Masa pancreática: una presentación inhabitual de nesidioblastosis en el adulto / Pancreatic mass: an unusual presentation of nesidioblastosis in adults

A 41-year-old woman, multiparous of 4, with personal or familiar history of diabetes, with a history of Nissen fundoplication due to pathological gastro-oesophageal reflux, is refer to an endocrinologjst during her post-operatiye follow up, 4 months after her surgery for a 14 kg weight loss in 10 months and symtomatic hypoglycemia to repetition. A positive prolonged fasting test for hypoglycemia was performed. In addition, abdominal computed axial tomography was performed, which resulted normal and endosonography, showing a lesion on the head of the pancreas. Octreoscan positive for pancreatic head focal lesion with positive somatostatin receptors compatible with insulinoma. Whipple surgery was performed where surgeon palpated pancreatic tumor, biopsy showed tissue compatible with diffuse nesidioblastosis. In the postoperative period, the patient decreased frequency and intensity of hypoglycemic episodes compared to their previous stage. Control prolonged fasting test and Octreoscan were within normal ranges. However, 4 months after surgery, the patient presented hypoglycemia of lower intensity and frequency than before surgery. Currently he remains in control with nutritionist and endocrinologist, mainly adjusting diet and with good control glycemias.

[Nesidioblastosis in adults]

Nesidioblastosis is a rare disorder in adults characterized by diffuse proliferation of islet cells. The ideal diagnostic and therapeutic approach is still controversial. The diagnosis in our case was made after distal pancreatectomy as the treatment of insulinoma. Finally, all the remaining pancreatic tissue had to be removed [total pancreatectomy] to control episodes of hypoglycemia

Non insulinoma pancreatogenous hypoglycemia syndrome in a Saudi male

Nesidioblastosis is focal or diffuse islet hyperplasia leading to hyperinsulinism with subsequent hypoglycemia in the absence of insulinoma, usually described in neonates and infancy. We described the first adult case of nesidioblastosis in Saudi Arabia and the Gulf region. The diagnosis and treatment of the condition can be very difficult and challenging. Despite the fact that our patient responded initially to surgical treatment, his hypoglycemic symptoms occasionally recurred and needed adjunctive medical treatment. Although initially thought to affect only infants and children, cases of nesidioblastosis can affect adults and pose a diagnostic and therapeutic challenge to the clinicians

Pancreatectomía proximal en nesidioblastosis / Proximal pancreatectomy in case of nesidioblastosis

En 1938 Laidiaw acuñó el término de nesidioblastosis refiriéndose a una neodiferenciación de los islotes de Langerhans, originada del epitelio de los conductos pancreáticos. Se presenta un caso clínico de un niño de 19 meses de edad, con convulsiones secundarias a hipoglucemia intratable. Se diagnosticó hiperinsulinismo con base en criterios clínicos y bioquímicos. Los hallazgos histopatológicos del páncreas mostraron la presencia de racimos de islotes de células P por todo el tejido acinar localizados en cabeza y un tercio proximal del cuerpo. Se realizó el diagnóstico de nesidioblastosis y para su tratamiento se practicó pancreatectomía proximal (60%), con una pancreático yeyunostomía distal (Y en Roux). Después del tratamiento, el paciente tuvo normalización de los niveles séricos de glucosa y de insulina. Se concluye que este procedimiento resultó efectivo al revertir la hipoglucemia, por lo que constituye el primer tratamiento alternativo al método convencional.

A 19 month old child who presented seizures secondary to intractable hypoglycemia, fulfilling the clinical and biochemical criteria for hyperinsulinism was studied. Histopathological findings of the pancreas showed the presence of small clusters of b cell islets throughout acinar tissue near ducts, in both the head and the proximal third of the body. Proximal pancreatectomy (60%) and distal pancreatic jejunostomy (Roux in Y) were performed. This procedure was effective in reverting hypoglycemia and constitutes the first successful alternative treatment.