohistochemical (IHC) data do not support more common causes, such as insulinoma.

Journal of the Association of Physicians of IndiaVol. 73(2)
Employees' State Insurance Model Hospital (IN)
Good health and well-being
Openalex Percentile: Top 96%
Neuroendocrine Tumor Research Advances
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Nonislet Cell Tumor Hypoglycemia: A Rare Paraneoplastic Syndrome — Anupama Gaur, Prabhat Narain Sharma, et al. · Journal of the Association of Physicians of India (2025) | TGRS Research Map | TGRS