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Insulinoma Presenting Acutely in a 15-Year-Old Female
Mahmuda Ahmed, MD1, Rushika Conroy, MD1, Meghana Gudala, MBBS1,
Hisham Alomar, MD2, and Ksenia Tonyushkina, MD1.
1Endocrinology, Baystate Children's Hospital, Springfield, MA, USA; 2Department of Emergency
Medicine, Baystate Medical Center, Springfield, MA, USA.
INTRODUCTION
CLINICAL CASE
baystatehealth.org/bch
 Insulinoma is the most common functioning neoplasm
of the pancreas
 Incidence of 1 to 4 per million people
 Median age at time of surgical diagnosis is 47 years
 Range from 3 to 82 years of age
 Females constitute 59% of the total population group
 Incidence in children is unknown, but is extremely low
with only cases reported worldwide (Table 3)
 Duration of symptoms in children before diagnosis
range anywhere from 2 weeks to 7 years
 Abnormal counterregulatory hormone response may
occur
CLINICAL LESSONS
1. Insulinoma can present acutely at a young age
without typical features
2. Unexplained hypoglycemia should be taken
seriously in a teenage patient – with full
investigation prior to discharge
3. Blunting of counterregulatory hormones may
occur due to fixed hypoglycemic stimulus
(Davis et al. 1991)
Table 1: Critical Sample No. 1 ^ No. 2 ^ No. 3 *
POC-glucose (mg/dL) 41 39 37
Serum glucose (mg/dL) 48 44
Serum insulin (mcIU/mL) 63.2 14.8 28.5
C-peptide (ng/mL) 5.5 5.3 4.06
Proinsulin (pmol/L) 338
Cortisol (mcg/dL) 7.1 5.4 1.4
Betahydroxybutyrate (mmol/L) 0.29 0.27 <0.30
Serum Lactate (mmol/L) 0.7 1.2 1.0
Growth hormone (ng/mL) 2.43 2.78
Salicylate (mg/dL) < 0.3
Acetaminophen (mg/L) < 15
^ Performed at Baystate Children’s Hospital
* Performed at Children's Hospital of
Philadelphia
Present
Case (not
published)
Mirion
et al.
(2016)
Jung et
al.
(2015)
Padidela et
al. (2014)
Peranteau
et al.
(2013)
Kao et
al.
(2013)
Ide et al.
(2012)
Yu et al.
(2009)
Ozen
et al.
(2009)
Strong
et al.
(2007)
Jaladayan
et al.
(2007)
Wolfsdorf
et al.
(1979)
No. of Cases 1 1 1 9 8 1 1 4 1 1 1 1
Age at diagnosis (yrs) 15 11 11 3-15 4-26 9 13 13-18 16 13 13 8
Sex F M F F 5/9 M 6/8 F M M 3/4 M M F F
Duration of
Symptoms (mos)
3 5 5 1-24 1-84 4 Unknown
Not
described
12 1 6 0.5
Seizure activity No No Yes Not described
Not
described
Yes Yes
Not
described
Yes Yes Yes No
Imaging MRI MRI MRI
MRI 7/9
CT 2/9
MRI 3/6
CT 2/7
18F-DOPA
PET 3/3
CT CT
Not
described
CT MRI MRI
No
imaging
Location of tumor Neck Tail Tail
Head 4/9
Uncinate 4/9
Junction-body
& tail 1/9
Tail 4/8
Head 2/8
Neck 1/8
Multiple 1/8
Uncinate
Pancreatico-
biliary
Junction
Head 4/4 Tail Neck Body Head
Size of Tumor (cm) 1.1 1.0 3.0 0.8-2.0 0.7 – 1.8 1.4 1.9 1.0-3.5 0.8 1.8 2.1 1.1
 15 year old previously healthy female
 3 month history of fatigue, tremors, jitteriness, and
sweating exclusively after school
 Frequently skipped both breakfast and lunch and
reported ~ 5 kg weight gain
 Brought to ED for unresponsiveness with POC-glucose
27 mg/dL – discharged after 5 hours of observation
 Readmitted to ED 2 days later with similar symptoms
and POC-glucose of 19 mg/dL
 Screening tests showed hyperinsulinemic
hypoglycemia (Table 1)
 ACTH (250 mcg) stimulation test was normal after
repeat low cortisol levels (Table 2)
Table 3: Comparison of Published Cases of Pediatric Insulinoma (English)
Figure 1: MRI - 1.1 cm Hypervascular
Enhancing Mass Anterior to Pancreatic Neck
EVALUATION
TREATMENT/FOLLOW UP
0
20
40
60
80
100
120
140
0 120 240 360 480 600 720
Time (minutes)
POC-Glucose(mg/dL)
Table 2: ACTH Stimulation Test ^ 0 min 60 min
Cortisol (mcg/dL) 6.6 27
Chart 1: Blood Glucose During Supervised Fast
 Surgical resection of 1.5 cm insulinoma from
pancreatic neck with partial pancreatectomy
 Post-operative monitoring with 48 hour cure
fast - ended at 41h with POC-glucose 61 mg/dL
and elevated ketones
 Continues to be euglycemic 4 months after
resection with weight loss of 8.5 kg
1 mg of glucagon
administered
*No conflict of interest
 Lesion confirmed on microbubble-contrast US,
CT with IV contrast and 18 F-DOPA PET
Poster # SAT-046

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ENDO SOCIETY - MAHMUDA AHMED Insulinoma Case

  • 1. Insulinoma Presenting Acutely in a 15-Year-Old Female Mahmuda Ahmed, MD1, Rushika Conroy, MD1, Meghana Gudala, MBBS1, Hisham Alomar, MD2, and Ksenia Tonyushkina, MD1. 1Endocrinology, Baystate Children's Hospital, Springfield, MA, USA; 2Department of Emergency Medicine, Baystate Medical Center, Springfield, MA, USA. INTRODUCTION CLINICAL CASE baystatehealth.org/bch  Insulinoma is the most common functioning neoplasm of the pancreas  Incidence of 1 to 4 per million people  Median age at time of surgical diagnosis is 47 years  Range from 3 to 82 years of age  Females constitute 59% of the total population group  Incidence in children is unknown, but is extremely low with only cases reported worldwide (Table 3)  Duration of symptoms in children before diagnosis range anywhere from 2 weeks to 7 years  Abnormal counterregulatory hormone response may occur CLINICAL LESSONS 1. Insulinoma can present acutely at a young age without typical features 2. Unexplained hypoglycemia should be taken seriously in a teenage patient – with full investigation prior to discharge 3. Blunting of counterregulatory hormones may occur due to fixed hypoglycemic stimulus (Davis et al. 1991) Table 1: Critical Sample No. 1 ^ No. 2 ^ No. 3 * POC-glucose (mg/dL) 41 39 37 Serum glucose (mg/dL) 48 44 Serum insulin (mcIU/mL) 63.2 14.8 28.5 C-peptide (ng/mL) 5.5 5.3 4.06 Proinsulin (pmol/L) 338 Cortisol (mcg/dL) 7.1 5.4 1.4 Betahydroxybutyrate (mmol/L) 0.29 0.27 <0.30 Serum Lactate (mmol/L) 0.7 1.2 1.0 Growth hormone (ng/mL) 2.43 2.78 Salicylate (mg/dL) < 0.3 Acetaminophen (mg/L) < 15 ^ Performed at Baystate Children’s Hospital * Performed at Children's Hospital of Philadelphia Present Case (not published) Mirion et al. (2016) Jung et al. (2015) Padidela et al. (2014) Peranteau et al. (2013) Kao et al. (2013) Ide et al. (2012) Yu et al. (2009) Ozen et al. (2009) Strong et al. (2007) Jaladayan et al. (2007) Wolfsdorf et al. (1979) No. of Cases 1 1 1 9 8 1 1 4 1 1 1 1 Age at diagnosis (yrs) 15 11 11 3-15 4-26 9 13 13-18 16 13 13 8 Sex F M F F 5/9 M 6/8 F M M 3/4 M M F F Duration of Symptoms (mos) 3 5 5 1-24 1-84 4 Unknown Not described 12 1 6 0.5 Seizure activity No No Yes Not described Not described Yes Yes Not described Yes Yes Yes No Imaging MRI MRI MRI MRI 7/9 CT 2/9 MRI 3/6 CT 2/7 18F-DOPA PET 3/3 CT CT Not described CT MRI MRI No imaging Location of tumor Neck Tail Tail Head 4/9 Uncinate 4/9 Junction-body & tail 1/9 Tail 4/8 Head 2/8 Neck 1/8 Multiple 1/8 Uncinate Pancreatico- biliary Junction Head 4/4 Tail Neck Body Head Size of Tumor (cm) 1.1 1.0 3.0 0.8-2.0 0.7 – 1.8 1.4 1.9 1.0-3.5 0.8 1.8 2.1 1.1  15 year old previously healthy female  3 month history of fatigue, tremors, jitteriness, and sweating exclusively after school  Frequently skipped both breakfast and lunch and reported ~ 5 kg weight gain  Brought to ED for unresponsiveness with POC-glucose 27 mg/dL – discharged after 5 hours of observation  Readmitted to ED 2 days later with similar symptoms and POC-glucose of 19 mg/dL  Screening tests showed hyperinsulinemic hypoglycemia (Table 1)  ACTH (250 mcg) stimulation test was normal after repeat low cortisol levels (Table 2) Table 3: Comparison of Published Cases of Pediatric Insulinoma (English) Figure 1: MRI - 1.1 cm Hypervascular Enhancing Mass Anterior to Pancreatic Neck EVALUATION TREATMENT/FOLLOW UP 0 20 40 60 80 100 120 140 0 120 240 360 480 600 720 Time (minutes) POC-Glucose(mg/dL) Table 2: ACTH Stimulation Test ^ 0 min 60 min Cortisol (mcg/dL) 6.6 27 Chart 1: Blood Glucose During Supervised Fast  Surgical resection of 1.5 cm insulinoma from pancreatic neck with partial pancreatectomy  Post-operative monitoring with 48 hour cure fast - ended at 41h with POC-glucose 61 mg/dL and elevated ketones  Continues to be euglycemic 4 months after resection with weight loss of 8.5 kg 1 mg of glucagon administered *No conflict of interest  Lesion confirmed on microbubble-contrast US, CT with IV contrast and 18 F-DOPA PET Poster # SAT-046