This clinical case study describes a patient with acute onset of hyperinsulinemic hypoglycemia. Her presentation was consistent with an insulinoma, but it was not visualized on standard cross-sectional imaging. Endoscopic ultrasound (EUS) was able to identify the lesion and aid in precise surgical planning, removal and cure.
Background
The patient, a 9-year-old female, was admitted to Children’s Hospital of Philadelphia (CHOP) with symptomatic hypoglycemia. She was noted to have elevated serum levels of insulin and c-peptide. There was a strong suspicion for a type of neuroendocrine tumor called an insulinoma, arising from pancreatic beta cells.
Despite multiple imaging modalities, including CT, MRI, and PET scans, a distinct lesion was not identified. Ultimately, the patient underwent an endoscopic ultrasound (EUS) where a sub-centimeter mass was found in the tail of the pancreas. She went on to a successful enucleation of the lesion – sparing any significant resection of normal pancreas. Her follow-up fasting studies demonstrated resolution of the hypoglycemia, and thus a durable cure.
Diagnostic findings
Diagnostic testing included both laboratory tests and imaging:
- Blood glucose tests showed elevated insulin and c-peptide levels at the time of hypoglycemia, as well as suppressed beta-hydroxybutyrate and free fatty acids.
- The patient had an MRI performed at the referring hospital without identification of a definitive lesion.
- At CHOP, she had additional fasting tests which confirmed the hyperinsulinemic hypoglycemia and required IV dextrose to maintain euglycemia.
- A Ga-DOTATATE PET scan was performed, which also did not reveal a lesion.
- Ultimately, EUS was performed and able to identify a sub-centimeter (6mm x 8mm) hypoechoic lesion in the tail of the pancreas. (see Fig. 1 & Fig. 2)
Fig. 1
Fig. 2
Clinical course and treatment
Using the information from the diagnostic EUS, the surgeon was able to perform a direct enucleation of the lesion from the tail of the pancreas without need for significant pancreatic resection or removal.
Outcomes
The patient recovered well from her surgery and was able to maintain euglycemia after the IV dextrose was weaned off. Results from her removed tumor were consistent with an insulinoma.
The patient received a final fasting test at the hospital that demonstrated a successful cure of her hypoglycemia/hyperinsulinism. Upon discharge, she was able to be followed safely by her home pediatrician without further need for endocrinology, gastroenterology or surgical follow-up.
Discussion
Patients with hyperinsulinemic hypoglycemia can present with life-threatening hypoglycemic episodes, and thus require close monitoring, diagnostic workup, and interventions tailored to the expected etiology.
Insulinomas are rare – and typically non-malignant – tumors of the pancreatic beta cells that oversecrete insulin. The excess insulin results in severe hypoglycemia, which can cause brain damage, coma or death. Their rarity in the pediatric population results in delayed diagnosis and providers who are not familiar with the optimal diagnostic approach. These tumors can be challenging to identify on traditional cross-sectional imaging, such as CT, MRI, or even PET scans.
EUS can be a helpful adjunctive diagnostic modality in cases of suspected insulinoma, as organs such as the pancreas (where insulinomas most often originate from) are very well imaged in detail using this diagnostic tool. EUS can often pick up very small sub-centimeter lesions, as in this case.
The Congenital Hyperinsulinism Center at the Children’s Hospital of Philadelphia is the largest center in the country for the treatment of pediatric hypoglycemic disorders. It offers comprehensive care and groundbreaking treatments for children with these rare disorders.
At CHOP, we can utilize EUS for myriad diagnostic and therapeutic efforts to help better diagnose, treat or assist colleagues in other disciplines, as well as better plan and prepare for surgical interventions. Therefore, potentially decreasing the need for more extensive surgeries and improving the chance of recovery.
The authors are participants in CHOP’s multidisciplinary Hyperinsulinism and Pancreatic Disorders programs. Alexander Coe, MD, MEd, is a pediatric gastroenterologist with additional training and special expertise in treating children with pancreatic disorders. Katherine Lord, MD, is a pediatric endocrinologist with expertise in patients with hyperinsulinism. N. Scott Adzick, MD, MMM, FACS, FAAP, is Chief of Surgery, and an attending surgeon with expertise in surgical procedures for hyperinsulinism, involving the pancreas.
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This clinical case study describes a patient with acute onset of hyperinsulinemic hypoglycemia. Her presentation was consistent with an insulinoma, but it was not visualized on standard cross-sectional imaging. Endoscopic ultrasound (EUS) was able to identify the lesion and aid in precise surgical planning, removal and cure.
Background
The patient, a 9-year-old female, was admitted to Children’s Hospital of Philadelphia (CHOP) with symptomatic hypoglycemia. She was noted to have elevated serum levels of insulin and c-peptide. There was a strong suspicion for a type of neuroendocrine tumor called an insulinoma, arising from pancreatic beta cells.
Despite multiple imaging modalities, including CT, MRI, and PET scans, a distinct lesion was not identified. Ultimately, the patient underwent an endoscopic ultrasound (EUS) where a sub-centimeter mass was found in the tail of the pancreas. She went on to a successful enucleation of the lesion – sparing any significant resection of normal pancreas. Her follow-up fasting studies demonstrated resolution of the hypoglycemia, and thus a durable cure.
Diagnostic findings
Diagnostic testing included both laboratory tests and imaging:
- Blood glucose tests showed elevated insulin and c-peptide levels at the time of hypoglycemia, as well as suppressed beta-hydroxybutyrate and free fatty acids.
- The patient had an MRI performed at the referring hospital without identification of a definitive lesion.
- At CHOP, she had additional fasting tests which confirmed the hyperinsulinemic hypoglycemia and required IV dextrose to maintain euglycemia.
- A Ga-DOTATATE PET scan was performed, which also did not reveal a lesion.
- Ultimately, EUS was performed and able to identify a sub-centimeter (6mm x 8mm) hypoechoic lesion in the tail of the pancreas. (see Fig. 1 & Fig. 2)
Fig. 1
Fig. 2
Clinical course and treatment
Using the information from the diagnostic EUS, the surgeon was able to perform a direct enucleation of the lesion from the tail of the pancreas without need for significant pancreatic resection or removal.
Outcomes
The patient recovered well from her surgery and was able to maintain euglycemia after the IV dextrose was weaned off. Results from her removed tumor were consistent with an insulinoma.
The patient received a final fasting test at the hospital that demonstrated a successful cure of her hypoglycemia/hyperinsulinism. Upon discharge, she was able to be followed safely by her home pediatrician without further need for endocrinology, gastroenterology or surgical follow-up.
Discussion
Patients with hyperinsulinemic hypoglycemia can present with life-threatening hypoglycemic episodes, and thus require close monitoring, diagnostic workup, and interventions tailored to the expected etiology.
Insulinomas are rare – and typically non-malignant – tumors of the pancreatic beta cells that oversecrete insulin. The excess insulin results in severe hypoglycemia, which can cause brain damage, coma or death. Their rarity in the pediatric population results in delayed diagnosis and providers who are not familiar with the optimal diagnostic approach. These tumors can be challenging to identify on traditional cross-sectional imaging, such as CT, MRI, or even PET scans.
EUS can be a helpful adjunctive diagnostic modality in cases of suspected insulinoma, as organs such as the pancreas (where insulinomas most often originate from) are very well imaged in detail using this diagnostic tool. EUS can often pick up very small sub-centimeter lesions, as in this case.
The Congenital Hyperinsulinism Center at the Children’s Hospital of Philadelphia is the largest center in the country for the treatment of pediatric hypoglycemic disorders. It offers comprehensive care and groundbreaking treatments for children with these rare disorders.
At CHOP, we can utilize EUS for myriad diagnostic and therapeutic efforts to help better diagnose, treat or assist colleagues in other disciplines, as well as better plan and prepare for surgical interventions. Therefore, potentially decreasing the need for more extensive surgeries and improving the chance of recovery.
The authors are participants in CHOP’s multidisciplinary Hyperinsulinism and Pancreatic Disorders programs. Alexander Coe, MD, MEd, is a pediatric gastroenterologist with additional training and special expertise in treating children with pancreatic disorders. Katherine Lord, MD, is a pediatric endocrinologist with expertise in patients with hyperinsulinism. N. Scott Adzick, MD, MMM, FACS, FAAP, is Chief of Surgery, and an attending surgeon with expertise in surgical procedures for hyperinsulinism, involving the pancreas.
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