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Related Concept Videos

Hypoglycemia and Glucagon01:15

Hypoglycemia and Glucagon

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Without prolonged fasting, healthy individuals maintain blood glucose levels above 3.5 mM due to a well-adapted neuroendocrine counterregulatory system that effectively prevents acute hypoglycemia, a potentially life-threatening condition. The primary clinical scenarios for hypoglycemia encompass diabetes treatment, inappropriate production of endogenous insulin or insulin-like substances by tumors, and the use of glucose-lowering agents in non-diabetic individuals. Notably, hypoglycemia in the...
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Diabetes Mellitus: Overview and Type I Subtype01:22

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Diabetes mellitus is a chronic metabolic disorder characterized by high blood glucose levels due to inadequate insulin production, insulin resistance, or both. The condition affects millions worldwide and can significantly impact their health and quality of life.
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The pancreatic islets comprising only 1%-2% of the volume are highly vascularized and innervated mini-organs. They contain five endocrine cell types, including β cells that secrete insulin, which is synthesized as a single polypeptide chain, preproinsulin, processed to proinsulin, and finally to insulin and C-peptide. This process is complex and regulated, involving the Golgi complex, the endoplasmic reticulum, and the secretory granules of the β cell.
Insulin and C-peptide are...
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Well-differentiated liposarcoma causing non-islet cell tumor hypoglycemia.

Yosuke Igarashi1, Hiroshi Hirukawa1, Tetsuya Nakano1

  • 1Department of Surgery, Tachikawa General Hospital, 1-24, Asahioka Nagaoka-shi, Niigata, 940-8621 Japan.

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|June 7, 2022
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Summary

Non-islet cell tumor hypoglycemia (NICTH), a rare paraneoplastic syndrome, can be caused by liposarcoma. Surgical removal of a large well-differentiated liposarcoma resolved severe hypoglycemia in a patient, highlighting the importance of considering NICTH in tumor cases.

Keywords:
HypoglycemiaIGF-IINon-islet cell tumor hypoglycemiaWell-differentiated liposarcoma

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Area of Science:

  • Endocrinology
  • Oncology
  • Surgical Pathology

Background:

  • Non-islet cell tumor hypoglycemia (NICTH) is a rare paraneoplastic syndrome characterized by severe hypoglycemia, often due to tumors producing insulin-like growth factor-II (IGF-II).
  • While various tumors can cause NICTH, liposarcoma (LPS) is an infrequent etiology.

Observation:

  • A 38-year-old male presented with severe hypoglycemia, vomiting, and abdominal distension due to a large 40-cm mesenteric mass.
  • Initial investigations revealed suppressed insulin and C-peptide levels, with low IGF-I, suggesting tumor-induced hypoglycemia.

Findings:

  • The patient was diagnosed with NICTH secondary to a large, well-differentiated liposarcoma (WDLPS).
  • Post-surgical resection, preoperative high-molecular-weight IGF-II levels normalized, confirming the tumor as the source.
  • The patient remained hypoglycemia-free for 4 years following surgical intervention.

Implications:

  • This case underscores the importance of considering NICTH in patients presenting with unexplained severe hypoglycemia and significant abdominal tumors.
  • Prompt surgical management of the underlying liposarcoma is crucial for resolving hypoglycemia and improving patient outcomes.
  • Early diagnosis and treatment of LPS-induced NICTH can prevent severe complications and ensure long-term recovery.