Abstract
Background: Multiple endocrine neoplasia type 1 (MEN1) is an inherited neoplasia syndrome that generally presents with hypercalcaemia due to hyperparathyroidism. Insulin-producing tumours are less common components of the syndrome that emerge later during the course of the disease. We report here a case of an adolescent who presented with symptomatic hypoglycaemia as the first indication of MEN1.
Case: A 14-year-old boy, known to use illicit drugs, was brought to the hospital with altered mental status. He was hypoglycaemic and further investigations revealed two pancreatic insulinomas. Despite having no relevant family history, genetic evaluation showed a mutation consistent with MEN1.
Conclusion: Insulinomas in adolescents are generally rare and even less common as a first presentation of MEN1. This diagnosis carries implications for potential future neoplasms, both benign and malignant. While intoxication is a more common case of altered mental status in adolescents, clinicians must maintain a high index of suspicion for organic disease.
References
1. Thakker RV. Multiple endocrine neoplasia type 1 (MEN1). Best Pract Res Clin Endocrinol Metab 2010;24:355–70.10.1016/j.beem.2010.07.003Search in Google Scholar
2. Service FJ, McMahon MM, O’Brien PC, Ballard DJ. Functioning insulinoma – incidence, recurrence, and long-term survival of patients: a 60-year study. Mayo Clin Proc 1991;66:711–9.10.1016/S0025-6196(12)62083-7Search in Google Scholar
3. Pieterman CR, Vriens MR, Dreijerink KM, van der Luijt RB, Valk GD. Care for patients with multiple endocrine neoplasia type 1: the current evidence base. Fam Cancer 2011;10:157–71.10.1007/s10689-010-9398-6Search in Google Scholar
4. Ozen S, Saz EU, Celik A, Aydin A, Simsek DG, et al. Many admissions to the emergency departments with recurrent syncope attacks and seizures in an adolescent boy. Eur J Pediatr 2009;168:761–3.10.1007/s00431-009-0974-4Search in Google Scholar
5. Grant C, Pan J. A comparison of five transition programmes for youth with chronic illness in Canada. Child Care Health Dev 2011;37:815–20.10.1111/j.1365-2214.2011.01322.xSearch in Google Scholar
6. Jaksic T, Yaman M, Thorner P, Wesson DK, Filler RM, et al. A 20-year review of pediatric pancreatic tumors. J Pediatr Surg 1992;27:1315–7.10.1016/0022-3468(92)90284-ESearch in Google Scholar
7. Dizon AM, Kowalyk S, Hoogwerf BJ. Neuroglycopenic and other symptoms in patients with insulinomas. Am J Med 1999;106:307–10.10.1016/S0002-9343(99)00021-2Search in Google Scholar
8. Boukhman MP, Karam JH, Shaver J, Siperstein AE, Duh QY, et al. Insulinoma – experience from 1950 to 1995. West J Med 1998;169:98–104.Search in Google Scholar
9. Jaladyan V, Darbinyan V. Insulinoma misdiagnosed as juvenile myoclonic epilepsy. Eur J Pediatr 2007;166:485–7.10.1007/s00431-006-0365-zSearch in Google Scholar PubMed PubMed Central
10. Baudin E, Caron P, Lombard-Bohas C, Tabarin A, Mitry E, et al. Malignant insulinoma: recommendations for characterisation and treatment. Ann Endocrinol (Paris) 2013;74:523–33.10.1016/j.ando.2013.07.001Search in Google Scholar PubMed
11. Grant CS. Insulinoma. Baillieres Best Pract Res Clin Gastroenterol 2005;19:783–98.10.1016/j.bpg.2005.05.008Search in Google Scholar PubMed
12. Okabayashi T, Shima Y, Sumiyoshi T, Kozuki A, Ito S, et al. Diagnosis and management of insulinoma. World J Gastroenterol 2013;19:829–37.10.3748/wjg.v19.i6.829Search in Google Scholar
13. Machado MC. Surgical treatment of pancreatic endocrine tumors in multiple endocrine neoplasia type 1. Clinics 2012;67:145–8.10.6061/clinics/2012(Sup01)24Search in Google Scholar
14. Moncayo VM, Martin DR, Sarmiento JM, Zbytek B, Fox T, et al. 111In Octreoscan SPECT-MRI fusion for the detection of a pancreatic insulinoma. Clin Nucl Med 2012;37:e53–6.10.1097/RLU.0b013e31823ea402Search in Google Scholar PubMed
15. Pery C, Meurette G, Ansquer C, Frampas E, Regenet N. Role and limitations of 18F-FDG positron emission tomography (PET) in the management of patients with pancreatic lesions. Gastroenterol Clin Biol 2010;34:465–74.10.1016/j.gcb.2009.04.014Search in Google Scholar PubMed
16. Tang HY, Garcia JM. Association between insulinoma and adrenal insufficiency: a case report and review of the literature. Pancreas 2010;39:544–6.10.1097/MPA.0b013e3181c07117Search in Google Scholar PubMed
17. Vezzosi D, Bennet A, Courbon F, Caron P. Short- and long-term somatostatin analogue treatment in patients with hypoglycaemia related to endogenous hyperinsulinism. Clin Endocrinol (Oxf) 2008;68:904–11.10.1111/j.1365-2265.2007.03136.xSearch in Google Scholar PubMed
18. Falchetti A, Marini F, Luzi E, Giusti F, Cavalli L, et al. Multiple endocrine neoplasia type 1 (MEN1): not only inherited endocrine tumors. Genet Med 2009;11:825–35.10.1097/GIM.0b013e3181be5c97Search in Google Scholar PubMed
19. Shepherd JJ. The natural history of multiple endocrine neoplasia type 1: highly uncommon or highly unrecognized? Arch Surg 1991;126:935–52.10.1001/archsurg.1991.01410320017001Search in Google Scholar PubMed
20. Burgess J. How should the patient with multiple endocrine neoplasia type 1 (MEN 1) be followed? Clin Endocrinol (Oxf) 2010;72:13–6.10.1111/j.1365-2265.2009.03662.xSearch in Google Scholar PubMed
©2014 by Walter de Gruyter Berlin/Boston
Articles in the same Issue
- Frontmatter
- Editorial
- Welcome to Assistant Editors at JPEM
- Review article
- Common approach to childhood obesity in Japan
- Images in pediatric endocrinology
- Aromatase deficiency in an adolescent girl misdiagnosed as congenital adrenal hyperplasia in infancy and childhood
- Original articles
- Prevalence of vitamin D deficiency among Iranian adolescents
- Analysis of body composition among children and adolescents – a cross-sectional study of the Polish population and comparison of body fat measurement methods
- Association of rs8066560 variant in the sterol regulatory element-binding protein 1 (SREBP-1) and miR-33b genes with hyperglycemia and insulin resistance
- Differences in anxiety and depression between male and female caregivers of children with a disorder of sex development
- Fasting and postprandial levels of a novel anorexigenic peptide nesfatin in childhood obesity
- The effect of growth hormone treatment on height in children with idiopathic short stature
- Clinical characteristics of type 1 diabetes over a 40 year period in Turkey: secular trend towards earlier age of onset
- Relationships of gamma-glutamyltransferase and beta 2-microglobulin on high sensitivity C-reactive protein among Japanese elementary school children
- Evaluation of insulin resistance and metabolic syndrome in a group of obese Czech children
- Circulating intact parathyroid hormone is suppressed at 25-hydroxyvitamin D concentrations >25 nmol/L in children
- Association between vitamin D level and cardiovascular risk in obese children and adolescents
- Serum paraoxonase activity and oxidative stress and their relationship with obesity-related metabolic syndrome and non-alcoholic fatty liver disease in obese children and adolescents
- Clinical features at diagnosis and responses to antithyroid drugs in younger children with Graves’ disease compared with adolescent patients
- Influence of hormonal parameters, bone mineral density and bone turnover on fracture risk in healthy male adolescents: a case control study
- Evaluation of lipid and glucose metabolism and cortisol and thyroid hormone levels in obese appropriate for gestational age (AGA) born and non-obese small for gestational age (SGA) born prepubertal Slovak children
- Chitotriosidase as a possible marker of clinically evidenced atherosclerosis in dyslipidemic children
- Exhaled carbon monoxide in adolescents with diabetic cardiovascular autonomic neuropathy
- Gut hormone profiles in preterm and term infants during the first 2 months of life
- Outcome analysis of aromatase inhibitor therapy to increase adult height in males with predicted short adult stature and/or rapid pubertal progress: a retrospective chart review
- A deletion including exon 2 of the TSHR gene is associated with thyroid dysgenesis and severe congenital hypothyroidism
- Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) in childhood
- Patient Reports
- Neonatal diabetes in an infant of diabetic mother: same novel INS missense mutation in the mother and her offspring
- Atypical neurologic presentations of new onset type 1 diabetes mellitus in pediatric age group: a report of five unusual cases and review of the literature
- Importance of dietary calcium and vitamin D in the treatment of hypercalcaemia in Williams-Beuren syndrome
- Vitamin D intoxication in two brothers: be careful with dietary supplements
- Transient diabetes insipidus in a preterm neonate and the challenge of desmopressin dosing
- A rare case of hypoglycaemia due to insulinoma in an adolescent with acutely altered mental status
- Holoprosencephaly: ZIC2 mutation in a case with panhypopituitarism
- An association of hypochondroplasia and immune deficiency
- Hypophosphatemic rickets caused by a novel PHEX gene mutation in an Indian girl
- Psoriasis vulgaris and autoimmune polyendocrine syndrome type I: a case report
- Identification of a combined missense/splice-site mutation in FAH causing tyrosinemia type 1
- Letter to the Editor
- Vitamin D intake and premature infants with intraventricular hemorrhage: how advisable is it?
Articles in the same Issue
- Frontmatter
- Editorial
- Welcome to Assistant Editors at JPEM
- Review article
- Common approach to childhood obesity in Japan
- Images in pediatric endocrinology
- Aromatase deficiency in an adolescent girl misdiagnosed as congenital adrenal hyperplasia in infancy and childhood
- Original articles
- Prevalence of vitamin D deficiency among Iranian adolescents
- Analysis of body composition among children and adolescents – a cross-sectional study of the Polish population and comparison of body fat measurement methods
- Association of rs8066560 variant in the sterol regulatory element-binding protein 1 (SREBP-1) and miR-33b genes with hyperglycemia and insulin resistance
- Differences in anxiety and depression between male and female caregivers of children with a disorder of sex development
- Fasting and postprandial levels of a novel anorexigenic peptide nesfatin in childhood obesity
- The effect of growth hormone treatment on height in children with idiopathic short stature
- Clinical characteristics of type 1 diabetes over a 40 year period in Turkey: secular trend towards earlier age of onset
- Relationships of gamma-glutamyltransferase and beta 2-microglobulin on high sensitivity C-reactive protein among Japanese elementary school children
- Evaluation of insulin resistance and metabolic syndrome in a group of obese Czech children
- Circulating intact parathyroid hormone is suppressed at 25-hydroxyvitamin D concentrations >25 nmol/L in children
- Association between vitamin D level and cardiovascular risk in obese children and adolescents
- Serum paraoxonase activity and oxidative stress and their relationship with obesity-related metabolic syndrome and non-alcoholic fatty liver disease in obese children and adolescents
- Clinical features at diagnosis and responses to antithyroid drugs in younger children with Graves’ disease compared with adolescent patients
- Influence of hormonal parameters, bone mineral density and bone turnover on fracture risk in healthy male adolescents: a case control study
- Evaluation of lipid and glucose metabolism and cortisol and thyroid hormone levels in obese appropriate for gestational age (AGA) born and non-obese small for gestational age (SGA) born prepubertal Slovak children
- Chitotriosidase as a possible marker of clinically evidenced atherosclerosis in dyslipidemic children
- Exhaled carbon monoxide in adolescents with diabetic cardiovascular autonomic neuropathy
- Gut hormone profiles in preterm and term infants during the first 2 months of life
- Outcome analysis of aromatase inhibitor therapy to increase adult height in males with predicted short adult stature and/or rapid pubertal progress: a retrospective chart review
- A deletion including exon 2 of the TSHR gene is associated with thyroid dysgenesis and severe congenital hypothyroidism
- Steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) in childhood
- Patient Reports
- Neonatal diabetes in an infant of diabetic mother: same novel INS missense mutation in the mother and her offspring
- Atypical neurologic presentations of new onset type 1 diabetes mellitus in pediatric age group: a report of five unusual cases and review of the literature
- Importance of dietary calcium and vitamin D in the treatment of hypercalcaemia in Williams-Beuren syndrome
- Vitamin D intoxication in two brothers: be careful with dietary supplements
- Transient diabetes insipidus in a preterm neonate and the challenge of desmopressin dosing
- A rare case of hypoglycaemia due to insulinoma in an adolescent with acutely altered mental status
- Holoprosencephaly: ZIC2 mutation in a case with panhypopituitarism
- An association of hypochondroplasia and immune deficiency
- Hypophosphatemic rickets caused by a novel PHEX gene mutation in an Indian girl
- Psoriasis vulgaris and autoimmune polyendocrine syndrome type I: a case report
- Identification of a combined missense/splice-site mutation in FAH causing tyrosinemia type 1
- Letter to the Editor
- Vitamin D intake and premature infants with intraventricular hemorrhage: how advisable is it?