Insulinoma simulando una epilepsia: reporte de caso / Insulinoma simulating epilepsy: case report

Insulinoma es un tumor neuroendocrino que surge de las células ß del páncreas y produce hiperinsulinemia endógena. Son neoplasias raras con una incidencia reportada de 4 casos por millón de habitantes por año. La presentación clínica típicamente cursa con síntomas adrenérgicos y neuroglucopénicos secundarios a hipoglicemia. Requiere estudios de niveles séricos de insulina, pro-insulina y péptido C, además de imágenes diagnosticas que confirmen los hallazgos. La mayoría de los insulinomas son benignos, su sitio primario más común es el páncreas y pueden extirparse quirúrgicamente. Se presenta el caso de un hombre de 36 años con déficit cognitivo leve y episodios de diaforesis con deterioro neurológico hasta convulsiones tónico clónicas generalizadas que curiosamente resolvían con uso doméstico de "panela molida". Se ingresó a urgencias por ataques recurrentes de hipoglicemia severa con requerimiento de altas dosis de dextrosa al 50% por acceso central, hasta confirmación diagnóstica, intervención y resección de tumor neuroendocrino pancreático bien diferenciado (G1 según clasificación OMS) tipo insulinoma en la cola del páncreas.

Insulinoma is a neuroendocrine tumor that arises from the ß cells of the pancreas and produces endogenous hyperinsulinemia. They are rare neoplasms with a reported incidence to 4 cases per million inhabitants per year. The clinical presentation typically presents with adrenergic and neuroglycopenic symptoms secondary to hypoglycemia. It requires studies of serum levels of insulin, pro-insulin and C-peptide, in addition to diagnostic images that confirm the findings. Most insulinomas are benign, their most common primary site is the pancreas, and they can be removed surgically. We present the case of a 36-year-old man with mild cognitive deficits and episodes of diaphoresis with neurological deterioration to generalized clonic tonic seizures that curiously resolved with domestic use of "ground brown sugar". He was admitted to the emergency department due to recurrent attacks of severe hypoglycemia with a high-dose requirement for 50% dextrose through central access, until diagnostic confirmation, intervention, and resection of a welldifferentiated pancreatic neuroendocrine tumor (G1 according to WHO classification) insulinoma in the tail of pancreas.

Insulinoma descubierto en paciente con aparente trastorno mental: reporte de un caso / Insulinoma found in patient with apparent mental disorder: a case report

El Insulinoma pancreático es un tumor neuroendocrino de las células beta de los islotes de Langerhans, tiene una incidencia de 4 casos por 1 000 000 habitantes. Se reporta el caso de una mujer de 36 años con alteración del comportamiento asociado a melancolía. Psiquiatría describe un examen mental anormal y diagnosticó depresión doble; sin embargo, la paciente tuvo la triada de Whipple y razón insulina/glucosa mayor a 0,3. El insulinoma fue localizado mediante resonancia magnética y tratado con cirugía. Los insulinomas pueden simular trastornos mentales, dificultando un diagnóstico temprano. Se compara los datos de la atención médica en nuestra localidad con otros obtenidos de la literatura a nivel mundial. Concluimos que a pesar de las limitaciones técnicas se debe incluir al insulinoma en el diagnóstico diferencial de trastornos mentales con hallazgo de hipoglucemia.

Pancreatic Insulinoma is a neuroendocrine tumor of the beta cells of the islets of Langerhans, has an incidence of 4 cases per 1 000 000 persons. We report the case of a 36-year-old woman with altered behavior associated with melancholy. Psychiatry describes an abnormal mental examination and diagnosed double depression; however, the patient had the Whipple triad and insulin/glucose ratio higher than 0.3. The insulinoma was localized by magnetic resonance imaging and treated with surgery. Insulinomas can mimic mental disorders, making early diagnosis difficult. We compare health care data in our locality with others obtained from the global literature. We conclude that despite the technical limitations, insulinoma should be included in the differential diagnosis of mental disorders with hypoglycemia.

Insulinoma canino: relato de caso / Canine insulinoma: case reports

O insulinoma é um tumor das células ß do pâncreas, que têm a função de produzir e secretar insulina e, geralmente são malignos em cães. O presente trabalho descreve o diagnóstico e o manejo terapêutico de três casos de insulinoma. Os sinais clínicos dos animais relatados variaram de acordo com a severidade e a duração da hipoglicemia. O diagnóstico presuntivo se deu através dos sinais clínicos e da dosagem de insulina sérica no momento de mais intensa hipoglicemia e, o diagnóstico definitivo foi obtido por meio de exame histopatológico nos três casos relatados. O tratamento realizado variou de acordo com a intensidade dos sinais clínicos.(AU)

Insulinoma is a tumor of the ß cells of the pancreas, which have the function to produce and secrete insulin and are usually malignant in dogs. This paper describes the diagnosis and therapeutic management of three cases of insulinoma. The clinical signs reported animals varied according to the severity and duration of hypoglycemia. The presumptive diagnosis was based on clinical signs and serum insulin dosage at the time of hypoglycemia and more intense, the definitive diagnosis was made by histopathological examination in three of the reported cases. The treatment was varied according to the severity of clinical signs.(AU)

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.

Hipoglicemia hiperinsulínica: a propósito de un caso / Hyperinsulinemic hypoglycemia: report of a case

El diagnóstico de hipoglicemia se confirma con la triada de Whipple: síntomas de hipoglicemia, concentración plasmática baja de glucosa, y desaparición de los síntomas con la elevación de los niveles de glucosa. Se presenta el caso de una paciente de 26 años de edad, que consultó a nuestro servicio en julio de 2013 por episodios intermitentes que se iniciaron en octubre del año precedente, consistentes en visión borrosa, cambios conductuales y discurso incoherente, sin relación con la ingesta o el ayuno, que aumentaron en frecuencia los meses previos a la consulta y llegaron a asociarse a pérdida de conocimiento y relajación de esfínteres, requiriendo asistencia médica prehospitalaria. Ocasionalmente hubo registros bajos de glicemia capilar, y hubo mejoría sintomatológica con la administración de glucosa e.v. Durante una prueba de ayuno presentó una hipoglicemia sintomática con glicemia 48 mg/dl, insulinemia 21,1 µU/ml, péptido C 2.257 ng/ml. La ecografía de abdomen identificó una imagen sólida en la unión de cuerpo y cola del páncreas. La lesión fue enucleada quirúrgicamente, y la anatomía patológica confirmó su origen neuroendócrino .

The diagnosis of hypoglycemia is confirmed by Whipple’s triad: symptoms of hypoglycemia, low plasma glucose concentrations, and disappearance of symptoms after glucose administration. The case of a 26-year-old woman is presented. She consulted our Service in July 2013 due to intermittent episodes that had begun in October of the preceding year, characterized by blurred vision, slurred speech, and behavioral changes, unrelated to food ingestion. They increased in frequency, and were associated eventually with loss of consciousness and loss of sphincters control, requiring medical care. Occasionally, low capillary glucose levels were recorded, and there was improvement in symptoms with i.v. glucose administration ev.During a fasting test she had symptomatic hypoglycemia with serum glucose of 48 mg/dl, serum insulin 21.1 µU/ml, and serum C-peptide 2.257 ng/ml. Abdominal ultrasound identified a rounded image in the union of the body and tail of the páncreas; this was confirmed by MRI. The lesion was surgically enucleated, and the pathology report confirmed its neuroendocrine origin .

Síntomas psiquiátricos e insulinoma: reporte de caso / Psychiatric symptoms and insulinoma: a case report

Insulinomas are pancreatic endocrine neoplasms with a low incidence between 1-4 cases per million per year. Case description: A female 49 years-old with neurological and psychiatric symptoms were treated for two years as a psychiatric patient. Presented a glucose value, which reflects hypoglycemia. The patient was operated with resolution of symptoms. Conclusion: Assess all patients with psychiatric symptoms and perform a complete medical history and laboratory findings, being the most opportune glucose...

Tratamento cirúrgico dos insulinomas do pâncreas / Surgical treatment of pancreatic insulinomas

RACIONAL - O insulinoma do pâncreas é neoplasia neuroendócrina proveniente das células beta das ilhotas pancreáticas. Apesar de rara, é a neoplasia endócrina pancreática mais comum, com cerca de quatro casos por milhão de pessoas. O tratamento preferencial dos insulinomas é cirúrgico. OBJETIVO - Analisar as características clínicas, epidemiológicas, cirúrgicas e anatomopatológicas dos pacientes tratados na última década em dois serviços. MÉTODOS - Foram revisados retrospectivamente os prontuários dos pacientes submetidos à cirurgia para tratamento de insulinoma no período de 1999 a 2011. Dados demográficos, tipo e duração dos sintomas, associação ou não à síndrome endócrina e exames diagnósticos foram obtidos dos registros de prontuários. Analisaram-se, também, o método cirúrgico, achados intra-operatórios e complicações imediatas e tardias. RESULTADOS - Dezesseis pacientes com diagnóstico de insulinoma foram submetidos ao tratamento cirúrgico. Foram excluídos dois por falta de registros completos no prontuário. Do total, 68,7% eram mulheres. A idade variou de 20 a 60 anos, com média de 39 anos. Apenas um caso era associado à síndrome de neoplasia endócrina múltipla tipo 1. Sintomas neuropsiquiátricos, principalmente a síncope, foram os mais comuns. A duração média das manifestações clínicas até o diagnóstico foi de um ano e meio. Exames de imagem foram utilizados em todos os pacientes com 68,7% de localização pré-operatória do tumor. Todas as operações foram realizadas de forma convencional (aberta), sem uso da laparoscopia. As lesões foram identificadas em todas as porções do pâncreas sendo a maioria na cabeça pancreática. O alívio dos sintomas só não foi obtido em um paciente. Não houve óbito entre os pacientes analisados. CONCLUSÃO - O diagnóstico de insulinoma é frequentemente estabelecido após vários meses do início das manifestações clínicas e o tratamento cirúrgico é curativo na quase totalidade dos pacientes.

BACKGROUND - Insulinoma is a pancreatic neuroendocrine tumor originated from pancreatic islet beta cells. Although rare, is the most common pancreatic endocrine tumor, with about four cases per million people. The preferential treatment of insulinoma is surgical. AIM - To analyze the epidemiological, pathological, clinical and surgical patients treated in the last decade in two surgical services. METHODS - Were retrospectively reviewed the medical records of patients undergoing surgical treatment of insulinoma in the period of 1999 to 2011. Demographic data, type and duration of symptoms, associated or not with endocrine syndrome and diagnostic tests were obtained from medical records. Were analyzed the method of surgery, intraoperative findings and immediate and late complications. RESULTS - Sixteen patients with insulinoma underwent surgical treatment, 68,7% were women. The age ranged from 20 to 60 years, with a mean age of 39 years. Only one case was associated with multiple endocrine neoplasia type 1. Neuropsychiatric manifestations, mainly syncope , were the most prevalent. The average duration of clinical manifestations until the diagnosis was one year and a half. Imaging tests were used in all patients with 68.7% of preoperative tumor localization. All operations were performed in a conventional (open) manner, without use of laparoscopy. The lesions were identified in all portions of the pancreas with the majority in the pancreatic head. Relief of symptoms was not obtained only in one patient. There were no deaths among the patients. CONCLUSION - The diagnosis of insulinoma is often established after several months of the onset of clinical manifestations and surgical treatment is curative in almost all patients.

Surgical treatment of pancreatic endocrine tumors in multiple endocrine neoplasia type 1

Surgical approaches to pancreatic endocrine tumors associated with multiple endocrine neoplasia type 1 may differ greatly from those applied to sporadic pancreatic endocrine tumors. Presurgical diagnosis of multiple endocrine neoplasia type 1 is therefore crucial to plan a proper intervention. Of note, hyperparathyroidism/multiple endocrine neoplasia type 1 should be surgically treated before pancreatic endocrine tumors/multiple endocrine neoplasia type 1 resection, apart from insulinoma. Non-functioning pancreatic endocrine tumors/multiple endocrine neoplasia type 1 >1 cm have a high risk of malignancy and should be treated by a pancreatic resection associated with lymphadenectomy. The vast majority of patients with gastrinoma/multiple endocrine neoplasia type 1 present with tumor lesions at the duodenum, so the surgery of choice is subtotal or total pancreatoduodenectomy followed by regional lymphadenectomy. The usual surgical treatment for insulinoma/multiple endocrine neoplasia type 1 is distal pancreatectomy up to the mesenteric vein with or without spleen preservation, associated with enucleation of tumor lesions in the pancreatic head. Surgical procedures for glucagonomas, somatostatinomas, and vipomas/ multiple endocrine neoplasia type 1 are similar to those applied to sporadic pancreatic endocrine tumors. Some of these surgical strategies for pancreatic endocrine tumors/multiple endocrine neoplasia type 1 still remain controversial as to their proper extension and timing. Furthermore, surgical resection of single hepatic metastasis secondary to pancreatic endocrine tumors/multiple endocrine neoplasia type 1 may be curative and even in multiple liver metastases surgical resection is possible. Hepatic trans-arterial chemo-embolization is usually associated with surgical resection. Liver transplantation may be needed for select cases. Finally, pre-surgical clinical and genetic diagnosis of multiple endocrine neoplasia type 1 syndrome and localization of multiple endocrine neoplasia type 1related tumors are crucial for determining the best surgical strategies in each individual case with pancreatic endocrine tumors.

Tumores neuroendocrinos del páncreas / Pancreatic neuroendocrine tumors

Pancreatic neuroendocrine tumors are infrequent and slow-growing neoplasms. They are classified basedon their clinical presentation as functioning and nonfunctioning tumors. The most common functionaltumors are the insulinoma and gastrinoma. They can be sporadic or be part of hereditary forms as MEN-1.The diagnosis is based on the detection of the specific clinical syndrome in association of high levels of the substance secreted by the tumor and conventional imaging studies or others such as stimulation tests, somatostatin receptor scintigraphy and endoscopic ultrasound. In general, these tumors have a better prognosis than the pancreatic adenocarcinoma and they can show metastasis to the liver and infrequently, in bones. The treatment can be managed medically diminishing the inappropriate secretion of the substances by the tumor using, for example, the somatostatin analogs. Surgery should be always considered, especially in case of insulinomas, small non-functioning tumors, and small gastrinomas that can be managed with surgery enucleation. More advanced resective surgery, such as Whipple resection, are not routinely recommended and they should be limited to selected patients. In advanced tumors, there are other treatment alternatives, for example, hepatic resection, radiofrequency, chemotherapy and new agents such as sunitinib and everolimus.

Los tumores neuroendocrinos pancreáticos son infrecuentes y de crecimiento lento. Se clasifican en tumores funcionantes o no funcionantes (TNEP-NF), de acuerdo a la presentación clínica. Los tumores funcionantes más frecuentes son los insulinomas y los gastrinomas. Pueden ocurrir en forma esporádica o asociados a síndromes hereditarios como la NEM- 1, entre otros. El diagnóstico se basa en la detección del síndrome clínico específico asociado a la demostración de niveles elevados de la sustancia secretada y exámenes imagenológicos convencionales u otros más específicos como de estimulación, cintigrafía de receptores de somatostatina y endosonografía. En general, tienen mejor pronóstico que los adenocarcinomas pancreáticos y pueden dar metástasis hepáticas y con menor frecuencia, óseas. El tratamiento puede ser médico disminuyendo la secreción inapropiada de las sustancias producidas por el tumor como los análogos de somatostatina. La cirugía siempre debe ser considerada, especialmente en caso de insulinomas, pequeños TNEP-NF, y gastrinomas pequeños, que pueden ser tratados con enucleación quirúrgica. Las cirugías resectivas más avanzadas, como la operación de Whipple no están recomendadas en forma rutinaria y sólo deben ser utilizadas en pacientes seleccionados. En casos de tumores avanzados, existen alternativas de tratamiento, como la resección hepática, radiofrecuencia, quimioterapia, y terapia con nuevos agentes en estudio como el sunitinib y everolimus.

Insulinoma: diagnóstico y tratamiento tras 5 años de manejo psiquiátrico / Insulinoma: diagnosis and treatment after 5 years of psychiatric management

El insulinoma es un tumor endocrinológico derivado de las células beta del páncreas, de baja frecuencia y de mayor prevalencia en la 5a década de la vida. Son pequeños, benignos y únicos, en la mayoría de los casos. Presentamos un caso, diagnosticado en el Servicio de Medicina Interna del Hospital Regional de Talca, en una paciente de 38 años de edad con antecedentes de depresión de 5 años de evolución en tratamiento farmacológico, que ingresó para estudio de hipoglicemias de larga data, persistentes, de ayuno. Se objetivaron hipoglicemias de ayuno e insulinemia inapropiadamente alta. TAC abdomen con contraste demostró masa hipervascular en relación al páncreas. Se realizó resección quirúrgica del tumor y el estudio inmunohistoquímico confirmó insulinoma. La evolución postoperatoria fue satisfactoria, con normalización de glicemias y notable mejoría de su cuadro depresivo.

Hiperinsulinismo na infância: quando apenas uma dosagem de insulina não é suficiente / Hyperinsulinism in infancy and childhood: when an insulin level is not always enough

INTRODUÇÃO: A hipoglicemia em bebês e crianças pode causar convulsões, atraso de desenvolvimento e dano cerebral permanente. O hiperinsulinismo (HI) é a causa mais comum de hipoglicemia, seja transitória ou permanente. A HI é caracterizada pela secreção inadequada de insulina, o que resulta em hipoglicemia persistente, de leve a grave. As diferentes formas de HI representam um grupo de doenças clínica, genética e morfologicamente heterogêneo. CONTEÚDO: Hiperinsulinismo congênito está associado às mutações de SUR-1 e Kir6.2, glucoquinase, glutamato desidrogenase, 3-hidroxiacil-CoA desidrogenase de cadeia curta e expressão ectópica de SLC16A1 na membrana plasmática das células beta. O HI pode estar associado ao estresse perinatal, como asfixia do nascimento, toxemia materna, prematuridade ou retardo do crescimento intra-uterino, resultando em hipoglicemia neonatal prolongada. Mimetismo de HI neonatal inclui pan-hipopituitarismo, hipoglicemia induzida por fármaco, insulinoma, anticorpos antiinsulina e estimuladores do receptor de insulina, síndrome de Beckwith-Wiedemann e distúrbios congênitos de glicosilação. Exames laboratoriais para HI podem incluir quantificação de glicose, insulina, β-hidroxibutirato, ácidos graxos, amônia e perfil de acilcarnitinas plasmáticos, além de ácidos orgânicos urinários. Os exames genéticos estão disponíveis em laboratórios comerciais para os genes sabidamente associados à hiperinsulinemia. Testes de resposta insulínica aguda (RIA) são úteis na caracterização fenotípica. Exames de imagem e histológicos também estão disponíveis para diagnosticar e classificar o HI. O objetivo do tratamento de crianças com HI é prevenir os danos cerebrais da hipoglicemia, mantendo níveis de glicose plasmática acima de 70mg/dl por terapia farmacológica ou cirúrgica. CONCLUSÃO:A terapêutica do HI requer abordagem multidisciplinar que inclui endocrinologistas pediátricos, radiologistas, cirurgiões e patologistas, os quais são treinados para diagnosticar..

BACKGROUND: Hypoglycemia in infants and children can lead to seizures, developmental delay, and permanent brain damage. Hyperinsulinism (HI) is the most common cause of both transient and permanent disorders of hypoglycemia. HI is characterized by dysregulated insulin secretion, which results in persistent mild to severe hypoglycemia. The various forms of HI represent a group of clinically, genetically, and morphologically heterogeneous disorders. CONTENT: Congenital hyperinsulinism is associated with mutations of SUR-1 and Kir6.2, glucokinase, glutamate dehydrogenase, short-chain 3-hydroxyacyl-CoA dehydrogenase, and ectopic expression of SLC16A1 on β-cell plasma membrane. Hyperinsulinism may be associated with perinatal stress such as birth asphyxia, maternal toxemia, prematurity or intrauterine growth retardation, resulting in prolonged neonatal hypoglycemia. Mimickers of hyperinsulinism include neonatal panhypopituitarism, drug-induced hypoglycemia, insulinoma, antiinsulin and insulin-receptor stimulating antibodies, Beckwith-Wiedemann Syndrome, and congenital glycosylation disorders. Laboratory testing for hyperinsulinism may include quantification of blood glucose, plasma insulin, plasma β-hydroxybutyrate, plasma fatty acids, plasma ammonia, plasma acylcarnitine profile and urine organic acids. Genetic testing is available at commercial laboratories for genes known to be associated with hyperinsulinism. Acute insulin response (AIR) tests are useful in phenotypic characterization. Imaging and histological tools are also available to diagnose and classify hyperinsulinism. The goal of treatment in infants with hyperinsulinism is to prevent brain damage from hypoglycemia by maintaining plasma glucose levels above 700 mg/l (70 mg/dl) through pharmacologic or surgical therapy. SUMMARY: The treatment of hyperinsulinism requires a multidisciplinary approach that includes pediatric endocrinologists, radiologists, surgeons, and pathologists who trained to diagnose...

Insulinoma, presentación y evolución de dos casos clínicos / Insulinoma, evolution and presentation of 2 clinical cases

Insulinoma is a very uncommon tumor in children, with an incidence in adults of 2 per million inhabitants. Clinical manifestations include neuroglycopenic or autonomic manifestations due to hypoglycemia. We describe 2 pediatric patients with insulinoma, characterized by repeated episodes of hypoglycemia associated to high insulin serum levels and presence of a small mass in the pancreas by imaging studies. The diagnosis was very prompt in one case and delayed in the other, emphasizing the need for an appropriate diagnosis of hypoglycemia during childhood.

El insulinoma es un tumor muy infrecuente en la edad pediátrica y la incidencia reportada en adultos es de 2 casos por millón de habitantes. La presentación de la enfermedad consiste en la presencia de síntomas neuroglicopénicos y autonómicos desencadenados por los episodios de hipoglicemia. Se describen dos pacientes con insulinoma esporádico. El cuadro clínico consistió en episodios repetidos de hipoglicemia asociados a niveles aumentados de insulina sérica y a imágenes sugerentes de un tumor pancreático. El diagnóstico fue muy oportuno en uno de los casos y muy tardío en el otro, lo que resalta la necesidad de estar muy alerta ante casos de hipoglicemia durante la niñez.

Insulinoma tem ocorrência incomum na faixa etária pediátrica: relato de um caso / Insulinoma occurence has been uncommon during childhood: report of a case

Os autores apresentam os achados clínicos, laboratoriais e radiológicos de um paciente de 14 anos. Os testes obtidos após jejum de 18 horas revelaram resultados de glicemia, insulinemia e pepitídeo-C compatíveis com a suspeita de diagnóstico de insulinoma...

Authors report a case of a 14 ys.o patient and his clinical, laboratory and image findings. Blood samples drawn during 18 hours fasting revealed glucose, insulin, and c-peptide serum levels matching the suspect of insulinoma...

Insulinoma induced hypoglycaemia in a Jamaican patient / Hipoglicemia inducida por insulinoma en un paciente de Jamaica

Herein reported is the case of a young woman who had hyperinsulinaemic hypoglycaemia which was biochemically consistent with an insulinoma. Initial imaging was negative and definitive treatment was delayed until repeat imaging localized the tumour several years later. This case demonstrates the importance of clinical judgment and biochemical testing in the diagnosis of insulinoma despite negative imaging.

Aquí se reporta el caso de una joven que presentó hipoglicemia hiperinsulinémica, bioquímicamente concordante con un insulinoma. La imagen inicial fue negativa y el tratamiento fue retardado hasta que mediante la repetición de la técnica de imaginología años más tarde localizó el tumor. Este caso demuestra la importancia de juicio clínico y las pruebas bioquímicas en el diagnóstico del insulinoma, especialmente frente a la obtención de una imagen negativa.

Hipoglicemia hiperinsulinêmica persistente endógena no adulto: relato de caso / Endogenous persistent hypoglicemia of adult: case report

A hipoglicemia hiperinsulinêmica persistente endógena em adultos é, na maioria dos casos, causada por insulinoma. A Nesidioblastose, uma hiperinsulinemia funcional rara por hiperplasia das células beta do pâncreas, tem sido descrita principalmente em neonatos. Apresentamos o caso de uma paciente de 34 anos com quadro clínico-laboratorial compatível com hipoglicemia hiperinsulinêmica endógena (Glicemia jejum: 54 mg/dl / Valor de referência (VR): 60­99 mg/dl; Insulina sérica: 70,9 mcU/ml / VR: < 29,1 mcU/ml; e Peptídeo C: 7,1 ng/ml / VR: 1,1­5,0 ng/ml - simultâneos à glicemia). Foi aventada a hipótese de insulinoma. Em função da ausência de imagem característica aos exames radiológicos (ultra-som e tomografia de abdome), optou-se pela laparotomia exploradora, onde também não foi evidenciado tumor pancreático macroscopicamente. Os exames histopatológico e imuno-histoquímico evidenciaram hiperplasia de células beta, de segmento distal do pâncreas, compatível com nesidioblastose. A paciente evoluiu com estabilidade clínica por cerca de dois meses, quando se verificou recidiva das crises hipoglicêmicas, refratárias ao uso de Octreotide. Optou-se pela realização de "octreosan", que indicou nesidioblastose difusa, sendo procedida pancreatectomia parcial extensa. Seguiu-se o uso contínuo de Octreotide, com controle eficaz das crises hipoglicêmicas. Uma vez que esse é um diagnóstico raro no adulto, objetiva-se, nesse artigo, divulgar o manejo diagnóstico-terapêutico em casos de hipoglicemia hiperinsulinêmica endógena.

Persistent Hyperinsulinemic Endogenous hypoglycemia in adults is, in most cases, due to Insulinoma. Nesidioblastosis, a peculiar functional hyperinsulinemia from hypertrophic beta cells, has been described mainly in newborns. This article describes a 34-year-old patient who presented hyperinsulinemic endogenous hypoglycemia clinical and laboratorial situation (Fasting glycemia: 54 mg/dl / Reference Interval (RI): 60­99 mg/dl; Serum insulin: 70.9 mcU/ml / RI: < 29.1 mcU/ml; e C peptide: 7.1 ng/ml / RI: 1.1­5.0 ng/ml). It was suspected Insulinoma. Because of the lack of typical images in radiologic exams (ultrasonography and computerized tomography) it had been decided to do laparotomy, but it was not found any macroscopic pancreatic tumor. Histological and histochemistry examination of a distal pancreatic segment showed alteration suitable to nesidioblastosis. The patient presented clinical stability during the next two months, however, after that, there was a recurrence of a hypoglycemia crisis, refractory to Octreotide administration. It was done "octreoscan", which showed expanded nesidioblastosis, being done extensive partial pancreatectomy. Octreotide was used again, with a good control of the hypoglycemia crisis. As it is an uncommon diagnosis in an adult, the objective of this article is to describe the diagnostic and therapeutic aspects in cases of hyperinsulinemic endogenous hypoglicemia.

Insulinoma: Diagnosis and surgical treatment. Retrospective analysis of 31 cases.

BACKGROUND : Insulinomas are rare tumors that are usually benign, single and curable by simple surgical excision. They can present problems in diagnosis and localization. STUDY DESIGN: Retrospective analysis of patients with insulinoma managed during a 13-year period (1992-2005) at a tertiary-level institution. RESULTS: 31 patients (mean age 38.4 [SD 13.3] years; 16 men) presented with hypoglycemic symptoms for 4.6 (5.5) years. In 22 (71%) patients, the lesion was successfully localized pre-operatively. Of various pre-operative localization techniques, CT angiography (5/6; 83%), intra-arterial digital subtraction angiography (11/17; 65%), dual-phase CT (8/14; 57%) and conventional MRI (4/13; 31%) had high rates of successful tumor localization. Intra-operative palpation and ultrasonography also had localization success rates (22/30 [76%] and 11/12 [92%], respectively); each identified one lesion that the other procedure did not localize. Of the 30 patients who underwent surgery, 28 had solitary tumor. CONCLUSION: Pre-operative investigations to localize insulinoma are helpful despite the availability of intra-operative ultrasound. Dual-phase CT should be the non-invasive investigation of first choice.

Neuroendocrine tumors of the pancreas.

Pancreatic Neuroendocrine tumors (PNET) are rare tumors that require a high degree of suspicion for timely diagnosis. They are best divided into functional and nonfunctional varieties. Functional tumors often are symptom specific and are diagnosed at an earlier stage than nonfunctional tumors. The severity of symptoms and pace of disease should dictate therapy. Surgical extirpation remains the only curative modality for localized disease, but palliation of hormone-related symptoms can be achieved with different modalities (Management of hormonal Excess, biologic therapy, chemotherapy and biochemotherapy, local-regional therapy with hepatic arterial embolization).