Noninsulinoma Pancreatogenous Hypoglycemia Syndrome: A Rare Case of Adult-onset Nesidioblastosis

Abstract

The most common cause of hyperinsulinemic hypoglycemia in adults is insulinoma. Nesidioblastosis is a rare, but well-recognized disorder of persistent hyperinsulinemic hypoglycemia in infancy, but adult-onset nesidioblastosis associated with hyperinsulinemic hypoglycemia, termed noninsulinoma pancreatogenous hypoglycemic syndrome (NIPHS), has been reported. Here, we describe an extremely rare case of NIPHS in an elderly man. A 78-year-old man was admitted to our hospital due to hypoglycemic coma. During the previous 3 months, he noticed excessive sweating at midafternoon. His low fasting plasma glucose level (27 mg/dl) and high immunoreactive insulin level (11.1 U/ml) were consistent with the possible presence of insulinoma. Localizing studies including computed tomography of the abdomen and celiac arteriography were negative, but selective arterial calcium infusion (SACI) test suggested the presence of insulinoma in the body and tail of the pancreas. Surgical exploration by palpation and intraoperative ultrasonography failed to detect any mass in the pancreas, and 60% distal pancreatectomy was performed. Postoperatively, his hypoglycemic episodes completely disappeared. Histological examination of the resected pancreas revealed diffuse islet cell hyperplasia consistent with a pathological diagnosis of nesidioblastosis. Thus, our case is a very rare case of NIPHS, or adult-onset nesidioblastosis, in which SACI test was proven to be a useful diagnostic tool for localization of the pancreatic lesion. Key words: hyperinsulinemic hypoglycemia, nesidioblastosis, noninsulinoma pancreatogenous hypoglycemia syndrome (NPHS), selective arterial calcium injection (SACI)

A Clinical Case of Insulinoma Presenting with Postprandial Hypoglycemia in a Patient with a History of Gastric Bypass Surgery

“…Not only insulinomas but also nesidioblastosis responds to calcium gluconate in the SASI test, as we previously reported (16). A previous study showed that the maximum increase in hepatic venous insulin concentration over the baseline after calcium injection is useful for distinguishing insulinomas from other causes; indeed, cut-offs of >91.5 μIU/ mL and >263.5 μIU/mL for insulin concentrations were reportedly 95% and 100% specific for insulinoma, respectively (6).…”

Hypoglycaemia following upper gastrointestinal surgery: case report and review of the literature

“…While some have achieved desirable results with limited pancreatic resection, others have suggested that near-total or total pancreatectomy is the only definitive solution to this condition - as is the case for infants with diffuse nesidioblastosis and familial hyperinsulinemic hypoglycaemia [11,12,15,16]. Pharmacologically, symptoms have been shown to be adequately controlled with the use of diazoxide[6].…”

“…This is the first description of the development of NIPHS following fundoplication surgery. Although octreotide has been successfully used to treat adult onset nesidioblastosis before, this is the first report of its use in post operative NIPHS [12]. …”

Nesidioblastosis as a cause of focal pancreatic 111In-pentetreotide uptake in a patient with putative VIPoma: another differential diagnosis

“…In adults, nesidioblastosis is a very infrequent condition, and a rare cause of symptomatic presentations [10][11][12]. There may be an association with hyperinsulinaemic hypoglycaemia, although without a clear causal relationship.…”