A Systematic Review of Proinsulin-Secreting Pancreatic Neuroendocrine Tumors

Murtha, Timothy D.; Lupsa, Beatrice; Majumdar, Sachin; Jain, Dhanpat; Salem, Ronald R.

doi:10.1007/s11605-017-3428-8

Cited by 27 publications

(26 citation statements)

References 24 publications

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“…This would also explain the delayed presentation of hypoglycemia at hour 72 of the fast. However, similar findings were not observed in other reported cases of proinsulin-secreting neuroendocrine tumors (6).…”

Section: Discussionsupporting

confidence: 84%

“…Recent advances in laboratory assays make the differentiation between proinsulin and insulin possible (5). This has led to the discovery that proinsulin-producing PNETs (proinsulinomas) that predominantly secrete proinsulin with normal or near-normal levels of insulin secretion can also cause hypoglycemia (5,6). In 2017, only 16 cases of proinsulin-secreting PNETs had been reported (6).…”

Section: Introductionmentioning

confidence: 99%

“…This has led to the discovery that proinsulin-producing PNETs (proinsulinomas) that predominantly secrete proinsulin with normal or near-normal levels of insulin secretion can also cause hypoglycemia (5,6). In 2017, only 16 cases of proinsulin-secreting PNETs had been reported (6). While there have been cases of insulinomas in patients after Roux-en-Y gastric bypass (RYGB) surgery (7), there are no reports of proinsulinomas after RYGB surgery.…”

Section: Introductionmentioning

confidence: 99%

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Proinsulin-Predominant Pancreatic Neuroendocrine Tumor-Induced Hypoglycemia After ROUX-EN-Y Gastric Bypass Surgery

Edwards

Portilla

Mirza

et al. 2019

AACE Clinical Case Reports

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Objective: To present a case of recurrent hypoglycemia following Roux-en-Y gastric bypass (RYGB) surgery whose etiology was determined to be a proinsulin-predominant pancreatic neuroendocrine tumor (a proinsulinoma). Methods: A case report along with a brief discussion and review of the pertinent literature is presented. Results: The patient is a 62-year-old female who presented with symptomatic hypoglycemia 11 years after RYGB surgery. Initial workup revealed low insulin levels with elevated proinsulin levels. A 72-hour fast confirmed the presence of proinsulin-induced hypoglycemia secondary to a pancreatic neuroendocrine tumor (PNET). She underwent distal pancreatectomy with splenectomy and a PNET tumor was successfully removed with resolution of her symptoms. Conclusion: Hypoglycemia after RYGB surgery is a well-established syndrome. While there are several etiologies for this, PNETs (including proinsulinomas) should be considered in the differential diagnosis in this population. Proinsulinomas are an increasingly recognized cause of hypoglycemia. Proinsulin levels must always be included as part of the workup of hypoglycemia in an adult.

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Section: Discussionsupporting

confidence: 84%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Proinsulin-Predominant Pancreatic Neuroendocrine Tumor-Induced Hypoglycemia After ROUX-EN-Y Gastric Bypass Surgery

Edwards

Portilla

Mirza

et al. 2019

AACE Clinical Case Reports

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show abstract

“…Additionally, one could argue that the ratio of proinsulin to insulin in our patient was not high enough to justify a diagnosis of proinsulinoma. However, others have reported similar levels in the literature (17). The commercially available proinsulin assays either measure intact proinsulin or total proinsulin (which includes intermediates) levels.…”

Section: Discussionmentioning

confidence: 77%

Use of a Continuous Glucose Monitor for Preoperative Monitoring and Treatment of Hypoglycemia in a Case of Pancreatic Neuroendocrine Tumor

Lyerla

Bajaj

Shrestha

2019

AACE Clinical Case Reports

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Objective: Pancreatic neuroendocrine tumors secreting proinsulin and insulin could lead to life-threatening hypoglycemia. We aim to show this can be avoided by utilizing continuous glucose monitoring.Methods: We describe a case of a 55-year-old female with hypoglycemia unawareness and seizures diagnosed with proinsulinoma. She utilized an intermittently scanned continuous glucose monitor (isCGM) to monitor hypoglycemia preoperatively.Results: The patient underwent biochemical and radiographic evaluation to confirm the diagnosis of proinsulinoma. Utilizing isCGM to monitor blood glucose, she was able to prevent hypoglycemia-related seizures prior to definitive surgery.Conclusion: In the time leading up to a definitive surgery, patients with proinsulinomas are at risk of hypoglycemic events leading to falls, seizures, and even death. isCGMs can be utilized for preoperative monitoring and treatment of hypoglycemia in these patients. (AACE Clinical Case Rep. 2019;5:e255-e258) Abbreviations: CGM = continuous glucose monitor; isCGM = intermittently scanned continuous glucose monitor; PNET = pancreatic neuroendocrine tumor e256 PNET with Seizures, AACE Clinical Case Rep. 2019;5(No. 4)

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“…Among the usual suspects, insulinoma is a more well described entity compared to the less commonly reported counterpart, proinsulin secreting neuroendocrine tumour. A series reported proinsulinoma has a 2:1 female preponderance with mean age of diagnosis at 56 years old (1). The lesion usually lies, unsurprisingly, at the body and tail of the pancreas, which signposts to the diagnosis (1-3).…”

Section: Introductionmentioning

confidence: 99%

Distracting spontaneous refractory hypoglycaemia

Cheah¹,

Rajan²,

Mathews³

et al. 2017

EJEA

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A Systematic Review of Proinsulin-Secreting Pancreatic Neuroendocrine Tumors

Abstract: Proinsulinomas are rare pancreatic neuroendocrine tumors that have the potential to cause hypoglycemia. While insulinomas and proinsulin-secreting tumors have many physiologic parallels, these cases illustrate several key distinctions in their diagnosis and management.

Cited by 27 publications

References 24 publications

Proinsulin-Predominant Pancreatic Neuroendocrine Tumor-Induced Hypoglycemia After ROUX-EN-Y Gastric Bypass Surgery

Proinsulin-Predominant Pancreatic Neuroendocrine Tumor-Induced Hypoglycemia After ROUX-EN-Y Gastric Bypass Surgery

Use of a Continuous Glucose Monitor for Preoperative Monitoring and Treatment of Hypoglycemia in a Case of Pancreatic Neuroendocrine Tumor

Distracting spontaneous refractory hypoglycaemia

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