A Case of Secretin-responsive Insulinoma with Low Serum C-peptide Levels

• Published in: Endocrine Journal Vol. 54; no. 1; pp. 113 - 121
• Main Authors: FUJIKURA, Junji, HOSODA, Kiminori, NOGUCHI, Michio, EBIHARA, Ken, MASUZAKI, Hiroaki, HIRATA, Masakazu, FUJIMOTO, Koji, DOI, Ryuichiro, IWANISHI, Masanori, NAKAO, Kazuwa
• Format: Journal Article
• Language: English
• Published: Japan The Japan Endocrine Society 2007
• Subjects: Blood Glucose - analysis; C-peptide; C-Peptide - blood; Cathepsin B; Female; Humans; Insulin - blood; Insulinoma; Insulinoma - blood; Insulinoma - drug therapy; Insulinoma - surgery; Middle Aged; Pancreatic Neoplasms - blood; Pancreatic Neoplasms - drug therapy; Pancreatic Neoplasms - surgery; Secretin; Secretin - therapeutic use
• ISSN: 0918-8959; 1348-4540
• DOI: 10.1507/endocrj.K06-148

Insulinoma is the most common cause of fasting hypoglycemia resulting from autonomous insulin hypersecretion. A 59-year-old woman who had previously had an insulinoma and had undergone a partial pancreatectomy was admitted to our hospital because of recurrence of hypoglycemia after 27 years. She had two unusual endocrinological features: 1) the serum insulin response to intravenous secretin injection was not impaired, and 2) the serum C-peptide levels and ratios of serum C-peptide to insulin were relatively low. Two pancreatic tumors were readily detectable by computed tomography (CT) and magnetic resonance imaging (MRI). The selective arterial calcium injection (SACI) test showed a hyperinsulinemic response by calcium administration to the gastroduodenal artery. A partial pancreatectomy was done and her hypoglycemia disappeared. Histology revealed that the tumors were composed of monotonous, small round cells that were positive for both insulin and cathepsin B. As previous in vitro studies have shown that C-peptide can be metabolized within human insulinoma cells by proteolytic cleavage by cathepsin B, our patient's low serum C-peptide levels might have been caused by degradation of C-peptide by cathepsin B. According to the data from the literature, the molar ratio of serum C-peptide to insulin is generally decreased in patients with insulinoma than normal subjects. This case highlights the need for careful interpretation of C-peptide levels and the intravenous secretin injection test in the diagnosis of insulinoma.